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.

Early Recognition and Institution of Treatment is Associated with a Good Prognosis in Peripheral Ischemic Lesions in Rheumatic Diseases

“…A single-centre study from Western India reported 22 cases of connective tissue disease-associated gangrene with the most common cause being mixed connective tissue disease. 16 They have variable presentations and outcomes ranging from complete recovery to amputation. These factors have been largely undefined in the literature.…”

Black Digits Matter: A Multispecialty Enigma

“…Connective tissue diseases like systemic lupus erythematosus (SLE), antiphospholipid antibody syndrome (APLA/APS), systemic sclerosis (SSc), and even systemic vasculitis may present with critical digital ischemia. About 9% of SSc and 5% of SLE patients present with digital gangrene [4]. APLA presents as pregnancy morbidity and/or thrombotic events due to the presence of antibodies like lupus anticoagulant, anticardiolipin antibodies, and anti-Beta2GP1 antibodies.…”

The Importance of Prompt Diagnosis and Management of Digital Gangrene Associated with Rheumatic Diseases

“…A few other studies had also evaluated the aetiologies of gangrene in the context of rheumatic diseases (summarised in Table 1). [1][2][3][4] The prevalence and prognosis of peripheral gangrene vary with the underlying rheumatic disease. As an example, in patients with systemic sclerosis, data from the EUSTAR cohort reveal that nearly a tenth of patients experience digital gangrene.…”