La Revue de médecine interne
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Innate immunity, and more specifically the complement system, has arised renewed interest in the medical field in recent years. Many innovative complement-inhibiting drugs have appeared, acting at various levels of the complement cascade. These drugs have made it possible to transform poor prognosis of certain diseases. ⋯ It then discusses the diseases in which the complement is involved on the pathophysiological level. The third part details the different classes of complement inhibitors and briefly recalls the indications for which these treatments seem the most promising. Finally, we end with a discussion that highlights the different aspects and questions induced by these new treatments.
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Case Reports
Muscular pseudotumor revealing a mycobacterial granuloma after bacillus Calmette-Guérin therapy.
The Bacillus Calmette-Guérin (BCG) is a live attenuated strain of Mycobacterium bovis. Intravesical therapy with BCG has long been proved to be effective in treating early-stage bladder carcinoma. ⋯ Mycobacterial infection should be carefully screened face to a granuloma presenting as muscular pseudotumor. A history of BCG therapy, even decades earlier, enhances this hypothesis and should lead to enforce microbiological testing, especially molecular test.
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Chlorpromazine is a commonly used drug in several medical conditions associated with a wide range of side effects. Few cases of hemostatic disorder have been reported in the literature. ⋯ A 39-year-old man had previously been diagnosed with a cavernous malformation of the medulla oblongata. Chlorpromazine was started to treat persistent hiccups. Twenty days later, the patient presented hepatitis and a pruritic rash. Haemostasis tests revealed a prolonged partial thromboplastin time associated with isolated decrease of factor VIII level and anti-factor VIII antibodies. Magnetic resonance imaging revealed recent asymptomatic bleeding. Introduction of eptacog alfa and prednisone allowed clinical and biological improvement as well as a prolonged remission after 12 months of follow-up.
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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).