R Blanco, V M Martínez-Taboada, V Rodríguez-Valverde, M García-Fuentes
Cutaneous vasculitis in children and adults. Associated diseases and etiologic factors in 303 patients.
Medicine (Baltimore). 1998 Nov;77(6):403-18.
Abstract/Text
Cutaneous vasculitis (CV), a condition characterized by palpable purpura and nonspecific histopathologic findings, presents a diagnostic and therapeutic challenge because it may be a primary disorder or it may be a cutaneous manifestation of another entity, such as systemic necrotizing vasculitis, connective tissue disease, systemic bacterial infection, or malignancy. We studied 303 unselected patients (172 adults and 131 children) with CV to assess the disease associations and etiologic factors, to identify the frequency of primary and secondary CV in different age-groups, and to characterize features that help to distinguish between primary and secondary CV. Of the 131 children, 130 had primary CV: Henoch-Schönlein purpura (HSP) in 116 and hypersensitivity vasculitis (HV) in 14. In contrast, of the 172 adults, only 120 had primary CV: HSP in 39, HV in 70, and essential mixed cryoglobulinemia in 11. CV was a manifestation of systemic necrotizing vasculitis in 23 adults (polyarteritis nodosa in 17, Wegener granulomatosis in 4, and Churg-Strauss syndrome in 2). CV was secondary to other processes in 29 adults: in 20 patients CV was associated with connective tissue disease or another autoimmune or rheumatic disease, in 5 patients CV was a manifestation of severe bacterial infection, especially bacterial endocarditis (4 cases), and in the other 4 patients CV was the presenting symptom of an underlying malignancy. The patients for whom CV was a manifestation of systemic necrotizing vasculitis or secondary to a connective tissue disease, severe bacterial infection, or malignancy had clinical and laboratory data suggestive of the associated disorder. The clinical picture and outcome of primary CV in both children and adults were benign. By contrast, the prognosis of patients with CV in the context of systemic necrotizing vasculitis or secondary to other entities depended on the primary process. Given the different disease association in children and adults, we propose a simple diagnostic workup in children with CV. By contrast the diagnostic approach in adults with CV should be more cautious and the workup more extensive. The early differentiation between primary CV, secondary CV, and CV presenting as a symptom of systemic necrotizing vasculitis, especially in adults, is of paramount importance for an adequate diagnosis and appropriate treatment.
血管炎・血管障害ガイドライン作成委員会編：血管炎・血管障害ガイドライン．日皮会誌 2008; 118（11）: 2095-2187.
川上民裕：顕微鏡的多発血管炎・結節性多発動脈炎. 日皮会誌 2011; 121（8）:1587-1594.
M C Calviño, J Llorca, C García-Porrúa, J L Fernández-Iglesias, P Rodriguez-Ledo, M A González-Gay
Henoch-Schönlein purpura in children from northwestern Spain: a 20-year epidemiologic and clinical study.
Medicine (Baltimore). 2001 Sep;80(5):279-90.
Abstract/Text
J C Davin, I J Ten Berge, J J Weening
What is the difference between IgA nephropathy and Henoch-Schönlein purpura nephritis?
Kidney Int. 2001 Mar;59(3):823-34. doi: 10.1046/j.1523-1755.2001.059003823.x.
Abstract/Text
Henoch-Schönlein purpura nephritis (HSPN) and IgA nephropathy (IgAN) are considered to be related diseases since both can be encountered consecutively in the same patient, they have been described in twins, and bear identical pathological and biological abnormalities. Apart from the presence of extrarenal clinical signs found only in HSPN, other differences are noticed between the two diseases. The peak age ranges between 15 and 30 years for a diagnosis of IgAN, whereas HSPN is mainly seen in childhood. Nephritic and/or nephrotic syndromes are more often seen at presentation in HSPN. In contrast to IgAN, HSPN has been described in association with hypersensitivity. Endocapillary and extracapillary inflammations as well as fibrin deposits in the glomerulus are more frequent in HSPN. No major biological differences have been found between the two illnesses, except for a larger size of circulating IgA-containing complexes (IgA-CC) and a greater incidence of increased plasma IgE levels in HSPN. As tissue infiltration by leukocytes is a major feature of HSPN vasculitis, a possible role of a more potent activation of the latter cells by IgA-CC and/or circulating chemokines in HSPN should be considered. Further studies are required to elucidate this possible mechanism as well as the role of hypersensitivity in HSPN.
Alex P Betrosian, Tom Berlet, Banwari Agarwal
Purpura fulminans in sepsis.
Am J Med Sci. 2006 Dec;332(6):339-45.
Abstract/Text
Sepsis-induced purpura fulminans is a rare but life-threatening disorder, characterized by hemorrhagic infarction of the skin caused by disseminated intravascular coagulation and dermal vascular thrombosis. The pathogenesis is linked to enhanced expression of the natural procoagulants and depletion of the natural anticoagulant proteins particularly protein C. Meningococcal sepsis is the most common cause, followed by pneumococcal sepsis in adults. The syndrome is associated with more than 50% mortality secondary to multiple organ dysfunction syndrome and is accompanied by long-term morbidity. Necrotic lesions usually progress to distal ischemia, and skin grafting and extremities or limb amputation are often required. Early antibiotic administration and intensive care management according to the recommendations of severe sepsis and shock is crucial for patients' survival. Adjuvant therapies against inflammatory and coagulation cascades and augmenting fibrinolysis are still controversial and need further assessment. Among them activated protein C and supplementation therapy have given promising results.
Mark D Denton, Subba R Digumarthy, Sarah Chua, Robert B Colvin
Case records of the Massachusetts General Hospital. Case 20-2006. An 84-year-old man with staphylococcal bacteremia and renal failure.
N Engl J Med. 2006 Jun 29;354(26):2803-13. doi: 10.1056/NEJMcpc069012.
Abstract/Text
V M Martinez-Taboada, R Blanco, M Garcia-Fuentes, V Rodriguez-Valverde
Clinical features and outcome of 95 patients with hypersensitivity vasculitis.
Am J Med. 1997 Feb;102(2):186-91.
Abstract/Text
PURPOSE: To evaluate the clinical features and outcome of patients with isolated hypersensitivity vasculitis (HV).
PATIENTS AND METHODS: Retrospective study of patients with cutaneous vasculitis followed up at a University Hospital from 1975 to 1994. Patients with vasculitis secondary to collagen vascular diseases, neoplasia, or major infections were excluded. Patients were classified as HV according to the differential criteria proposed by Michel et al (J Rheumatol. 1992;19:721-728).
RESULTS: Ninety-five patients were classified as HV. The mean age was 42.7 +/- 21.7 years, with similar disease frequency in both sexes. In 43 patients, the precipitating event was drug therapy, either alone or as a treatment for a coexistent infection, usually an upper respiratory tract infection. The most frequent clinical manifestation was palpable purpura followed by joint symptoms. Systemic involvement was infrequent: 7 patients had nephropathy, manifested almost exclusively by microhematuria, and 5 patients had gastrointestinal symptoms. In 54 subjects the vasculitis did not require treatment; 26 patients were treated with NSAIDs, and 14 required corticosteroids (associated to immunosuppressive agents in 2 of them). After a mean follow-up of 15.5 +/- 28.9 months (median 6), only 2 patients had slight renal impairment, whereas the remaining had a complete recovery.
CONCLUSION: Hypersensitivity vasculitis is usually a benign syndrome, often secondary to drugs or infections, or both. Its main clinical manifestations are skin and joint symptoms. The systemic involvement is scarce and its prognosis is excellent.
G Sais, A Vidaller, A Jucglà, F Gallardo, J Peyrí
Colchicine in the treatment of cutaneous leukocytoclastic vasculitis. Results of a prospective, randomized controlled trial.
Arch Dermatol. 1995 Dec;131(12):1399-402.
Abstract/Text
BACKGROUND AND DESIGN: Cutaneous leukocytoclastic vasculitis is an inflammatory vascular disease with a variable course. There is no defined therapy for this entity. Contradictory data on the effect of colchicine have been reported. To determine the efficacy of colchicine in cutaneous leukocytoclastic vasculitis, 41 patients were randomly selected to receive oral colchicine, 0.5 mg twice daily, or topical emollients. Response to treatment was judged according to the reduction in the number of lesions. After 1 month, in those patients in whom a complete or no response was achieved, therapy was withdrawn; in those with a partial response, treatment was maintained for the following 2 months. At the end of 3 months, treatment was continued only in those patients in whom a relapse occurred.
RESULTS: Twenty patients in each group completed 1 month of treatment. One patient taking colchicine dropped out because of diarrhea. At the end of the first month of the study, five patients in the control group and four in the colchicine group achieved a complete response. Nine patients who had a partial response (four in the colchicine group and five in the control group) continued to receive treatment for the following 2 months. Three patients in the colchicine group suffered a relapse after discontinuing therapy but experienced remission with reinstitution of therapy. At the end of the 3 month period, 12 patients in the colchicine group and 10 patients in the control group showed no significant response. Complete response was achieved in five patients in the colchicine group and in seven in the control group. At the 1-year follow-up, 10 patients in each group had no clinical evidence of cutaneous vasculitis.
CONCLUSIONS: Colchicine had no significant therapeutic effect in this controlled study. However, the finding that relapse occurred on cessation of colchicine therapy in three complete responders suggests that colchicine can be effective in some patients, despite our negative results.
R Wolf, B Tüzün, Y Tüzün
Dapsone: unapproved uses or indications.
Clin Dermatol. 2000 Jan-Feb;18(1):37-53.
Abstract/Text
日本皮膚科学血管炎・血管障害診療ガイドライン改訂版作成委員会編：血管炎・血管障害診療ガイドライン2016年改訂版．日皮会誌 2017；127：299-415.
L H Calabrese, G F Duna
Drug-induced vasculitis.
Curr Opin Rheumatol. 1996 Jan;8(1):34-40.
Abstract/Text
Vasculitis resulting from drug use includes a wide variety of clinical and pathologic conditions that are, in general, empirically defined and poorly understood. Further complicating our grasp of these disorders are ambiguous terms such as hypersensitivity vasculitis, allergic vasculitis, leukocytoclastic vasculitis, serum sickness, and others, which are often used interchangeably without clear definition. The clinical picture varies widely from self-limiting to progressive and even fatal illness. These syndromes have now been reported in association with newer classes of therapeutic agents including biologic response modifiers. Vasculitis affecting the central nervous system may be related to a variety of drugs and remains one of the more important syndrome sets within the spectrum of drug-induced vasculitis. These disorders are clinically important, because removal of the offending drug often is associated with regression of the vasculitic condition.
F G Mullick, H A McAllister, B M Wagner, J J Fenoglio
Drug related vasculitis. Clinicopathologic correlations in 30 patients.
Hum Pathol. 1979 May;10(3):313-25.
Abstract/Text
Drug related vasculitis has variously been described as necrotizing hypersensitivity or allergic angiitis or microscopic panarteritis nodosa. We reviewed tissue sections from 30 patients with validated drug hypersensitivity and vasculitis in order to precisely define this entity. No evidence of necrotizing vascular lesions or of fibrinoid associated with necrosis was found. The vascular lesions in all 30 patients involved small arteries, arterioles, capillaries, and venules. The inflammatory infiltrate consisted primarily of mononuclear cells and prominent numbers of eosinophils and was present in all three layers of the involved vessel walls. Clinically the patients developed either localized or systemic vasculitis, which could not be predicted on the basis of the associated drug. The findings of a skin rash, fever, or eosinophilia and the development of symptoms consistent with a hypersensitivity reaction while medication was being taken were all suggestive of the diagnosis of drug related vasculitis.
C W Parker
Allergic reactions in man.
Pharmacol Rev. 1982 Mar;34(1):85-104.
Abstract/Text
The general features of allergic drug reactions in man have recently been reviewed by Parker (85). By definition allergic drug reactions are produced by specific immunologic processes. Allergic drug reactions must be distinguished from adverse reactions due to overdosage, normal pharmacologic action, toxic metabolite formation, idiosyncrasy, nonspecific release of pharmacologic effector molecules, or drug interactions. The clinical manifestations of drug allergy are quite protean. In addition to classical manifestations of allergy such as serum sickness, anaphylaxis, contact dermatitis or urticaria, drug allergy may produce hemolytic anemia, thrombocytopenia, granulocytopenia, hepatitis, nephritis, pneumonitis, vasculitis, or neuritis where a single organ or cell type is affected. While many drugs produce reactions with suggestive of allergy, definitive experimental evidence either for or against mechanism is usually not available. Some of these reactions may involve allergic mediators released or produced nonimmunologically through pharmacologic, osmotic, or toxic effects on cells involved in immune inflammation (mast cells, basophils, phagocytes, and lymphocytes) or through nonspecific activation of effector molecules in extracellular fluid such as the complement proteins. Drugs may also induce the formation of autoantibodies through mechanisms that are largely obscure, but may in some instances involve the direct participation of the drug as a hapten and in other instances occur indirectly through a pharmacologic or toxic action on the cells responsible for immune homeostasis.
Wen-Liang Chang, Yao-Hsu Yang, Li-Chieh Wang, Yu-Tsan Lin, Bor-Luen Chiang
Renal manifestations in Henoch-Schönlein purpura: a 10-year clinical study.
Pediatr Nephrol. 2005 Sep;20(9):1269-72. doi: 10.1007/s00467-005-1903-z. Epub 2005 Jun 10.
Abstract/Text
Henoch-Schönlein purpura (HSP) is an IgA-mediated systemic small vessel vasculitis of childhood. It is characterized by the symptoms including nonthrombocytopenic purpura, abdominal pain, hematuria/proteinuria, and arthargia/arthritis. We conducted a retrospective study of 261 patients diagnosed with HSP from December 1991 to December 2001. Of the 261 patients, fifty-three (20.3%) developed renal manifestations after onset of the disease. Two patients developed nephrotic syndrome. Four patients had group A beta-hemolytic streptococcal pharyngitis and subsequent depressed serum C3 concentration typical of post streptococcal glomerulonephritis. During the study period, the renal survival rate after disease onset was 100%. The prognosis of renal involvement was better than reports from other series. In this study we also found factors associated with HSP nephritis; these included older age at onset, GI bleeding, and central nervous system involvement. The long-term morbidity of HSP is predominantly attributed to renal involvement. It is thus recommended that patients with HSP nephritis are followed for longer periods of time.
Sandra Trapani, Annalisa Micheli, Francesca Grisolia, Massimo Resti, Elena Chiappini, Fernanda Falcini, Maurizio De Martino
Henoch Schonlein purpura in childhood: epidemiological and clinical analysis of 150 cases over a 5-year period and review of literature.
Semin Arthritis Rheum. 2005 Dec;35(3):143-53. doi: 10.1016/j.semarthrit.2005.08.007.
Abstract/Text
OBJECTIVE: To examine epidemiological, clinical, and outcome in Italian children affected with Henoch Schönlein purpura (HSP).
METHODS: Retrospective study of children discharged with a diagnosis of HSP from the Meyer Children's Hospital, between 1998 and 2002. Epidemiological, clinical, laboratory data, treatment, and outcome were collected by reviewing medical charts. One year after data collection, the children's parents were interviewed by telephone about the outcome.
RESULTS: 150 children entered the study: M:F=1.8:1; mean age 6.1+/-2.7 years. At onset, purpura was present in all cases, arthritis/arthralgias in 74%, abdominal involvement in 51%, scrotal edema in 13%, renal involvement in 54%, severe nephropathy in 7%, acute renal insufficiency in 2%, and intussusception in 0.6%. Purpura was the presenting symptom in 74%, arthritis in 15%, and abdominal pain in 12%. The most frequent laboratory abnormalities were high-erythrocyte sedimentation rate (ESR) (57%), hyper-IgA (37%), and proteinuria (42%). All patients recovered within 2 months. Recurrences, verified in 35%, were correlated with high ESR values and corticosteroid (CS) treatment, independently from other variables. After a mean 2.5-years follow-up, 2 patients had hematuria with normal renal function.
CONCLUSION: Epidemiological and clinical findings in our cohort are similar to those in the literature, even though the mean disease duration was shorter than previously reported. Relapses occurred significantly more frequently in children treated with CS. This finding supports the recommendation to limit the use of steroids to a carefully selected group of HSP children. The prognosis was excellent; although severe nephropathy was found in a small percentage of the children, at follow-up all had normal renal function. Thus, our study confirms the benignity of HSP in Italian children, especially regarding renal outcome.
F T Saulsbury
Henoch-Schönlein purpura in children. Report of 100 patients and review of the literature.
Medicine (Baltimore). 1999 Nov;78(6):395-409.
Abstract/Text
Henoch-Schönlein purpura (HSP) is an acute leukocytoclastic vasculitis that primarily affects children. In the current report, the author presents the clinical features of 100 children with HSP and reviews the literature, placing particular emphasis on new information concerning the etiology, immunopathogenesis, and treatment of HSP. The dominant clinical features of HSP are cutaneous purpura (100%), arthritis (82%), abdominal pain (63%), gastrointestinal bleeding (33%), and nephritis (40%). The etiology of HSP remains unknown, but it is clear that IgA plays a critical role in the immunopathogenesis of HSP, as evidenced by increased serum IgA concentrations, IgA-containing circulating immune complexes, and IgA deposition in vessel walls and renal mesangium. There are 2 subclasses of IgA, but HSP is associated with abnormalities involving IgA1 exclusively, and not IgA2. This finding may be a consequence of abnormal glycosylation of O-linked oligosaccharides unique to the hinge region of IgA1 molecules. Although several lines of evidence suggest a genetic susceptibility to HSP, the fundamental basis for the abnormalities involving IgA remain unclear. In general, HSP is an acute, self-limited illness, but one-third of patients will have 1 or more recurrences of symptoms. Corticosteroid therapy may hasten the resolution of arthritis and abdominal pain, but does not prevent recurrences. To date, no form of therapy has been shown to shorten appreciably the duration of HSP. The long-term prognosis of HSP is directly dependent on the severity of renal involvement. Corticosteroids in usual doses have no effect on established nephritis. Evidence is emerging that treatment with high-dose intravenous pulse methylprednisolone coupled with azathioprine or cyclophosphamide may be beneficial in patients with severe nephritis.
Tae-Sun Ha, Jin-Seok Lee
Scrotal involvement in childhood Henoch-Schönlein purpura.
Acta Paediatr. 2007 Apr;96(4):552-5. doi: 10.1111/j.1651-2227.2006.00173.x. Epub 2007 Feb 14.
Abstract/Text
AIM: Henoch-Schönlein purpura (HSP) is a common childhood systemic vasculitis involving the skin, gastrointestinal tract, joint, kidneys and even scrotum.
METHODS: We retrospectively reviewed the clinical and laboratory data of 120 male patients with HSP and also evaluated the risk factors for scrotal involvement and the relation between scrotal involvement and other clinical features. Twenty-six out of 120 boys (21.7%) diagnosed with HSP had scrotal involvement.
RESULTS: Scrotal symptoms manifested as swelling in 88.5% and pain (or tenderness) in 69.2% of HSP patients with scrotal involvement. Neurologic symptoms, mainly headache and localized edema among various manifestations and high serum C3 level of laboratory profiles were more frequently observed in scrotal-involved group than in those of non-involved group. However, there was no difference in the outcomes of scrotal symptoms according to therapeutic modalities and the occurrence of scrotal involvement had no correlation with renal involvement from acute to chronic phase.
CONCLUSIONS: We found that neurologic symptoms, localized edema and high serum C3 level show a significant relation with scrotal involvement in male HSP patients. Because scrotal involvement in male HSP patients is not rare, the accurate early diagnosis of HSP is mandatory by the early notification of purpura and imaging evaluations in order to avoid unnecessary procedures.
A L Belman, C R Leicher, S L Moshé, A P Mezey
Neurologic manifestations of Schoenlein-Henoch purpura: report of three cases and review of the literature.
Pediatrics. 1985 Apr;75(4):687-92.
Abstract/Text
Three patients developed prominent neurologic symptoms and signs associated with Schoenlein-Henoch purpura. A 7 1/2-year-old boy was seen with status epilepticus after a 2-week history of generalized headaches, irritability, and intermittent colicky abdominal pain. A left hemiparesis and a left homonymous hemianopia with a right gaze preference that were present on initial examinations gradually resolved, but a mild left arm paresis persisted. Cutaneous, renal, and joint involvement followed initial CNS manifestations. The second patient, a 7-year-old girl, had a complex partial seizure with secondary generalization and a postictal hemiparesis seven days after presentation with classic signs of Schoenlein-Henoch purpura. Behavioral changes were noted during the acute phase of the illness. The third patient, a 13-year-old boy, developed signs of a left brachial plexopathy and transient weakness of his right leg during a complicated course of Schoenlein-Henoch purpura. Review of the world literature indicates that headaches and mental status changes are the most frequent neurologic complications of Schoenlein-Henoch purpura, followed by seizures, focal neurologic deficits, mononeuropathies, and polyradiculoneuropathies. The vasculitis of Schoenlein-Henoch purpura can involve the nervous system and may add significantly to the morbidity of the illness.
A K Misra, A Biswas, S K Das, P K Gharai, T Roy
Henoch-Schonlein purpura with intracerebral haemorrhage.
J Assoc Physicians India. 2004 Oct;52:833-4.
Abstract/Text
Henoch-Schonlein purpura is a leucocytoclastic vasculitis commonly seen among children and young adults. Neurological complications, though rare, include focal cerebral deficit, coma, convulsion, subarachnoid hemorrhage and chorea. We are reporting a 12 years boy with Henoch-Schonlein purpura who developed a large intracerebral hematoma in right occipital lobe. He made an uneventful recovery with conservative treatment and one year follow up revealed no major neurological sequelae.
A Bulun, R Topaloglu, A Duzova, I Saatci, N Besbas, A Bakkaloglu
Ataxia and peripheral neuropathy: rare manifestations in Henoch-Schönlein purpura.
Pediatr Nephrol. 2001 Dec;16(12):1139-41. doi: 10.1007/s004670100048.
Abstract/Text
Henoch-Schönlein purpura (HSP) is a multisystemic vasculitis. Nervous system involvement is usually underestimated. Headaches, mental status changes and seizures are the most frequent neurologic symptoms. Ataxia and mononeuropathy are both very rare. We present an 11-year-old boy with HSP who suffered from ataxia during the initial presentation and peripheral neuropathy at the time of a relapse. Brainstem vasculitic involvement was shown by magnetic resonance imaging, while cranial tomography was normal. All the neurologic symptoms and signs resolved following bolus methylprednisolone administration. Ten months later he had a second course of HSP with skin and renal involvement. A percutaneous renal biopsy, which was performed due to persistent hematuria, revealed mesangial proliferation with IgA deposition. During that period the patient experienced pain and numbness in the right foot and leg; electromyography showed signs of mononeuritis multiplex involving the right posterior tibial nerve. The patient responded to steroid therapy.
K R Vats, A Vats, Y Kim, D Dassenko, A R Sinaiko
Henoch-Schönlein purpura and pulmonary hemorrhage: a report and literature review.
Pediatr Nephrol. 1999 Aug;13(6):530-4. doi: 10.1007/s004670050652.
Abstract/Text
Pulmonary hemorrhage, a rare complication of Henoch-Schönlein purpura (HSP) reported primarily in adults and adolescents, is associated with significant mortality. Although it has been suggested that pulmonary hemorrhage also occurs in children with HSP, the few cases reported lack a clear differentiation from pauci-immune vasculitis. We report a prepubertal child with HSP, pulmonary hemorrhage, and immunofluorescence-documented IgA deposits on renal biopsy. Aggressive supportive management and steroid therapy led to successful recovery. A review of the current literature is presented. Because other conditions clinically mimic HSP, appropriate serological studies and a kidney biopsy to confirm the diagnosis should be performed in severely affected patients with renal disease.
M M K Muqit, M J Gallagher, M Gavin, F Roberts, A G Jardine
Henoch-Schonlein purpura with keratitis and granulomatous anterior uveitis.
Br J Ophthalmol. 2005 Sep;89(9):1221-2. doi: 10.1136/bjo.2004.064519.
Abstract/Text
H Narchi
Risk of long term renal impairment and duration of follow up recommended for Henoch-Schonlein purpura with normal or minimal urinary findings: a systematic review.
Arch Dis Child. 2005 Sep;90(9):916-20. doi: 10.1136/adc.2005.074641. Epub 2005 May 4.
Abstract/Text
BACKGROUND: The duration of follow up to assess the risk of long term renal impairment in Henoch-Schönlein purpura (HSP) without nephritic or nephrotic syndrome or renal failure on diagnosis remains undetermined.
AIMS: To undertake a systematic review of the literature to assess whether the risk of long term renal impairment without renal involvement on diagnosis could be estimated and to determine the time period when renal involvement is very unlikely after the diagnosis of HSP.
METHODS: Search of studies of unselected children with HSP, and available information on urinary findings, renal involvement, and long term renal function follow up. Studies of selected children with HSP nephropathy at diagnosis were excluded.
RESULTS: Twelve studies of 1133 children were reviewed. The follow up period ranged from 6 weeks to 36 years. Proteinuria and/or haematuria, which occurred in 34.2%, of which only one fifth were in association with nephritic or nephrotic syndrome, developed in 85% of cases within 4 weeks of the diagnosis of HSP, in 91% within 6 weeks, and in 97% within 6 months. Permanent renal impairment never developed after normal urinalysis; it occurred in 1.6% of those with isolated urinary abnormalities, and in 19.5% of those who developed nephritic or nephrotic syndrome.
CONCLUSION: No long term renal impairment occurred after normal urinalysis. Even if urinalysis is normal at presentation, the testing should be continued for six months. There is no need to follow up after the first six months those whose urinalysis remains normal.
Evangéline Pillebout, Eric Thervet, Gary Hill, Corinne Alberti, Philippe Vanhille, Dominique Nochy
Henoch-Schönlein Purpura in adults: outcome and prognostic factors.
J Am Soc Nephrol. 2002 May;13(5):1271-8.
Abstract/Text
Henoch-Schönlein Purpura nephritis (HSPN) has been extensively studied in children but, its natural history in adults is much less known. A cohort of 250 adults suffering HSP was retrospectively analyzed for a median follow-up period of 14.8 yr. All patients had biopsies consistent with HSP (predominant IgA mesangial deposits) associated with purpura, bowel angina, and/or abdominal pain. At presentation, palpable purpura was present in 96% of patients, and arthritis was reported in 61%, and gastrointestinal involvement in 48%. Thirty-two percent of the patients showed renal insufficiency (Creatinine clearance [CrCl] <50 ml/min), usually associated with proteinuria (99%) and/or hematuria (93%). Endocapillary glomerulonephritis was the most frequent lesion on renal biopsy (61%). At the end of follow-up, patient survival was only 74%. The first cause of death was carcinoma (most of them of respiratory or digestive tract). Regarding renal function, 11% of patients reached end-stage renal failure, 13% exhibited severe renal failure (CrCl <30 ml/min), and 14% moderate renal insufficiency (CrCl <50 ml/min). Clinical remission defined as the absence of proteinuria, hematuria, and a normal renal function was achieved in only 20%. This is a retrospective study; therefore, it is not possible to demonstrate any steroid and/or cyclophosphamide efficacy in diminishing the incidence of renal insufficiency. Multivariate analysis demonstrated that renal function impairment and proteinuria level at presentation and, on renal biopsy, the degree of interstitial fibrosis, percentage of sclerotic glomeruli, and presence of glomeruli with fibrinoid necrosis were associated with a poor renal prognosis. The data indicate that clinical presentation of HSPN in adults is severe and its outcome relatively poor, worse than in children. Identification of clinical and histologic prognostic factors may permit the design of appropriate therapeutic prospective studies.
H Iqbal, A Evans
Dapsone therapy for Henoch-Schönlein purpura: a case series.
Arch Dis Child. 2005 Sep;90(9):985-6. doi: 10.1136/adc.2004.061598.
Abstract/Text
D Pyne, R Mootoo, A Bhanji
Colchicine for the treatment of recurrent Henoch-Schönlein purpura in an adult.
Rheumatology (Oxford). 2001 Dec;40(12):1430-1.
Abstract/Text
Jaana Ronkainen, Olli Koskimies, Marja Ala-Houhala, Marjatta Antikainen, Jussi Merenmies, Jukka Rajantie, Timo Ormälä, Juha Turtinen, Matti Nuutinen
Early prednisone therapy in Henoch-Schönlein purpura: a randomized, double-blind, placebo-controlled trial.
J Pediatr. 2006 Aug;149(2):241-7. doi: 10.1016/j.jpeds.2006.03.024.
Abstract/Text
OBJECTIVE: To evaluate the efficacy of early prednisone therapy in preventing renal and treating extrarenal and renal symptoms in Henoch-Schönlein purpura (HSP) in a placebo-controlled trial.
STUDY DESIGN: A total of 171 patients (84 treated with prednisone and 87 receiving placebo) were included and followed up for 6 months. The endpoints were renal involvement at 1, 3, and 6 months and healing of extrarenal symptoms. The analyses were performed on an intent-to-treat basis.
RESULTS: Prednisone (1 mg/kg/day for 2 weeks, with weaning over the subsequent 2 weeks) was effective in reducing the intensity of abdominal pain (pain score, 2.5 vs 4.8; P = .029) and joint pain (4.6 vs 7.3; P = .030). Prednisone did not prevent the development of renal symptoms but was effective in treating them; renal symptoms resolved in 61% of the prednisone patients after treatment, compared with 34% of the placebo patients (difference = 27%; 95% confidence interval = 3% to 47%; P = .024).
CONCLUSIONS: The general use of prednisone in HSP is not supported, but patients with disturbing symptoms may benefit from early treatment, because prednisone reduces extrarenal symptoms and is effective in altering (but not preventing) the course of renal involvement.
Pamela F Weiss, James A Feinstein, Xianqun Luan, Jon M Burnham, Chris Feudtner
Effects of corticosteroid on Henoch-Schönlein purpura: a systematic review.
Pediatrics. 2007 Nov;120(5):1079-87. doi: 10.1542/peds.2007-0667.
Abstract/Text
OBJECTIVE: No consensus exists among general pediatricians or pediatric rheumatologists regarding whether corticosteroid therapy ameliorates the acute manifestations of Henoch-Schönlein purpura or mitigates renal injury. Therefore, we sought to synthesize the reported experimental and observational data regarding corticosteroid use.
METHODS: We performed a meta-analysis based on a comprehensive review of the literature in the Medline database (1956 to January 2007) and the Cochrane Controlled Trials Register. On the basis of reported outcomes among patients with Henoch-Schönlein purpura who were treated at diagnosis with corticosteroids compared with patients treated with supportive care only, we calculated odds ratios for the resolution of abdominal pain, the need for surgical intervention secondary to severe pain or intussusception, the likelihood of Henoch-Schönlein purpura recurrence, and the development of transient or persistent renal disease.
RESULTS: Of 201 articles retrieved from the initial literature search, 15 were eligible for inclusion. Corticosteroid treatment did not reduce the median time to resolution of abdominal pain but did significantly reduce the mean resolution time and increased the odds of resolution within 24 hours. Early corticosteroid treatment significantly reduced the odds of developing persistent renal disease. In addition, although the results were not statistically significant, the prospective data suggest reduced odds of both surgical intervention and recurrence.
CONCLUSIONS: Corticosteroids, given early in the course of illness, seem to produce consistent benefits for several major clinically relevant Henoch-Schönlein purpura outcomes.
稲葉基之、小坂洋子、田中周他、十二指腸潰瘍を伴ったHenoch-Schonlein紫斑の一例。皮膚科の臨床2010；52：7-11.
P Niaudet, R Habib
Methylprednisolone pulse therapy in the treatment of severe forms of Schönlein-Henoch purpura nephritis.
Pediatr Nephrol. 1998 Apr;12(3):238-43.
Abstract/Text
Between 1980 and 1994, 38 children with severe forms of Schönlein-Henoch purpura glomerulonephritis were entered into a prospective study to evaluate methylprednisolone pulse therapy on the outcome of nephropathy in terms of clinical symptoms and histopathological changes. The patients were considered at risk of developing chronic renal failure when they presented with a nephrotic syndrome and/or had 50% or more crescentic glomeruli. Initial renal biopsies were obtained from all patients and revealed diffuse proliferative endocapillary glomerulonephritis in 2, focal and segmental glomerulonephritis in 4, and endo- and extracapillary glomerulonephritis in 32, 21 of whom had 50% or more glomeruli with crescents. Patients were treated with intravenous pulse methylprednisolone (3 days) followed by oral prednisone (3.5 months). At the latest follow-up, 1-16 years after initiation of therapy, 27 children had clinically recovered, 3 showed minimal urinary abnormalities, 4 persistent nephropathy, and 4 had progressed to end-stage renal failure. Sequential renal biopsies were obtained from 30 patients, 7-25 months after initiation of therapy. The clinical outcome correlated well with of the activity (hypercellularity, cellular and fibrocellular crescents, and interstitial edema with mononuclear cell infiltrates) and the chronicity (fibrous crescents, glomerular sclerosis, tubular atrophy, and interstitial fibrosis) indexes of post-therapy biopsies. Of particular interest were the post-therapy biopsies of the 18 patients who clinically recovered. They showed a significant decrease of the activity index from 5.1+/-1.1 to 0.4+/-0.8 with a decrease or even a disappearance of IgA deposits, while the chronicity index remained low (0.4+/-0.8 compared with 1.4+/-1). Although uncontrolled, our study suggests that methylprednisolone pulse therapy is effective in those patients at risk of progression of their nephropathy, especially if started early during the course of the disease before the crescents become fibrous.
Marco Zaffanello, Milena Brugnara, Massimo Franchini
Therapy for children with henoch-schonlein purpura nephritis: a systematic review.
ScientificWorldJournal. 2007 Jan 10;7:20-30. doi: 10.1100/tsw.2007.23. Epub 2007 Jan 10.
Abstract/Text
Although severe kidney involvement in children with Henoch-Shonlein purpura (HSP) is rarer than that in adults, morbidity should not be underevaluated and follow-up is mandatory. Some drugs are introduced as well-defined treatment options, others can be promising therapeutic alternatives. Therapy of HSP nephritis in children can range from simply steroids to combined immunosuppressant treatments. The prophylactic treatment for renal complication of patients with HSP has been sometimes suggested, but with conflicting results and ultimately not clearly proven. The treatment of overt HSP nephritis includes steroids and other immunosuppressant drugs. Methylprednisolone pulse therapy and prednisone per os are tested drugs. These steroids could be used in combination with other immunosuppressant drugs, such as cyclosporin A and cyclophosphamide. Unfortunately, of these two drugs, only cyclophosphamide is demonstrated as effective in a recent randomized controlled trial. However, since there are insufficient data and unstructured study designs, ACE-I, azathioprine, mycophenolate mofetil, and urokinase need to be more tested in childhood HSP nephritis. In addition to drugs, other techniques are used to treat the severe form of nephritis. Of these, in a multicenter study, plasmapheresis demonstrated efficacy in delaying the progression of kidney disease. However, no convincing studies have been made to date concerning either intravenous immunoglobulin, factor XIII administration, antioxidant vitamin E, and fish oil to treat HSP nephritis.
Jee Min Park, Sung Chul Won, Jae Il Shin, Hyunee Yim, Ki Soo Pai
Cyclosporin A therapy for Henoch-Schönlein nephritis with nephrotic-range proteinuria.
Pediatr Nephrol. 2011 Mar;26(3):411-7. doi: 10.1007/s00467-010-1723-7. Epub 2010 Dec 24.
Abstract/Text
To evaluate the therapeutic role of cyclosporin A (CyA) for the treatment of Henoch-Schönlein nephritis (HSN), 29 patients (18 boys, 11 girls) with nephrotic-range proteinuria were analyzed retrospectively. Mean age was 8.6 years (range 2.0-15.5 years) at diagnosis of Henoch-Schönlein purpura (HSP). All patients had developed the nephrotic-range proteinuria at a mean interval of 4.4 months (range 0-50.7 months) after the diagnosis of HSP. Mean duration of CyA treatment was 12.3 months (range 2.6-55.0 months). Mean follow-up times were 3.7 years (range 1.2-12.9 years) from the beginning of the CyA treatment. Steroids were tapered off and stopped gradually after initiation of CyA. All patients responded to the CyA treatment within a mean of 1.8 months (range 1 week to 3.5 months). Twenty-three patients achieved stable remission with mean follow-up duration of 3.2 years and 6 patients seemed to become CyA-dependent, since they developed proteinuria when the treatment was stopped. Renal function was preserved in all patients but one who developed end-stage renal disease after poor compliance with CyA. We concluded that CyA treatment for HSN showing nephrotic-range proteinuria is very effective and a safe method, although some patients become CyA-dependent.
J I Shin, J M Park, Y H Shin, J H Kim, J S Lee, H J Jeong
Henoch-Schönlein purpura nephritis with nephrotic-range proteinuria: histological regression possibly associated with cyclosporin A and steroid treatment.
Scand J Rheumatol. 2005 Sep-Oct;34(5):392-5. doi: 10.1080/03009740510026544.
Abstract/Text
OBJECTIVE: To clarify the therapeutic role of cyclosporin A (CyA) for patients with Henoch-Schönlein purpura nephritis (HSPN) showing nephrotic-range proteinuria.
METHODS: The clinical and histological findings of eight children (7.7+/-3.8 years), who were treated with CyA and prednisolone, were evaluated retrospectively. All underwent a renal biopsy before therapy, and six of the eight patients received a follow-up biopsy after therapy.
RESULTS: The histological grade of the International Study of Kidney Disease in Children (ISKDC) was improved in all six patients who received a follow-up biopsy (pre-therapy, four grade IIIa and two grade IIIb; post-therapy, one grade I and five grade II) and it was statistically significant (p = 0.031). The activity index was significantly decreased after therapy (8.3+/-1.6 vs. 3.5+/-1.5, p = 0.031), and the chronicity index (0.5+/-0.5 vs. 0.7+/-1.0) and tubulointerstitial (TI) scores (1.5+/-1.3 vs. 0.8+/-1.6) did not change. There was a reduction in proteinuria from 3.2+/-2.3 to 0.1+/-0.1 g/m2/day (p = 0.008) and renal function remained normal in all patients after therapy. However, one patient showed CyA-induced nephrotoxicity at a second biopsy. After an average follow-up period of 3.8 years, six patients showed normal urine and renal function, and two showed minor urinary abnormalities.
CONCLUSION: This study suggests that CyA therapy is effective in reducing proteinuria, which is a known risk factor for the development of renal insufficiency in HSPN and may regress the renal pathology in patients with nephrotic-range proteinuria.
Penina Tarshish, Jay Bernstein, Chester M Edelmann
Henoch-Schönlein purpura nephritis: course of disease and efficacy of cyclophosphamide.
Pediatr Nephrol. 2004 Jan;19(1):51-6. doi: 10.1007/s00467-003-1315-x. Epub 2003 Nov 22.
Abstract/Text
Nephritis in Henoch-Schönlein purpura (HSP) is the primary cause of morbidity and mortality. Although many therapeutic regimens have been reported to be effective, no therapy has been shown in a controlled trial to be beneficial. Fifty-six patients with histopathologically severe HSP nephritis were randomized to receive supportive therapy with or without cyclophosphamide, 90 mg/m(2)/day for 42 days. Patients were classified according to status at final follow-up: Fully Recovered 48.2%, Persistent Abnormalities 39.3%, or ESRD/Death 12.5%. There were no differences in onset data or outcome between the two trial groups or in outcome between trial and 23 non-trial patients followed concurrently. Therefore, data from trial and non-trial patients were combined for further analysis. There was no correlation between outcome and age, blood pressure, serum total protein, or serum albumin. Although rates of proteinuria did not correlate with outcome, all those with progression to ESRD had nephrotic levels of proteinuria at onset. Only five of 28 patients with nephrotic levels of proteinuria and severe onset histopathology recovered fully. No patient with crescents in 50% or more of glomeruli went on to full recovery. Recurrence of non-renal symptoms did not correlate with outcome. Nephrotic syndrome, decreased GFR, and more severe histopathology at onset, as well as persistence of urinary abnormalities for several years, are ominous signs.
J T Flynn, W E Smoyer, T E Bunchman, D B Kershaw, A B Sedman
Treatment of Henoch-Schönlein Purpura glomerulonephritis in children with high-dose corticosteroids plus oral cyclophosphamide.
Am J Nephrol. 2001 Mar-Apr;21(2):128-33. doi: 46235.
Abstract/Text
BACKGROUND: Henoch-Schönlein Purpura (HSP) is a common childhood vasculitis with manifestations in numerous organ systems, including glomerulonephritis. Patients with more severe HSP-associated glomerulonephritis may develop chronic renal failure. Currently, no widely accepted treatment protocols exist for patients with significant renal involvement.
METHODS: We retrospectively reviewed the clinical courses of 12 children (mean age 9 years) with HSP glomerulonephritis treated with high-dose corticosteroids plus oral cyclophosphamide. All patients had nephrotic-range proteinuria, and all had significant histopathologic changes on biopsy, including crescentic nephritis in 10 patients. Treatment consisted of either intravenous pulse methylprednisolone or oral prednisone followed by oral cyclophosphamide (2 mg/kg/day) for 12 weeks, along with either daily or alternate-day oral prednisone. Prednisone was tapered following completion of cyclophsophamide.
RESULTS: Serum albumin rose significantly after treatment from 2.8 +/- (SD) 0.5 to 3.7 +/- 0.4 g/dl (p < 0.001), and there was a concurrent reduction in proteinuria, as reflected by decreasing serial protein-to-creatinine ratios: from 6.3 +/- 4.4 to 0.8 +/- 0.8 (p = 0.002). Renal function remained normal in all patients. Hypertension developed during treatment in 10 patients, all but 1 of whom were normotensive at last follow-up, 35 +/- 17 months following biopsy.
CONCLUSIONS: We conclude that treatment of children with HSP nephritis with high-dose corticosteroids plus oral cyclophosphamide is safe and, as in nephrotic syndrome, appears to significantly reduce proteinuria which is a known risk factor for the development of renal insufficiency in HSP. Further studies with larger numbers of patients should be conducted to confirm this finding.
Copyright 2001 S. Karger AG, Basel
Hiroshi Tanaka, Koichi Suzuki, Tohru Nakahata, Etsuro Ito, Shinobu Waga
Early treatment with oral immunosuppressants in severe proteinuric purpura nephritis.
Pediatr Nephrol. 2003 Apr;18(4):347-50. doi: 10.1007/s00467-003-1094-4. Epub 2003 Mar 28.
Abstract/Text
Nine Japanese children with severe proteinuric Henoch-Schönlein purpura nephritis (HSPN) received prompt initiation of oral prednisolone (1.5 mg/kg/day) combined with an 8-week course of cyclophosphamide (2 mg/kg/day) therapy. All underwent renal biopsy before and after treatment. At presentation, urine protein excretion and histologic indices of the mean activity index, the mean chronicity index and the tubulointerstitial (TI) scores in the patients were 5.0+/-1.4, 4.7+/-1.0, 3.9+/-1.6 and 3.7+/-0.5 g/day, respectively. Urine protein excretion, the activity index and the TI scores decreased significantly at the second renal biopsies obtained at a mean interval of 23 months after the first [0.3+/-0.3, 2.4+/-0.5 and 1.4+/-0.7 g/day ( P<0.01), respectively], while the chronicity index did not change. At the latest observation (mean interval 78 months), all except two showed negative proteinuria while no patient showed renal impairment. Although this case series is without controls, our experience suggests that early treatment with oral prednisolone and cyclophosphamide may be beneficial to a proportion of patients with severe proteinuric HSPN.
Yukihiko Kawasaki, Junzo Suzuki, Hitoshi Suzuki
Efficacy of methylprednisolone and urokinase pulse therapy combined with or without cyclophosphamide in severe Henoch-Schoenlein nephritis: a clinical and histopathological study.
Nephrol Dial Transplant. 2004 Apr;19(4):858-64. doi: 10.1093/ndt/gfg617.
Abstract/Text
BACKGROUND: There have been few controlled studies of combined therapy with multiple drugs, including immunosuppressives, for severe Henoch-Schoenlein nephritis (HSPN). We evaluated the efficacy of methylprednisolone and urokinase pulse therapy combined with cyclophosphamide for patients with HSPN of at least grade IVb.
METHODS: We studied 37 patients who had been diagnosed with HSPN of at least grade IVb. Of them, 20 (Group A) were treated with methylprednisolone and urokinase pulse therapy, and 17 (Group B) were treated with methylprednisolone and urokinase pulse therapy combined with cyclophophamide. We analysed the clinical features, laboratory and pathological findings of the two groups retrospectively.
RESULTS: After 6 months of treatment, mean urinary protein excretion in Group B had significantly decreased compared with Group A, and the activity index of both groups at the second biopsy was lower than that at the first. Furthermore, at the second biopsy, the chronicity index of Group B was lower than that of Group A. Four patients of Group A but none of Group B had persistent nephropathy (P<0.05).
CONCLUSIONS: Our study suggests that methylprednisolone and urokinase pulse therapy combined with cyclophosphamide is useful for patients with severe HSPN.
J Bergstein, J Leiser, S P Andreoli
Response of crescentic Henoch-Schoenlein purpura nephritis to corticosteroid and azathioprine therapy.
Clin Nephrol. 1998 Jan;49(1):9-14.
Abstract/Text
The benefits of treating severe Henoch-Schoenlein Purpura (HSP) glomerulonephritis have not been established. In this study, we evaluate the outcome of 21 children with severe HSP nephritis treated with corticosteroids and azathioprine. Between 1977 and 1995, 78 children (age range 1 to 16 years) were seen for evaluation of HSP. Thirty-one underwent kidney biopsy; indications included nephritic and/or nephrotic onset (15 patients), persistently decreased creatinine clearance (5 patients), or proteinuria > 4 g/24 h (11 patients). Twenty treated patients had diffuse mesangial proliferation with crescents in 6-100% (mean 40%) of glomeruli. One treated patient, not biopsied due to extreme obesity, had a creatinine clearance of 49 ml/min/1.73 m2 and proteinuria of 21.3 g/24 h. These 21 patients were initially treated with azathioprine and daily oral prednisone (13 patients) or i.v. methyl-prednisolone (8 patients), followed by azathioprine and alternate-day prednisone for 9-24 (mean 15) months. The average follow-up was 32 months. Over the course of follow-up, 19 treated patients showed a decline in hematuria (> 5 red blood cells/high power field) from 100% to 16% (p < 0.01), a fall in the serum creatinine from 1.71 +/- 2.20 to 0.78 +/- 0.25 mg/dl (p < 0.01), an increase in creatinine clearance from 76 +/- 43 to 122 +/- 26 ml/min/1.73 m2 (p < 0.01), and a reduction in proteinuria from 8.8 +/- 7.5 to 0.47 +/- 0.39 g/24 h (p < 0.01). Two treated patients progressed to end-stage renal failure. There was no difference in outcome comparing patients initially treated with prednisone versus methyl-prednisolone. These observations suggest that corticosteroid and azathioprine therapy is effective in crescentic HSP nephritis.
M Hattori, K Ito, T Konomoto, H Kawaguchi, T Yoshioka, M Khono
Plasmapheresis as the sole therapy for rapidly progressive Henoch-Schönlein purpura nephritis in children.
Am J Kidney Dis. 1999 Mar;33(3):427-33.
Abstract/Text
To clarify the therapeutic role of plasmapheresis (PP) for patients with Henoch-Schönlein purpura (HSP) nephritis, the clinical courses of nine children with a rapidly progressive type of HSP nephritis, who were treated with PP as the sole therapy, were retrospectively evaluated. All patients had nephrotic-range proteinuria (4.9 +/- 2.5 g/m2/d, mean +/- SD) and decreased glomerular filtration rate (GFR) (46.5 +/- 9.5 mL/min/1.73 m2) at the time of the initiation of PP. Biopsy specimens taken before PP showed large crescents involving more than 50% of the glomerular circumference in 56.8 +/- 6.9% of the glomeruli examined. The mean interval between disease onset and initiation of PP was 39.1 +/- 22.1 days. The PP regimen consisted of thrice-weekly treatment for 2 weeks, then weekly treatment for 6 weeks. No patients received any steroids or cytotoxic drugs, except for the use of steroids to manage severe abdominal pain. All patients responded promptly to PP with improvement in renal function, reduction of proteinuria, and subsidence of purpuric rash and abdominal pain. Six of nine patients showed further improvements without any other treatments; four had complete recovery, and two had only microscopic hematuria at the latest observation (follow-up period, 9.6 +/- 4.3 years). The remaining three patients showed a rebound increase of proteinuria after completion of PP; two of whom progressed to end-stage renal failure at 14.1 years and 1.8 years after disease onset. Because all patients had the most severe forms of nephritis, reported to carry a grave prognosis, this study suggests that PP as the sole therapy is effective in improving the prognosis of patients with rapidly progressive HSP nephritis, particularly if instituted early in the course of the disease. The role of PP in treating HSP nephritis deserves to be assessed further in larger randomized controlled trials.
H Fukui, H Kamitsuji, T Nagao, K Yamada, J Akatsuka, M Inagaki, S Shike, Y Kobayashi, K Yoshioka, S Maki
Clinical evaluation of a pasteurized factor XIII concentrate administration in Henoch-Schönlein purpura. Japanese Pediatric Group.
Thromb Res. 1989 Dec 15;56(6):667-75.
Abstract/Text
Arthral, abdominal and renal symptoms in Henoch-Schönlein purpura (HSP) were scored. Coagulation factor XIII (F XIII) activity was determined in fifty-six children with HSP and the correlation with the severity score of the clinical symptoms was investigated. As a result, it was found that the decrease in F XIII level was correlated with the severity score of clinical symptoms, particularly abdominal symptoms. Based on the results, a controlled study was performed in 24 cases with moderate symptoms divided into a group treated with F XIII concentrate and a non-treated group to investigate clear-cut efficacy as a next study. In three days after the administration the symptoms were improved remarkably in accordance with the increase of F XIII level compared with non-treated group and scoring of clinical symptoms was confirmed to be useful for assessing the application of the F XIII concentrate to HSP.
