Age: 46.0
Sex: M
Vaccine: COVID19, COVID19
Symptoms: Mixed connective tissue disease, Nausea, Overlap syndrome, Pleural effusion, Pyrexia
Clinical narrative: 46 yo M with PMH significant for HTN who presented 2 weeks after Moderna COVID19 Vaccine with approximately one week of a febrile illness. He was found to have AKI and pancytopenia. Exhaustive infections work-up negative. Autoimmune work up positive for multiple antibodies. He was treated for possible SLE with overlap syndrome and possible HLH/MAS. He developed multisystem organ failure and died 4/29/21. Patient was a 46 y.o. male patient with a past medical history only significant for HTN on amlodipine. He was evaluated by his PCP on 3/18/2021 for routine follow-up of HTN. Progress note documents that the patient was doing well; no issues. He was vaccinated with the Moderna COVID-19 vaccination 3/24/21. He presented to Hospital on 4/7/2021 with complaints of SOB, fever, fatigue, recurrent nausea with some vomiting, and poor PO intake for one week. He was found to have AKI, leukopenia, thrombocytopenia, elevated CPK and elevated AST/ALT. Initial concern for viral febrile illness, so supportive measures were instituted. His infectious work up remained negative over the next few days and kidney function improved with fluids but continued to spike fevers. His LFTs were worked up with an US that showed a possible hepatic mass that turned out to be a hemangioma on subsequent MRI abdomen. Repeat expanded infectious work up was also negative. He was maintained on Vanc/Zosyn and ID was following. Expanded work up for fungal and viral etiologies was negative. His renal function began to worsen so Nephrology was consulted and started work up for SLE given his other lab abnormalities (cytopenias, fever, weight loss, UA) with AKI. His autoimmune work up was consistent with possible SLE and some overlap syndrome. He ultimately underwent a Kidney biopsy on 4/15/21 which showed ATN, with few tubular casts, positive for myoglobin. Immunofluorescence findings suggestive of autoimmune disease, favor lupus nephritis ISN/RPS Class 1). Although the rapid renal decline in renal function could not be completely explained by Lupus Nephritis alone. He had a dialysis catheter placed 4/19/21 with initiation of HD (last session 4/23/21). Rheumatology was consulted given the likely diagnosis of SLE. They started plaquenil, high dose methylpred and gave initial dose of Benlysta. The plaquenil and tapered methylpred was continued with addition of dapsone for PJP ppx. Rheumatology also obtained a muscle biopsy of the right quadriceps muscle to evaluate for a myositis given the CPK elevation (type 2 fiber atrophy: nonspecific myopathic changes; results were not available until after death). Pt was started on Rituximab for myositis with biopsy pending. There was a work up into the monoclonal gammopathy on serum IFX which culminated in a bone marrow biopsy looking for MM. Oncology was consulted for his cytopenias which were felt to be related to underlying rheumatologic/autoimmune condition and expected to improve on appropriate immunosuppressive medications. These counts have been roughly stable requiring 1 unit platelets and 1 unit PRBCs throughout admission. Bone marrow biopsy result showed normocellular marrow with focal hemophagocytosis identified. Soluble CD25 (1623-4720.5) and NK cell activity (cancelled by lab) labs sent. Oncology stated that HLH should be on the differential as he fulfilled several of the criteria (H score 175 fever, 5/9 clinical criteria: hypertriglyceridemia, cytopenias, elevated ferritin, and focal hemophagocytosis on bone marrow biopsy). At this point his mental status started to decline significantly and was having some muscular rigidity with dysautonomia. Neurology was consulted and felt that this was all due to his multisystem dysfunction. Given the progressive clinical deterioration despite several immunosuppressive therapies to treat the underlying autoimmune condition, it was recommended he be transferred to hospital. He was transferred to hospital on 4/23/21 for further management until a bed was available at hospital. Upon arrival to hospital he was grossly encephalopathic but otherwise stable. Petechiae and bullae noted on bilateral lower extremities. He was transferred to hospital 4/24/21 for ongoing management of autoimmune condition, renal dysfunction, encephalopathy and possible diagnosis of HLH/MAS. His mental status initially improved with treatment of HLH and lupus and he was transferred out of the ICU. However, he was transferred back to the ICU for respiratory failure. He developed hypotension requiring levophed, then went into asystole. CPR initiated, but there was no ROSC and patient died 4/29/21.