Clinical characteristics and outcomes of digital gangrene in connective tissue disorders: a longitudinal single-centre experience from Jodhpur, India

Abstract

Epidemiology, clinical presentation, and outcomes for digital gangrene in connective tissue disorders (CTD) remain underreported from tropical countries like India. In this series, we aimed to explore the clinical profile and outcomes of patients who presented with digital gangrene and a diagnosis of CTD. Hospital-based longitudinal observational study. Patients with digital gangrene and underlying diagnosis of CTD presenting to our tertiary-care centre in Jodhpur, India between1^st January 2018 and 31^st June 2021 were included. Clinical outcomes including mortality, limb outcomes, functional status and other systemic involvement were assessed. Of the 312 patients registered in the rheumatology clinic during this period, 22 (7%) patients were found to satisfy the inclusion criteria. Mean age was 46 years and 90% were females. The most common underlying diagnosis was Mixed connective tissue disorder (MCTD). Digital gangrene was the presenting symptom in 13 (60%) patients. Half of the patients received only corticosteroids as immunosuppression. Two died due to systemic complications. Complete resolution occurred in 17 (85%), autoamputation in 3, and infection requiring surgical drainage in one patient. All surviving patients reported good functional limb outcome on 6 months follow-up. MCTD is an important cause of digital gangrene in rheumatology practice. In patients presenting with digital gangrene, an active search for an underlying CTD is imperative, as this could result in timely initiation of appropriate limb-saving therapy. Corticosteroids alone with rapid tapering may be an appropriate option to consider in the initial management of digital gangrene in CTD. Key Points • Mixed connective tissue disorder is an important cause of digital gangrene in rheumatology practice in western India. • In patients presenting with digital gangrene, an active search for an underlying connective tissue disorder is imperative, as this could result in timely initiation of appropriate therapy and can prove limb saving. • Corticosteroids alone with rapid tapering may be an appropriate option to consider in the initial management of digital gangrene in connective tissue disorders.

Keywords: Critical-limb ischemia; Mixed connective tissue disorder; Raynaud’s phenomenon; Systemic lupus erythematosus; U1RNP.