2019 Research Forum

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 Department of Medicine, Kern Medical, Bakersfield , CA, United States GRANULOMATOSIS WITH POLYANGITIS PRESENTING AS NEW ONSET SCLERITIS AND HEMOPTYSIS

INTRODUCTION

INTERVAL FINDING

IMAGING

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. 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 that prompted him to seek medical attention. On examination he was afebrile, tachypnic with pulse oxygen 90% on room air and noted to have bilateral erythematous scleral injection with associated pain . CASE PRESENTATION

The patient required initiation of Rituximab IV infusion and a prolonged course of oral steroid taper with significant clinical improvement including resolution of hemoptysis, dyspnea, scleritis, and renal function improvement. Renal Biopsy completed while the patient was on Rituximab and steroid therapy showed: •Inactive Pauci-Immune Crescentric Glomerulonephritis •Secondary Focal and Segmental Glomerulosclerosis- 10% •Inactive Rapidly progressive Glomerulosclerosis

Figure (a): MPO Pauci-immune crescentric glomerulonephritis with Mason trichrome stain showing cellular crescent and segmental fibrinoid necrosis (red)

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Figures: Portable Upright Chest X-rays ( A) On initial presentation showing numerous bilateral pulmonary nodules more so in the lower lobes . (B) After initiation of empiric antibiotics and antifungal therapy showing interval persistence of multiple pulmonary nodules with development of right-sided pulmonary infiltrates (C) After initiation of systemic steroid therapy showing resolution of previous pulmonary nodules and infiltrates.

Figure (b) : Hematoxylin and eosin stain histopathological image of rapidly progressive crescentric glomerulonephritis

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COMPUTED TOMOGRAPHY SCAN OF CHEST

CONCLUSION

As inflammation and varied multi-organ dysfunction occurs in patients with ANCA associated Vasculitis, diagnosis and appropriate treatment can be difficult. 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. 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 lead to appropriate diagnosis and treatment. 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 (2). Thus it is important to consider multiple etiologies of underlying clinical findings. REFERENCES 1. Kubaisi et al. Granulomatosis with polyangitis: an updated review of ocular disease manifestations. Intractable and Rare Disease research, 2016: 5(2): 61- 69. Doi 10.5582/irdr.2016.01014. 2. Ide L, Thibo P. A newly diagnosed Wegener's disease as the underlying cause for a disseminated coccidioidomycosis. Acta Clin Belg. 2013 May-Jun;68(3):245- 7. 3. Mohammad et al. Pulmonary involvement in ANCA-associated vasculitis from the view of the pulmonologist. Clinical and Experimental Nephrology. 2013 Oct; 17 (5): 667-671. Doi: 10.1007/s10157-012-0710-7. Eupb 2012 Nov 28. 4.Lionaki S., Boletis J. The Prevalence and Management of Pauci-Immune Glomerulonephritis and Vasculitis in Western Countries. Department of Nephrology and Transplantation Unit, Laiko Hospital, Athens, Greece. Kidney Diseases 2015;1:224-23

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. However, the patient continued to remain symptomatic with progressively worsening hemoptysis and scleritis despite treatment. Bronchoscopy completed showed hemorrhage in the right upper lobe with alveolar infiltrates bilaterally. AFB smears, Coccidioidomycosis serology, sputum cultures returned negative. As the patient continued to have no clinical improvement and infectious etiology was ruled out patient was initiated on systemic steroid for possible auto-immune etiology.

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Auto-immune laboratory studies all returned negative except for Anti-myeloperoxidase antibody diagnosing the patient with Granulomatosis with Polyangitis

Figure (1-4): CT Chest with IV Contrast - Serial Coronal images through the lungs revealing numerous pulmonary nodules with the largest in the left lower lobe posteriorly shown in image 4.

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