La Revue de médecine interne
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Infections are responsible for a part of the overall mortality in primary Sjögren's syndrome patients (pSS). Our retrospective monocentric study aimed at describing infections reported in a population of pSS hospitalized patients, along with the characteristics of their disease. ⋯ The impact of HCQ exposure on infectious risk needs further investigations. Broad vaccination campaign and tight control of sicca syndrome could lead to a better control of infection risk.
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Castleman disease is a group of rare disorders characterized by lymph node enlargement, specific microscopic changes to the lymph nodes, and a broad range of symptoms and laboratory findings. The two main subtypes are unicentric Castleman disease (UCD) and multicentric Castleman disease (MCD). The multicentric subtype can be further classified into two categories: HHV-8 positive multicentric Castleman disease and idiopathic multicentric Castleman disease (iMCD). ⋯ Approximately 75 percent were estimated to be unicentric CD and the remaining 25 percent were estimated to be split between HHV-8-associated MCD or HHV-8-negative/idiopathic MCD. Diagnostic criteria for iMCD have been established by an international working group of pediatric and adult pathology and clinical experts. The proposed consensus criteria require characteristic histopathologic findings on lymph node biopsy, enlargement of multiple lymph node regions, the presence of multiple clinical and laboratory abnormalities, and the exclusion of infectious, malignant, and autoimmune disorders that can mimic iMCD. © 2022 Published by Elsevier Masson SAS on behalf of Société nationale française de médecine interne (SNFMI).
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Histologically, Castleman's disease associates three subtypes: 1-the vascular hyaline (HV) subtype more often seen in unicentric forms; 2-the plasmacytic (PV) subtype, more frequently associated with the HHV8+ and idiopathic multicentric form; 3-the mixed subtype associating both HV and PV aspects that may be encountered in any type of Castleman's disease. If the diagnosis of unicentric (isolated mass) and multicentric HHV8+ Castleman's disease is easy, the diagnosis of the idiopathic multicentric form remains particularly difficult because it is at the crossroads of many other pathologies (infectious, tumoral and dysimmune), making an anatomoclinical comparison necessary. The role of the pathologist, in the context of disseminated lesions (polyadenopathy and splenomegaly), is to identify lesions that may be part of Castleman's disease, to systematically perform HHV8 testing and to perform complete phenotyping associated with molecular analysis (B and T-cell clonality) in order to rule out a lymphomatous process and certain infectious etilogies. In all cases, its role will be a warning bell and the diagnosis of Castleman's disease will be retained only after a rigorous anatomic and clinical confrontation. © 2022 Published by Elsevier Masson SAS on behalf of Société nationale française de médecine interne (SNFMI).