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DISEASES FOR EXCLUSION

Table 3: Diseases that must be excluded for an iMCD diagnosis1

AUTOIMMUNE
DISEASE
MALIGNANT/
LYMPHO-PROLIFERATIVE
DISORDERS
INFECTION-RELATED
DISORDERS
Rheumatoid arthritis Lymphoma (Hodgkin and non-Hodgkin) HHV-8 infection
Systemic lupus erythematosus Multiple myeloma Clinical EBV-lymphoproliferative disorders e.g. infectious mononucleosis or chronic active EBV
Adult-onset Still disease Primary lymph node plasmacytoma Inflammation and adenopathy caused by other uncontrolled infections e.g. toxoplasmosis, HIV, active TB
Autoimmune lymphoproliferative syndrome FDC sarcoma -
Juvenile idiopathic arthritis POEMS syndrome* -

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*  MCD patients with POEMS are distinguished from iMCD because POEMS has a different natural history and therapeutic approach to iMCD.

References

  1. Fajgenbaum DC, Uldrick TS, Bagg A, Frank D, Wu D, Srkalovic G, et al. International, evidence-based consensus diagnostic criteria for HHV-8-negative/idiopathic multicentric Castleman disease. Blood. 2017;129(12):1646-57.
  2. Liu AY, Nabel CS, Finkelman BS, Ruth JR, Kurzrock R, van Rhee F, et al. Idiopathic multicentric Castleman’s disease: a systematic literature review. Lancet Haematol. 2016;3(4):e163-75.
  3. Sitenga J, Aird G, Ahmed A, Silberstein PT. Impact of siltuximab on patient-related outcomes in multicentric Castleman’s disease. Patient Relat Outcome Meas. 2018;9:35-41.
  4. Srkalovic G, Marijanovic, I., Srkalovic, M.B., Fjagenbaum, D.C.,. TAFRO syndrome: New subtype of idiopathic multicentric Castleman disease. Bosn J Basic Med Sci 2017 [Available from: http://www.bjbms.org/ojs/index.php/bjbms/article/view/1930].
  5. Nishimoto N, Kishimoto T, Yoshizaki K. Anti-interleukin 6 receptor antibody treatment in rheumatic disease. Ann Rheum Dis. 2000;59 Suppl 1:i21-7.