Multicentric Castleman disease with infiltration of eosinophils to the lung

• Published in: Respiratory medicine case reports 2021-01, Vol.34, p.101458, Article 101458
• Main Authors: Nakai, Shogo, Murano, Moeko, Hiramatsu, Toshiya, Matsushima, Sayomi, Uto, Tomohiro, Sato, Jun, Imokawa, Shiro, Suda, Takafumi
• Format: Article
• Language: English
• Subjects: Case Report; IgG4-related disease; Interleukin-6; Myeloperoxidase-anti-neutrophil cytoplasmic antibody
• ISSN: 2213-0071; 2213-0071
• DOI: 10.1016/j.rmcr.2021.101458

A 41-year-old man presented with multiple superficial lymph nodes (LNs) swollen with elevated levels of serum immunoglobulin (Ig)G4 and C-reactive protein. Histological findings of his left inguinal LN revealed lymphoplasmacytic infiltration with numerous IgG4-positive plasma cells; IgG4+/IgG+ plasma cell ratio >40%. Chest computed tomography (CT) showed poorly defined centrilobular nodules, interlobular septal thickening, consolidations, and mediastinal LNs swelling. Bronchoalveolar lavage fluid (BALF) showed elevated eosinophils. A surgical lung biopsy showed focal dense eosinophil infiltration, in addition to lymphoplasmacytic infiltration, but few IgG4+ plasma cells. The diagnosis of multicentric Castleman disease (MCD) was made because of serum interleukin-6elevation. Treatment with prednisolone and tocilizumab improved his symptoms and lung lesions. This case shows that overlapping clinical and pathological features of MCD and IgG4-related disease may present in a single patient, showing the difficulty in distinguishing between these two diseases.