Background/Purpose:

Rituximab has recently been shown to be equipotent to cyclophosphamide for induction of remission in generalized ANCA-associated vasculitis. A substantial number of observational pro- and retrospective studies also investigate the efficacy of RTX in refractory ANCA-assiciated vasculitis.  However, there are only few data regarding the effectiveness of rituximab in Churg-Stauss syndrome. Objective: To investigate the overall efficacy and safety of rituximab in Churg-Strauss syndrome at a tertiary vasculitis referral center.

Methods:

This study represents a retrospective, standardized data collection from all Churg-Strauss syndrome patients treated with rituximab from 06/2007 to 06/2012. Patients were assessed in a standardized diagnostic procedure (ANCA, CRP, B cell levels, immunoglobulin levels, Eosinophil count, Birmingham Vasculitis Activity Score, glucocorticoid demand etc.) before and after receiving rituximab. After achieving complete remission or a response under rituximab, patients were switched on maintenance therapy with methotrexat or azathioprine or leflunomide.

Results:

11 patients were included in the study. Five were ANCA positive. Five were refractory to standard cyclophophamide treatment, three were relapsers and three patients could not be treated with cyclophosphamide in order to preserve fertility (2) or because of hemorrhagic cystitis (1). Manifestations prior to rituximab were sinusitis (7), alveolitis (7), polyneuropathy/mononeuritis (4), myositis (2), glomerulonephritis (1), cardiac involvement (1), skleritis (1), gastrointestinal involvement (1), purpura (2) and arthritis (2). Eight patients had more than one manifestation before the start of RTX. The median BVAS was 7 (range 1-27).

Regarding overall efficacy eight patients had a response (1 remission, 7 response), one patient was refractory. In two patients follow-up was pending.  Birmingham Vasculitis Activity Score version 3 (BVAS 3), glucocorticoid demand, Eosinophil count, CRP and immunoglobulin levels decreased in all patients (table 1). ANCA and peripheral blood lymphocyte counts became undetectable after rituximab treatment. After a median follow-up of eight months (1-54) there was no increase in disease activity. Three infections occurred during follow-up: one pneumonia, two bronchopulmonary infection.

Table 1

	Before rituximab Median (range)	After rituximab Median (range)
BVAS 3	7 (1-27)	4 (1-8)
glucocorticoid demand (mg)	30 (5-80)	9 (4-12)
Eosinophil count (Eosinophils/µl)	300 (0-3300)	200 (0-1600)
CRP (mg/dl)	0,6 (0,1-5,5)	0,2 (0-8)
immunoglobulin G (g/l)	7,4 (0,3-11)	5,3 (5-7,7)

Conclusion:

The overall response rate of Churg-Strauss syndrome to RTX was high (1 remission, 7 respond). There was no increase in disease activity in all patients until the end of follow-up.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/successful-treatment-of-churg-strauss-syndrome-with-rituximab/