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A3408 - Microscopic Polyangiitis Complicated Due to Mucormycosis
Author Block: S. Pourshahid, R. Lopez, D. Genin; Internal Medince, Icahn School of Medicine at Mount Sinai/Queens Hospital Center, Jamaica, NY, United States.
INTRODUCTION
Complications due to fungal infections have been described as a consequence of high dose immunosuppression. We describe a case of MPA vasculitis with positive ANCA and MPO serologies complicated by disseminated mucormycosis. No extensive literature has described thrombotic arteritis associated with mucormycosis and ANCA positivity (Ermak D, 2014).
CASE PRESENTATION
46 Y/O Male with PMHX of HTN, HLD and DM2 presented with shortness of breath and dry cough for 1 week. Examination revealed tachypnea, oxygen saturation of 68% on room air, diffuse bilateral crackles and expiratory wheezing. Labs significant for WBC of 24.2, BUN/Cr 93/9.30 (2 months prior 25/2.53). Imaging showed diffuse bilateral opacities. The patient was intubated and noted to have bloody secretions. On MICU day 2, bronchoscopy performed and BAL suggestive of alveolar hemorrhage. Serum labs showed P-ANCA Ab positive 1:160, Myeloperoxidase Ab positive 59.5, RF 15.4, CRP 268.3 and ESR 85, consistent with Microscopic Polyangiitis (MPA). Significant clinical improvement was observed with pulse steroids, plasmapheresis, Cytoxan, and hemodialysis. The patient was extubated on MICU day 8. On MICU day 15, patient developed severe abdominal pain with guarding on exam. CT abdomen showed pneumatosis of cecum with pneumoperitoneum. Exploratory laparotomy revealed necrotic colon from cecum up to hepatic flexure. Pathology revealed multiple foci of transmural ulceration with hyphae fungal organisms with fungal vasculitis, consistent with mucormycosis with no evidence of non-fungi vasculitis. Despite aggressive treatment, the patient suffered cardiac arrest and expired.
DISCUSSION
The above case illustrates the inherit risks with high dose immunosuppression to treat severe vasculitis. Although mucormycosis can cause thrombotic microangiopathy, and is a possible mechanism for pulmonary renal syndrome, there are very few reports of mucormycosis presenting in this way. Primary mucormycosis can’t explain the clinical response to immunosuppression in the first 10 days, however, high dose immunosuppression can mask any inflammation. In addition, the consistently high titers of P-ANCA and anti MPO antibodies makes false positive unlikely. Thus, MPA responding to treatment and disseminated mucormycosis was a complication of immunosuppression is more likely in this case. Given his history of diabetes, epistaxis, and construction work it is possible that patient had chronic colonization of mucormycosis, which became disseminated with immunosuppression.
CONCLUSION
Mucormycosis-related hospitalizations are rare, estimated as 0.12 per 10,000 discharges during January 2005 -June 2014 (Kontoyiannis D, 2016). Our case highlights the complexity of treating vasculitis and managing the possible complications from high dose immunosuppression.