Treatment of refractory or recurrent cutaneous IgA vasculitis in adults

Introduction

IgA vasculitis is a systemic vasculitis with deposits of immune complexes affecting small vessels. It typically presents as a constant purpura, arthralgia, and abdominal pain. Severe manifestations are possible, such as gastrointestinal bleeding or glomerulonephritis. It is a rarer vasculitis in adults than in children, with more frequent relapses or refractory disease, and a poorer prognosis due to more frequent and more serious renal damage. In non-severe IgA vasculitis, treatment involves symptomatic measures and in case of failure colchicine can be used, but evidence-based data are lacking. In order to evaluate the possible therapeutic strategies of refractory or relapsing forms, we studied the therapeutic efficacy of different strategies, their maintenance times and their toxicity.

Patients and methods

This French retrospective multicenter study included patients from 17 different hospital centers, followed in internal medicine or dermatology departments. The inclusion criteria were: to have had a diagnosis of IgA vasculitis and to have presented at least one skin relapse, or refractory skin disease, after a first line of medication. The primary endpoint was the skin response rate at 3 months. The secondary endpoints were the length of time each treatment was continued, and their toxicity.

Results

Fifty-two patients were included. The mean age at diagnosis was 36.7 years and 64% were male. All patients presented with purpura, 79% joint involvement, 52% digestive involvement, and 17% renal involvement. Before inclusion in the study, the first-line treatment was colchicine alone in 58% of patients, glucocorticoids (GCs), alone or in combination with colchicine or dapsone, in 37%, and dapsone alone at 4%. Azathioprine associated with GCs, rituximab alone, and hydroxychloroquine associated with GCs, were each received by a patient. After inclusion, patients received a median 1 (1-2.3) additional treatment line. Twenty-one (40%) of the patients received two additional lines of therapy (i.e. three lines in total), 13 (25%) three additional lines, 4 (8%) four additional lines, and 4 (8%) five additional lines or more. At 3 months, a cutaneous response was obtained in 85% of patients treated with dapsone alone, and in 75% of patients treated with GCs, alone or in combination with colchicine or dapsone. The relapse rates for these two strategies were similar (35% and 29%, respectively). A skin response at 3 months was obtained in 63% of patients treated with cDMARDs (conventional disease-modifying antirheumatic drugs), in combination with GCs, and in 57% of patients treated with colchicine alone. Rituximab only produced a skin response in 28% of cases. The cutaneous responses obtained with the other therapies, budesonide, hydroxychloroquine and cyclophosphamide, used less than 5 times each, were variable. On the other hand, in the presence of a monoclonal peak, chemotherapy targeted on the clone made it possible to obtain a cutaneous response in all the patients, without relapse. Dapsone and cDMARDs had the longest median maintenance times (19.1 months and undefined, respectively) compared to rituximab (15.5 months), GCs (14.1 months) and colchicine (6 month). No patient died and no severe adverse effects occurred in our study.

Conclusion

The therapeutic management of adult patients with relapsing or refractory non-severe IgA vasculitis is not clearly defined. Dapsone, GCs and cDMARDs showed the best skin response rates at 3 months, with similar relapse rates. The use of rituximab, on the other hand, has shown disappointing results. In the presence of a monoclonal peak, chemotherapy targeted on the clone revealed efficacy without relapse in all of our patients.

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