Background Henoch-Schonlein purpura (HSP) is a non thrombopenic leucocytoclasic acute systemic vasculitis of the small vessels with IgA-immun complex deposits, most commonly affecting the skin, joints, gastro-intestinal tract and the kidneys. It commonly affects the children aged between 4 to 10 years. The occurrence of HSP in adults is significantly less frequent with atypical profile, and more serious complications.
Objectives Analysis of the clinical presentations, biological characteristics, treatment and evolutions of HSP in this population, compared with other tunisian and international studies.
Methods This is a monocentric retrospective study using an operating record data from 30 adult patients aged 16 to 82 years hospitalized for HSP in Internal Medicine Department in Monastir university hospital between May 2005 to July 2015. Inclusion criteria's where those defined in 2006 by the European League Against Rheumatism (ELAR) and the Pediatric Rheumatology European Society (PRES) for the diagnosis of HSP.
Results Thirty patients with Henoch-Schönlein purpura (18 women and 12 men) were included in the study. The mean age at onset of the disease was 48.80±18.77 years (16 -82 years). The vascular purpura was the most frequent inaugural clinical manifestation (90%). It was diffuse and necrotic in 3.3% of cases. Cutaneous biopsy practiced in 26 cases (86.6%), found leukocytoclastic vasculitis in 21 cases (80.7%). Joint involvement was present in 80%: Inflammatory arthralgia in 56.6% and arthritis in 23,3%. Sixteen patients (53.3%) had gastro-intestinal involvement: Gastric and duodenal ulcers, occlusive syndrome, gastrointestinal bleeding and pancreatitis in 26,6%, 16,6%, 10% and 3,3% respectively. Eighteen patients (60%) had renal involvement. Nephritic syndrome was observed in thirteen patients (43,33%). Hematuria was objectified in 11 patients. Four patients had renal failure (13,33%). The renal biopsy practiced in 4 cases, objectified IgA nephropathy in 3 patients and glomerulonephritis extra-capillary in 1 case. Interstitial lung disease with bilateral pleurisy was observed in a patient. A woman had a cerebral vasculitis. Corticosteroids were prescribed in 8 patients for gastrointestinal and renal impairment. Two patients received cyclophosphamide cures for cerebral vasculitis and severe digestive impairment. Anti-inflammatory drugs were prescribed in 9 cases. A favorable spontaneous development was observed in 9 patients. A recurrence was reported in 2 cases. One patient dead by severe sepsis.
Conclusions HSP manifests in adult patients as a more severe and atypical disease with more relapses and more frequent and severe gastro-intestinal and renal disease. HSP in adults requires a sustainable monitoring and more aggressive treatment
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Danhua Shu et al. Risk factors of progressive IgA nephropathy which progress to end stage renal disease within ten years: a case–control study, BMC Nephrol. 2017; 18: 11.
Acknowledgements Department of Immunology, university Hospital, F Bourguiba Monastir Tunisia.
Disclosure of Interest None declared