Takayasu's arteritis- experience in a tertiary care centre in eastern India

Abstract

Introduction: Limited data on Churg Strauss syndrome from western India. Aims: To describe the clinical profile and outcome of patients with Churg Strauss syndrome. Methods: Demographic profile, clinical, laboratory and treatment details, BVAS, EULARdisease stages and therapeutic outcome of 28 patients presented at two centers from 2003-2014 were analyzed. Results: There were 28 patients [16 (57.14%) males & 12 (42.86%) females]. Mean age at presentation was 46.6±15.14 yrs and mean duration of illness at diagnosis was 18.1±17.2 months. Mean duration of asthma prior to CSS was 4.4 ± 5.6 years. The most common systemic features were peripheral neuropathy 25 (89.28%) and pulmonary infiltrate 12 (42.85%). ANCA was positive in 22/28 (78.57%) patients. Mean BVAS at presentation was 14.7 ±5.6. The EULAR disease stage at presentation was early systemic 25 (89.28%), and generalized 3 (10.71%). Steroid was used in induction (Parenteral 25%, oral 75%) and maintenance in all patients. Other immunosuppressant's used for induction were cyclophosphamide 22 (78.57%), azathioprine 5 (17.85%) and methotrexate 1 (3.57%). Maintenance immunosuppressant's were azathioprine 11 (39.28%), methotrexate 6 (21.42%), cyclophosphamide 7 (25%), only steroid 3 (10.71%) and Mycofenolate 1(3.57%). The mean duration of follow-up was 46.9 ± 29.3 months with remission in 27 (96.42%) patient after mean duration of 6.5 ± 2.5 months. Mean number of relapses were 1.4 ± 1.8. No death occurred during follow up. Conclusions: Apart from chest symptoms, peripheral neuropathy was an important manifestation. Steroids with cyclophosphamide were most commonly used for induction. Remission was achieved in most and relapses were uncommon.