Autoimmune pancreatitis in the setting of multifocal Castleman disease in an HIV-negative, HHV-8-negative, 70-year-old man.

Castleman disease (CD), also known as angiofollicular lymph node hyperplasia and giant lymph node hyperplasia, was first described in 1956 by Dr. Benjamin Castleman as localized mediastinal lymph node proliferation. Nodal hyperplasia has been described as hyaline vascular (80–90%), plasma cell (10–20%), and mixed.1 CD is a rare disease with no racial or sex predominance.2 Clinical characteristics of patients with CD can range from no symptoms in unifocal cases to systemic complaints, including autoimmune sequelae in multifocal presentations.2,3 Development of multifocal CD most commonly occurs in patients with a documented human herpesvirus 8 (HHV-8) infection. Multifocal CD has been described in patients with other immunosuppressive conditions, such as human immunodeficiency virus (HIV), lymphomas, and autoimmune diseases.2 Laboratory findings include anemia, thrombocytopenia, elevated erythrocyte sedimentation rate (ESR), elevated C-reactive protein (CRP), increased liver enzymes, hypoalbuminemia, and polyclonal hypergammaglobulinemia.2,4 There have been numerous reports of both unifocal and multifocal CD in the pancreas.3,5-7 Most commonly, CD produces masses in the head of the pancreas.3,5-8 Biopsy of these pancreatic masses have demonstrated lymphoplasmacytic tissue with evidence of sclerosis. This pathology is often found in autoimmune pancreatitis (AIP).5 AIP is most commonly seen in men during their sixth and seventh decades and among patients of Asian descent.9 Presenting symptoms of AIP include obstructive jaundice with or without abdominal pain, mild