SESSION TITLE: Medical Student/Resident Pulmonary Manifestations of Systemic Disease 1 SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: 10/09/2018 01:15 PM - 02:15 PM INTRODUCTION: IgG4-related disease (IgG4-RD) can involve multiple organs and mimic malignant, other autoimmune, or infectious processes. CASE PRESENTATION: A 66 year-old man with COPD chronically on 4L O2, Type 2 Diabetes, and a 30 pack-year smoking history, presented with 1 month of worsening dyspnea. Chest CT showed new pleural thickening, and an echocardiogram showed a loculated pericardial effusion. He received a course of steroids for presumed COPD exacerbation and improved. Pleural biopsy was considered but felt high risk given his poor functional status. The patient had multiple episodes of respiratory decompensation in the following months requiring hospitalization. He developed scrotal swelling, indurated skin plaques, abdominal distension and pain. Diagnostic workup included an ultrasound showing bilateral hydroceles and epididymo-orchitis. Urine gonorrhea and chlamydia PCR and culture were negative. A skin lesion was biopsied, and pathology showed panniculitis with many plasma cells. PET-CT showed activity in the pleura, pericardium, omentum, peritoneum, thoracic and abdominal lymph nodes. Abdominal CT showed carcinomatosis-like omental thickening with subcutaneous extension. Percutaneous omental biopsies were performed. Pathology showed a mixed inflammatory infiltrate and reactive changes with scattered giant cells but no malignancy. Stains for bacteria, mycobacteria, and fungi were negative. Rare plasma cells were positive for IgG4. The differential diagnosis still included carcinomatosis, IgG4-RD, and less likely disseminated infection. The consideration of an inflammatory disorder prompted empiric institution of prednisone. Serum IgG4 was elevated then at 238 mg/dL (normal 8-140 mg/dL). But after 6 months of struggling with the disease and now critically ill with decompensated respiratory and heart failure, the patient elected for hospice care. He was continued on prednisone 20mg and diuretics with modest improvement.The patient decompensated at hospice and required ICU admission. After 24 hours of IV methylprednisolone and aggressive diuresis, he transitioned to prednisone 60mg daily and standing diuresis. His abdominal pain and distension improved, as did his skin and scrotal swelling. His O2 requirement decreased from high flow at 80% FiO2 to 9L nasal cannula. After 1 month on prednisone, he had dramatic symptomatic improvement and his oxygen requirement decreased to 4L. PET-CT showed reduced thickening and activity of the pleura, pericardium, and omentum, but new lymphadenopathy. DISCUSSION: IgG4-RD can affect the pleura, pericardium, retroperitoneum, omentum, testicles, skin, and other sites. The disease typically responds well to glucocorticoids. A trial of steroids has both diagnostic and therapeutic utility when the distinction between IgG4-RD and carcinomatosis is unclear. CONCLUSIONS: Suspicion for IgG4-RD should remain high even if histopathologic criteria are not met. Reference #1: Kamisawa T, Zen Y, Pillai S, Stone JH. IgG4-related disease. Lancet. 2015;385(9976):1460-1471. DISCLOSURES: No relevant relationships by Mark Fuster, source=Web Response No relevant relationships by Matthew Light, source=Web Response No relevant relationships by Alexandra Rose, source=Web Response No relevant relationships by Cameron Wales, source=Web Response No relevant relationships by Hairan Zhu, source=Web Response
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