Triple Threat: The Importance of Diagnosing “Triple-Negative” Primary Biliary Cholangitis

A 68-year-old man with history of hypertension, gastroesophageal reflux disease, and suspected Sjögren’s syndrome was initially seen by his primary care physician for an 18-pound involuntary weight loss, night sweats, and chills. Given findings of lymphadenopathy, elevated urine protein, and urine IgG heavy chains, he was referred to our tertiary care cancer center for evaluation of possible underlying lymphoproliferative disorder. Preliminary evaluation included bone marrow biopsy, PET/CT, urine protein electrophoresis (UPEP), and immunofixation electrophoresis (IFE), all of which were normal. A rheumatologist confirmed the diagnosis of Sjögren’s syndrome based on sicca symptoms (dry mouth and dry eyes), lymphadenopathy, polyclonal gammopathy, and autoimmune panel remarkable for SSA > 8. …