Comparative clinical and epidemiological study of hypersensitivity vasculitis versus Henoch-Schönlein purpura in adults

• Published in: Seminars in arthritis and rheumatism Vol. 28; no. 6; pp. 404 - 412
• Main Authors: García-Porrúa, Carlos, González-Gay, Miguel A.
• Format: Journal Article
• Language: English
• Published: Philadelphia, PA Elsevier Inc 01.06.1999; Elsevier
• Subjects: Adult; adults; Aged; Aged, 80 and over; Biological and medical sciences; Cutaneous vasculitis; Dermatology; Female; Follow-Up Studies; Henoch-Schönlein purpura; Humans; hypersensitivity vasculitis; Incidence; leukocytoclastic vasculitis; Male; Medical sciences; Middle Aged; Purpura, Schoenlein-Henoch - diagnosis; Purpura, Schoenlein-Henoch - drug therapy; Purpura, Schoenlein-Henoch - epidemiology; Retrospective Studies; Risk Factors; Sarcoidosis. Granulomatous diseases of unproved etiology. Connective tissue diseases. Elastic tissue diseases. Vasculitis; Spain - epidemiology; Treatment Outcome; Vascular disorders of the skin; Vasculitis, Leukocytoclastic, Cutaneous - diagnosis; Vasculitis, Leukocytoclastic, Cutaneous - drug therapy; Vasculitis, Leukocytoclastic, Cutaneous - epidemiology; Spain; Henoch-Schönlein purpura; Cutaneous vasculitis; leukocytoclastic vasculitis; hypersensitivity vasculitis; adults; Human; Immunopathology; Allergy; Skin disease; Diseases of the osteoarticular system; Clinical form; Cardiovascular disease; Epidemiology; Retrospective; Incidence; Vascular disease; Vasculitis; Capillary vessel disease; Diagnosis; Comparative study
• ISSN: 0049-0172; 1532-866X
• DOI: 10.1016/S0049-0172(99)80006-7

To assess the incidence and clinical features of adults with hypersensitivity vasculitis (HV) and Henoch-Schönlein purpura (HSP) in a well-defined population. Retrospective study of an unselected population of adult patients (>20 years) with biopsy-proven cutaneous vasculitis diagnosed as having HV or HSP who presented at a primary hospital between 1988 and 1997. Patients with cutaneous vasculitis secondary to collagen vascular diseases, neoplasia, severe infections, and those with other well-defined clinical entities were excluded. Patients were classified as having either HV or HSP according to the criteria proposed by Michel et al (J Rheumatol 1992;19:721-28). Fifty-six adults (35 men/21 women), were classified as having HV and27 adults as having HSP (19 men/8 women). The annual incidence rate for HV was 29.7/million and 14.3/million for HSP. At the onset of the disease, adults with HSP were younger than those with HV (46 ± 18 years versus 59 ± 18 years in HV; P = .005). Precipitating events were found in 50% of HV and in 30% of HSP patients. A history of drug therapy before the onset of vasculitis was found in 46% of HV and in 26% of HSP ( P = .074). At disease onset, skin lesions were the most common manifestation in both groups. During the disease course, adults with HSP had joint manifestations more commonly (59% in HSP v25% in HV; P < .003) and more gastrointestinal (82% v 5% in HV; P < .001) and renal complications (48% v 5% in HV; P < .001). HSP subjects required more aggressive therapy consisting of steroids ( P < .001) or cytotoxic agents ( P < .001). After 37 ± 28 (median, 31) months, complete recovery was observed in 98% of adults with HV. After 40 ± 27 (median, 36) months, complete recovery was observed in only 67% of adults with HSP ( P < .001). Renal insufficiency was observed in 8% of adults with HSP In adults, HV and HSP as defined by these criteria, behave as two well-differentiated diseases. HV has a milder course and lack of severe complications, and HSP a higher risk of gastrointestinal and renal complications.