2019 Research Forum

Applicant: Maryam Talai-Shahir MD R3 Principal Investigator & Faculty Sponsor: Arash Heidari MD

Granulomatosis with Polyangitis Presenting as New Onset Scleritis and Hemoptysis

Maryam Talai-Shahir MD R3, Arash Heidari MD, Mandakini Patel MD R3, Carlos D’Assumpcao MD R1, Augustine Munoz MD, Bao Quynh Huynh MD, Everardo Cobos MD

INTRODUCTION ANCA Associated Vasculitis is a rare group of disorders characterized by small-medium vessel inflammation leading to occlusion, ischemia and organ dysfunction. Varied clinical presentations can be observed such as scleritis, hemoptysis, dyspnea, pulmonary nodules with cavitation, and renal impairment to failure. These varied clinical presentations can mimic other common disorders making diagnosis difficult. CASE PRESENTATION A 41 year-old male presented with fever, productive cough, malaise and dyspnea progressively worsening over three weeks. He also endorsed new onset hemoptysis, pleuritic chest pain and bilateral ocular erythema with pain for three days. He was afebrile, tachypnic with pulse oxygen 90% on room air and noted to have bilateral erythematous scleral injection with associated pain. Initial laboratory studies showed leukocytosis with left shift and renal impairment. Sputum cultures were collected and empiric treatment for presumptive pneumonia with antibiotics and antifungal therapy as coccidioidomycosis infection is endemic to the region was initiated. As the patient continued to have no clinical improvement and infectious etiology was ruled out this patient was initiated on systemic steroid for possible auto-immune etiology. Auto-immune laboratory studies all returned negative except for Anti-myeloperoxidase antibody diagnosing the patient with Granulomatosis with Polyangitis. DISCUSSION As inflammation and varied multi-organ dysfunction occurs in patients with ANCA associated Vasculitis, diagnosis and appropriate treatment can be difficult. As this patient resided in Kern County, known to be an endemic region for Coccidioidomycosis that presents with similar initial clinical symptoms and findings, he was presumed to have Pulmonary Coccidioidomycosis and initiated on treatment. With no improvement in symptoms broadening the differential diagnosis led to appropriate diagnosis and treatment. CONCLUSION To prevent premature exclusion of disorders thought to rarely occur in a certain region, a broad differential diagnosis should be taken into consideration and re-addressed with each treatment and clinical change observed. Also as patients with Vasculitis become immune-compromised underlying otherwise subclinical diseases can evolve to severe diseases. A Case report noted underlying diagnosis of Vasculitis leading to disseminate coccidioidomycosis. Thus it is important to consider multiple etiologies of underlying clinical findings.

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