Idiopathic multicentric Castleman disease resembling autoimmune diseases: A case report

Castleman disease (CD) is a rare lymphoproliferative disorder characterized by nonmalignant lymph node enlargement, often associated with systemic symptoms. It is classified into unicentric disease (involving a single enlarged lymph node) and multicentric disease (affecting multiple lymph node stations). In some cases of idiopathic multicentric Castleman disease (iMCD), elevated levels of various serum inflammatory markers are observed, and histologically, the lymph node enlargement resembles that caused by autoimmune diseases, making diagnosis challenging.

A 35-year-old female patient presented with fatigue, low-grade fever, shoulder erythema, and generalized lymphadenopathy for 2 years. Persistent systemic inflammation, anemia, thrombocytosis, hypoalbuminemia, and hyperglobulinemia were noted.

Blood tests revealed systemic inflammation, including elevated levels of C-reactive protein and interleukin-6, along with increased rheumatoid factor levels. Computed tomography scans showed a large, well-defined mass with uniform enhancement in the left neck. Skin erythema pathology was suggestive of allergic purpura. Bone marrow biopsy showed increased plasma cells. Lymph node pathology revealed an increase in IgG4-positive cells, with a high number of CD38 and CD138 plasma cells, and the morphology was consistent with Castleman disease (plasmacytic type). The diagnosis was iMCD.

Intravenous tocilizumab (400 mg every 4 weeks), methylprednisolone (40 mg daily), and oral thalidomide (75 mg daily) were administered. Symptomatic treatment included intravenous albumin (10 g daily), topical application of denaseide cream (0.5 g twice daily) for rash and pruritus, oral cetirizine (10 mg daily), and oral ebastine (10 mg daily).

The patient no longer experienced low-grade fever or fatigue but continued to have lymphadenopathy and shoulder erythema.

This case highlights the rarity and uniqueness of iMCD, which can easily be confused with lymphadenopathy caused by autoimmune diseases (e.g., rheumatoid arthritis-related lymphadenopathy, IgG4-related diseases). Clinicians should consider lymph node histology in conjunction with clinical and serological findings, as well as imaging results, for accurate diagnosis.