Connective tissue diseases (CTD) encompass entities such as systemic lupus erythematosus (SLE), primary Sjögren's syndrome (pSS), systemic sclerosis (SSc), idiopathic inflammatory myopathies (IIM), rheumatoid arthritis (RA) and mixed connective tissue disorders (MCTD) [1]. These are relatively rare diseases that are sometimes difficult to diagnose and often require immunosuppressive therapy [1,2]. Patients with CTD can present to the intensive care unit (ICU) for various reasons including acute features that reflect the activity of the disease (“flare-up”), a comorbid condition, and/or complications resulting from its treatment [3–5].
