We present a rare case of PL-7 antisynthetase syndrome (ASS) in association with Sjögren’s, systemic lupus erythematosus (SLE), and seropositive rheumatoid arthritis (RA). Initially, the patient was diagnosed with Sjögren’s followed by Sjögren’s/SLE overlap and then Sjögren’s/SLE/RA overlap. She was eventually diagnosed with Sjögren’s/SLE/RA overlap with PL-7 ASS with interstitial lung disease (ILD). ILD was discovered after complaints of pleuritic chest pain with subsequent workup with coronary computed tomography (CT) revealing pulmonary fibrosis. This case demonstrates the ambiguity with which symptoms of ASS can present; given the high respiratory morbidity and mortality of ASS especially in non-Jo-1 patients, those who present with Raynaud’s, myositis, or joint pain, whether together or in isolation, should be assessed for presence of additional features of ASS and potentially undergo testing for ASS antibodies if appropriate.

We present two cases of patient’s with long-standing autoimmune diseases being treated with immunosuppressants that developed aggressive lymphoproliferative disorders. Immunosuppressants have a well-known association with disorders. Sustained regression of these lymphoproliferative disorders occurred with simple discontinuation of these immunosuppressive agents.

Objective There is thought to be an association between Hidradenitis Suppurativa (HS) and autoimmune diseases. This retrospective longitudinal cohort study looked to identify whether certain autoimmune diseases or autoantibody specificities are more closely associated with HS than others and, whether such associations are related to severity of HS. Methods Patients were identified using the SlicerDicer search tool in Epic from January 1, 2010 through August 15, 2020. Search criteria included HS diagnosis by ICD-10 code and at least one visit in dermatology. Charts were reviewed to determine HS disease severity, treatment modalities, presence of autoimmune disease, and autoantibody positivity. Results 627 patients were identified. Most patients were females (75.3%) and had obese BMIs (71.1%), but there were no significant demographic differences between HS patients with and without autoimmune diseases. 101 (16.1%) patients in the total cohort had at least one autoimmune disease, most commonly, thyroid disease, lupus, psoriasis, and inflammatory bowel disease (IBD). 212 patients were also tested for the presence of autoantibodies. The most common positive autoantibody, found in 54 patients (28.4%), was antinuclear antibody (ANA). 54 patients with more severe HS disease manifestations required biologic medications to treat their HS. Neither HS severity nor biologic treatment was associated with presence of autoimmune disease or positive autoantibodies. Conclusion In a large cohort of patients with HS followed longitudinally, autoimmune disorders (especially lupus, psoriasis and IBD) and presence of autoantibodies were more commonly observed than expected in the normal population.

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