The novel Coronavirus-2019 pandemic brings a largely unfamiliar landscape and continual quest for understanding of the spectrum of presentation for this disease. Recent studies have shown endothelial injury as the trigger for the cytokine storm seen in Covid-19 infection. Hypercoagulability and excessive complement activation can lead to a diffuse thrombotic angiopathy and organ dysfunction CASE PRESENTATION: 40 year old Hispanic male with no past medical history presented to the emergency room with weakness, AMS, recurrent hematemesis. Upon arrival, he was immediately intubated for airway protection. On exam pertinent findings included fever of 102 F, response to noxious stimuli only, and scleral icterus. Laboratory data revealed creatinine 8.08 mg/dL, LDH >2500 IU/L, haptoglobin <10 mg/dL, bilirubin 1.2 mg/dL, hemoglobin 6.8 g/dL, platelets 7,000/L with presence of 3+ schistocytes and positive test for COVID-19. PCR was negative for STEC and shigella. Given a PLASMIC score of 7, a presumptive diagnosis of thrombotic thrombocytopenic purpura (TTP) was made and daily plasma exchange (PEX) with dialysis was urgently initiated. A normal ADAMTS13 level led to discontinuation of PEX after 3 sessions; however, after 4 days hemolysis worsened with low C3 complement, therefore PEX was restarted. A complement panel was suggestive of alternative pathway dysregulation. Patient underwent 15 additional sessions of PEX with resolution of hemolysis, improved renal function and mental status.
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.
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