A Rare Case of Calcific Rheumatoid Constrictive Pericarditis Complicated by Diffuse Alveolar Hemorrhage and MPO-ANCA-Associated Systemic Vasculitis With Pauci-Immune Glomerulonephritis

Abstract

SESSION TITLE: Pulmonary Manifestations of Systemic Disease 4 SESSION TYPE: Affiliate Case Report Poster PRESENTED ON: Tuesday, October 31, 2017 at 01:30 PM - 02:30 PM INTRODUCTION: Diffuse alveolar hemorrhage(DAH) is a rare complication in patients with Rheumatoid arthritis(RA). The added calcific constrictive pericarditis, systemic vasculitis and pauci-immune glomerulonephritis(PIGN) in the same patient poses a unique diagnostic and therapeutic dilemma CASE PRESENTATION: A 60-year-old male with a history of RA, calcific constrictive pericarditis was admitted with fever, chills, dry cough and worsening exertional dyspena. He had diminished breath sounds in bilateral lower lung fields on exam. Computed tomography of the chest revealed patchy nodular infiltrate in the left lung and confluent ground glass infiltrate in the right lung with persistent pericardial calcifications. He developed worsening hemoglobin drop and severe hypoxemic respiratory failure worsening and was intubated. Bronchoscopy with sequential bronchoalveolar lavage was progressively more hemorrhagic and hemosiderin laden macrophages confirmed the diagnosis of DAH. He was started on intravenous methylprednisolone. A workup revealed high titers of antibodies (Table1). Due to severity of his illness he had serial plasmapheresis followed by treatment with intravenous cyclophosphamide. Renal biopsy showed PIGN. By hospital day 20 his respiratory and renal function improved and was successfully extubated DISCUSSION: We report for the first time to the best of our knowledge a rare case with chronic rheumatoid constrictive pericarditis complicated by DAH in the setting of MPO-ANCA associated vasculitis and PIGN. One of the rare cardiac complications of RA is constrictive pericarditis confirmed here by cardiac catheterization with equalization of diastolic pressures and discordant right and left ventricular pressures and pericardial calcifications on imaging. DAH complicates 40% of cases of MPO-ANCA vasculitis however, limited data is available in adults with concurrent RA and is associated with higher mortality. The incidence of ANCA in RA is around 40% and both p- and c-ANCA staining patterns are expressed. one study described three RA patients with renal manifestations who improved with a combination of plasmapheresis and cyclophosphamide with or without methylprednisolone such as in our patient. CONCLUSIONS: A high index of suspicion, careful analysis and early detection is essential to improve chances of recovery in these patients Reference #1: Gauhar UA et al. Pulmonary manifestations of rheumatoid arthritis.Semin RespirCrit Care Med 2007;28:430-40. Reference #2: Kurita N et al. MPO-ANCA glomerulonephritis with RA.ClinExpNephrol.2010Aug;14(4):325-32. DISCLOSURE: The following authors have nothing to disclose: Kavitha Gopalratnam, Despina Michailidou, Ashley Rosario-Durchhalter, Paul Cohen, Armand Wolff No Product/Research Disclosure Information