Anti ‐synthetase syndrome associated interstitial lung disease after combination dual immune checkpoint inhibition

In this study, we describe the first reported case of antisynthetase syndrome; characterized by circulating anti-EJ (glycyl) t-RNA synthetase antibodies accompanied by relapsing, fibrosing and treatment-resistant interstitial pneumonitis. This case study highlights the need to also consider rarer phenotypes  of ICI-induced inflammatory lung injury beyond conventional ‘ICI pneumonitis’ in the diagnostic workup of these patients. Differentiating between rare forms of CTD-ILD (in particular IIM-ILD) and conventional ‘ICI pneumonitis’ has important implications for the expected natural history and optimal treatment paradigms after immunotherapy. AbstractImmune checkpoint inhibitors (ICIs) unleash potent anti-tumour responses but with frequent off-target immune-mediated adverse events (irAE). ICIs can induce a spectrum of rheumatologic manifestations including inflammatory arthritis, Sj ögren's syndrome, scleroderma and systemic lupus erythematosus. Here, we describe a case of antisynthetase syndrome associated interstitial lung disease (ILD) following dual Programmed Cell Death 1 and Cytotoxic T Lymphocyte-Associated Protein 4 checkpoint inhibition in a patient with metastatic me lanoma. Initial treatment course was complicated by a number of irAEs including pneumonitis, colitis and thyroiditis. Suspicion of an underlying systemic rheumatic disease was heightened by the severe, relapsing and fibrosing nature of the interstitial pneumonitis. A diagnosis of amyopathic ant...
Source: Respirology Case Reports - Category: Respiratory Medicine Authors: Tags: CASE REPORT Source Type: research