Idiopathic multicentric Castleman disease and associated autoimmune and autoinflammatory conditions: practical guidance for diagnosis

Abstract

Idiopathic multicentric Castleman disease (iMCD) is an infrequent and life-threatening disorder characterised by systemic inflammatory symptoms, generalised lymphadenopathy, polyclonal lymphocyte proliferation, and organ dysfunction caused by a hyperinflammatory state. It accounts for one third to one half of all multicentric Castleman disease (MCD) cases. iMCD is often associated with autoimmune manifestations that may precede the iMCD diagnosis, be identified at the same time or follow it. In addition, iMCD may also coincide with a number of autoimmune diseases (such as psoriasis or myasthenia gravis) or autoinflammatory diseases (such as familial Mediterranean fever). Moreover, diverse inflammatory disorders, such as rheumatoid arthritis, systemic lupus erythematosus, adult-onset Still disease, systemic juvenile idiopathic arthritis, immunoglobulin (IgG4) related disease, or the recently described VEXAS syndrome, can present clinical features or lymphadenopathy with histopathological “Castleman-like” findings compatible with those of iMCD. Given the iMCD clinical heterogeneity and the overlap with other autoimmune or autoinflammatory disorders, iMCD diagnosis can be challenging. In this review, we explore the overlap between iMCD and inflammatory diseases and provide practical guidance on iMCD diagnosis in order to avoid misdiagnosis and confusion with other autoimmune or autoinflammatory conditions.

Rituximab as a First-line Treatment for Autoimmune Hemolytic Anemia in Multicentric Castleman's Disease

“…Arthritis, proteinuria, Sicca syndrome, polyneuropathy (without diagnosing POEMS (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal gammopathy, and Skin abnormalities) syndrome), interstitial lung disease, immune thrombocytopenic purpura, and AIHA are all autoimmune characteristics of MCD. Comorbid autoimmune diseases in CD include myasthenia gravis, psoriasis, amyloidosis, sarcoidosis, and others [ 4 ]. Liu et al discovered that among 1923 CD cases, 38 exhibited autoantibodies indicative of AIHA [ 2 ].…”

Unicentric Castleman’s disease in the parotid gland associated with psoriasis: a case report

“…In recent years, Castleman’s disease was often found to be complicated with autoimmune diseases (AID). However, the association between these two diseases was not fully illustrated due to limited studies [ 9 ]. It was supposed that the emergence of CD may be related to prolonged use of immunomodulatory therapy for the treatment of psoriasis [ 3 ].…”

Castleman Disease in an Older Patient With the Onset of Right Pleural Effusion

“…CD is prevalent around the age of 50 years; an older patient with CD is relatively atypical, like in this case [ 7 ]. This emphasizes the importance of considering CD in the differential diagnosis of elderly patients with pleural effusion and systemic symptoms, although its prevalence in this age group is low.…”