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Arthritis Rheumatol. 2017 Sep;69(9):1862-1870. doi: 10.1002/art.40178.

Characteristics and Management of IgA Vasculitis (Henoch-Schönlein) in Adults: Data From 260 Patients Included in a French Multicenter Retrospective Survey.

Author information

1
Department of Internal Medicine, National Referral Center for Rare Systemic Autoimmune Diseases, Hôpital Cochin, AP-HP, Université Paris Descartes, Paris, France.
2
Center for Clinical Epidemiology, Hôtel Dieu, AP-HP, Paris, France.
3
Department of Dermatology, Hôpital Tarnier, AP-HP, Paris, France.
4
Department of Internal Medicine, Hôpital Pitié-Salpétrière, AP-HP, Paris, France.
5
Department of Internal Medicine and Nephrology, Centre Hospitalier de Valenciennes, Valenciennes, France.
6
Department of Nephrology, AP-HM, Marseille, France.
7
Department of Internal Medicine and Vascular Disease, Centre Hospitalier Universitaire d'Angers, Angers, France.
8
Department of Nephrology, Centre Hospitalier Universitaire d'Angers, Angers, France.
9
Department of Internal Medicine, Centre Hospitalier Universitaire de Toulouse, Toulouse, France.
10
Department of Internal Medicine, Centre Hospitalier Universitaire de la Réunion, Réunion, France.
11
Department of Internal Medicine, Centre Hospitalier Universitaire de Grenoble, Grenoble, France.
12
Department of Nephrology, Hôpital Necker, AP-HP, Paris, France.
13
Department of Internal Medicine, Centre Hospitalier Universitaire de Tours, Tours, France.
14
Department of Internal Medicine, Centre Hospitalier Universitaire de Poitiers, Poitiers, France.
15
Department of Nephrology and Organ Transplantation, Centre Hospitalier Universitaire de Toulouse, Toulouse, France.
16
Department of Internal Medicine, Centre Hospitalier Universitaire de St. Etienne, St. Etienne, France.
17
Department of Internal Medicine, Centre Hospitalier Universitaire de Nice, Nice, France.
18
Department of Internal Medicine, Hôpitaux Privés de Metz, Metz, France.
19
Department of Internal Medicine, Centre Hospitalier Universitaire de Bordeaux, Bordeaux, France.
20
Centre Hospitalier Universitaire Lille, Département de Médecine Interne et Immunologie Clinique, Lille, France.
21
Department of Internal Medicine, Centre Hospitalier Universitaire de Caen, Caen, France.
22
Department of Internal Medicine, Centre Hospitalier Universitaire de Clermont-Ferrand, Clermont-Ferrand, France.
23
Department of Internal Medicine, Hôpital de Bondy, AP-HP, Paris, France.
24
Department of Nephrology, Centre Hospitalier de St. Lo, St. Lo, France.
25
Department of Internal Medicine, Centre Hospitalier Universitaire de Lyon, Lyon, France.
26
Department of Nephrology, Hôpital Européen Georges Pompidou, AP-HP, Université Paris Descartes, Paris, France.
27
Department of Nephrology, Hôpital Saint Louis, AP-HP, Université Paris Descartes, Paris, France.

Abstract

OBJECTIVE:

Data on adult IgA vasculitis (Henoch-Schönlein) (IgAV) are scarce. This survey was designed to better define the clinical spectrum of IgAV and efficacy of treatments in a French patient population.

METHODS:

Data on clinical characteristics, histologic features, and treatment response from 260 patients with IgAV included in a French multicenter retrospective survey were analyzed. Efficacy data were compared using different statistical models.

RESULTS:

The mean ± SD age of the patients with IgAV at diagnosis was 50.1 ± 18 years, and 63% of patients were male. Baseline manifestations included purpura (100%), arthralgias/arthritis/myalgia (61%), glomerulonephritis (70%), and/or gastrointestinal involvement (53%). Thirty percent of patients showed renal failure at baseline. In univariate analysis, the response to therapy was 80% (64 of 80) in patients treated with corticosteroids (CS) alone, compared to 77% (23 of 30) in patients treated with CS plus cyclophosphamide (CYC) and 59% (10 of 17) in patients treated with colchicine (P = 0.17). Multivariable analysis showed that treatment with CS or CS plus CYC was more effective than colchicine in achieving a response. Efficacy differences were demonstrated using different statistical models: in the multivariable logistic regression model, odds ratio (OR) 3.68, 95% confidence interval (95% CI) 1.10-12.33 (P = 0.03); in the inverse probability weighting on propensity score model, OR 3.75, 95% CI 1.28-10.99 (P = 0.02). The efficacy of CS plus CYC as compared to CS alone was discordant according to the analytic method used. Analysis with the multivariable logistic regression model did not demonstrate a difference between CS plus CYC and CS alone (OR 0.88, 95% CI 0.29-2.67; P = 0.82). In contrast, inverse probability weighting on propensity score showed that CS plus CYC was more effective than CS alone (OR 1.79, 95% CI 1.00-3.20; P = 0.049).

CONCLUSION:

This series constitutes the largest series of adults with IgAV reported in the literature so far. It provides data on clinical and histologic presentation and therapeutic efficacy, suggesting that CS alone appears to be a reasonable first-line therapy in patients with IgAV, while the benefit of adding CYC to CS remains uncertain.

PMID:
28605168
DOI:
10.1002/art.40178
[Indexed for MEDLINE]
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