Glucocorticoids combined with tofacitinib in the treatment of Castleman's disease: A case report

Table 2 Idiopathic multicentric Castleman's disease diagnostic criteria

Diagnostic criteria	Contents
Main diagnostic criteria	Lymph node pathology conforms to iMCD spectrum; Enlargement of lymph nodes in 2 or more lymph nodes (short axis ≥ 1 cm)
Secondary diagnostic criteria	Laboratory standards: (1) C-reactive protein > 10 mg/L. ESR > 20 mm/h (female) or 15 mm/h (male); (2) Anemia (Hgb < 100 g/L). Thrombocytopenia (PLT < 100) × 109/L) or increased (PLT > 350 × 109/L); (3) Serum albumin < 35 g/L; and (4) Estimated glomerular filtration rate < 60 mL/(min· 73 m2) or proteinuria (total urinary protein > 150 mg/24 h or 100 mg/L), serum IgG > 17 g/L
	Clinical criteria: (1) Systemic symptoms: night sweats, fever (> 38 °C), weight loss (≥ 10% after 6 mo) or fatigue (affecting instrumental activities of daily living); (2) Hepatomegaly and/or splenomegaly; (3) Edema or serous effusion; and (4) Cherry or violet skin rash. Lymphocytic interstitial pneumonia
Exclusion criteria	(1) Infection-related diseases (such as EBV, HIV, tuberculosis); (2) Autoimmune diseases (such as rheumatoid arthritis, systemic lupus erythematosus, adult still disease, adolescent idiopathic arthritis, autoimmune lymphoproliferative syndrome); and (3) Neoplastic diseases (such as multiple myeloma, lymphoma, primary lymph node plasmacytoma, follicular dendritic cell sarcoma, POEMS syndrome)

Note: the diagnosis of iMCD needs to meet two main criteria and at least two secondary criteria (at least one of which is a laboratory standard) and exclude diseases that may be accompanied by pathological changes of lymph nodes similar to that in CD. EBV: Epstein-Barr virus; ESR: Erythrocyte sedimentation rate; Hgb: Hemoglobin; HIV: Human immunodeficiency virus; iMCD: Idiopathic multicentric Castleman's disease; PLT: Platelets; POEMS: Polyneuropathy, organomegaly, endocrinopathy, M-protein, skin changes.