C41-18 Diffuse Alveolar Hemorrhage in a Patient With Cryoglobinemia

Abstract Introduction Cryoglobulinemia is a vasculitis caused by the deposition of circulating cryoglobins that precipitate at cold temperatures and redissolve upon rewarming. Manifestations include palpable purpura, livedo reticularis, arthralgias, and peripheral neuropathy. Pulmonary involvement is reported in fewer than 5% of patients. Early recognition and prompt initiation of immunotherapy is essential to prevent respiratory failure. We describe a patient with HCV-associated cryoglobulinemia who presented with diffuse alveolar hemorrhage (DAH). Case presentation A 65-year-old man with past medical history of end-stage renal disease (ESRD) on hemodialysis secondary to diabetic nephropathy and immune complex-mediated glomerulonephritis, rheumatic heart disease (with bioprosthetic mitral and aortic valves), and chronic HCV presented with acute-onset dyspnea and hemoptysis. Physical exam was remarkable for a nonblanching petechial rash. Labs revealed polyclonal hypergammaglobulinemia and elevated NT-proBNP, troponin, and procalcitonin. D-dimer was markedly elevated. Urinalysis showed proteinuria and microscopic hematuria without red blood cell casts. CT angiography ruled out pulmonary embolism, and was remarkable for bilateral pleural effusions, interstitial edema, and mediastinal lymphadenopathy without definitive radiographic evidence of DAH. Bronchoalveolar lavage was obtained and revealed characteristic blood tinged sequential samples. Cytology revealed hemosiderin-laden macrophages with 13,500 RBCs suggestive of alveolar bleeding. Infectious labs were negative. Autoimmune testing demonstrated low complement levels, elevated rheumatoid factor, and type III cryoglobulins containing polyclonal IgG. Quantitative immunoglobulins showed elevated IgA, IgG, and IgM. Proteinase 3 (PR3) antibody was mildly positive, whereas antineutrophil cytoplasmic antibody (ANCA) and anti-glomerular basement membrane (anti-GBM) antibody were negative. Serum protein electrophoresis showed a polyclonal pattern without monoclonal bands. A right lower-extremity skin biopsy of the rash demonstrated leukocytoclastic vasculitis with granular IgA and C3 deposition along small-vessel walls.He was treated with high-dose glucocorticoids, plasma exchange, and rituximab. He tolerated therapy well and demonstrated improvement in respiratory symptoms. Discussion Cryoglobulinemia presenting as DAH is associated with high morbidity and mortality. Further increasing the challenge of diagnosis, imaging findings are non-specific and may resemble infection or pulmonary edema related to cardiac dysfunction. ESRD, hypervolemia, and valvular heart disease complicated our patients’ evaluation. BAL containing hemosiderin-laden macrophages confirmed prior alveolar bleeding. Cryoglobulinemic vasculitis should be considered in patients with previous HCV infection who develop pulmonary hemorrhage, even after viral clearance. In those with ESRD or cardiac comorbidities, respiratory findings may be misattributed to volume overload or infection, delaying diagnosis. Early recognition and coordinated management across pulmonary, renal, rheumatologic, and infectious disease specialties lead to a successful recovery. This abstract is funded by: None