[Cardiac sarcoidosis: Diagnosis and therapeutic challenges]. / Sarcoïdose cardiaque : avancées diagnostiques et thérapeutiques.

Sarcoidosis is a granulomatous disorder of unknown cause characterized by non-caseating granuloma in young adults. Cardiac involvement is rare and range from 2 to 75% depending on diagnostic criteria. Cardiac involvement in sarcoidosis may be asymptomatic or may manifest as rhythm/conduction troubles or congestive heart failure. The diagnosis and treatment of cardiac sarcoidosis may be challenging. However, advances have come in recent years from the use of cardiac MRI and 18FDG-TEP scanner, as well as from the stratification of the risk of ventricular tachycardia/fibrillation. Due to the rarity of the disease, there is no reliable prospective large study to guide therapeutic strategy for cardiac sarcoidosis. Corticosteroids are probably efficacious, in particular in case of atrio-ventricular block or moderate heart failure. Immunosuppressive drugs have not been largely studied but methotrexate could be helpful. In refractory forms, TNF-α antagonists have been used with success.

[Neurosarcoidosis: Diagnosis and therapeutic issues]. / Les atteintes neurologiques au cours de la sarcoïdose : diagnostic et traitement.

Neurological localizations of sarcoidosis are heterogeneous and may affect virtually every part of the central or peripheral nervous system. They are often the inaugural manifestation of sarcoidosis. The diagnosis may be difficult due to the lack of extra-neurological localization. Diagnosis may be discussed in the presence of an inflammatory neurological disease, in particular in case of suggestive radiological or biological pattern. Cerebrospinal fluid analysis shows lymphocytic pleiocytosis, often with low glucose level. The diagnosis relies on a clinical, biological and radiological presentation consistent with neurosarcoidosis, the presence of non-caseating granuloma and exclusion of differential diagnoses. Screening for other localizations of sarcoidosis, in particular cardiac disease may be obtained during neurosarcoidosis. The treatment of neurosarcoidosis relies on corticosteroids although immunosuppressive drugs are usually added because of the chronic course of this condition and to limit the side effects of steroids. Treatments and follow-up may be prolonged because of the high rate of relapses.

[The pulmonary-renal syndrome: a diagnostic and therapeutic emergency for the internist and the intensivist]. / Syndrome pneumo-rénal : une urgence diagnostique et thérapeutique pour le réanimateur et l'interniste.

The pulmonary-renal syndrome is a rare and life-threatening condition. It is defined as the association of a diffuse alveolar hemorrhage and a rapidly progressive glomerulonephritis. The characteristic histological lesion common to all underlying diseases is a necrotizing and crescentic glomerulonephritis. The pulmonary-renal syndrome is a diagnostic and therapeutic emergency: any delay in its management will lead to death or serious functional damage as pulmonary and renal impairment. ANCA-associated vasculitis and Goodpasture's disease are the main disorders associated to pulmonary-renal syndrome. More rarely systemic lupus, cryoglobulinaemia, Henoch-Schonlein purpura or subacute endocarditis may induce a pulmonary-renal syndrome. Differential diagnosis can sometimes be difficult, highlighting some ambiguity in the definition of the syndrome. Initial treatment usually associates systemic corticosteroid, cyclophosphamide and plasma exchange. The role of biotherapy as first line therapy remains to be determined.

[Hemophagocytic lymphohistiocytosis]. / Le syndrome d'activation lymphohistiocytaire de l'adulte.

Hemophagocytic lymphohistiocytosis is a life-threatening condition associated with multiple organ dysfunctions. This entity is related to inappropriate stimulation and proliferation of cytotoxic lymphocytes and macrophages inducing phagocytosis of blood cells. Hemophagocytic lymphohistiocytosis should be considered rapidly in any unexplained febrile cytopenia. Biological markers are high ferritin and triglyceride levels, and low fibrinogen. Hemophagocytic lymphohistiocytosis diagnosis should not be ruled on or out solely on the presence or absence of hemophagocytosis features on smear or biopsy sampling. It is either "primary/genetic" (pediatric or familial disorders) and characterized by a lack of intrinsic cytotoxicity of NK cells or T CD8 lymphocyte, or "secondary/reactive" due to malignancy, infectious or autoimmune origin. Mortality is 50% (including all etiologies), and this severity requires rapid and "aggressive" investigations with multidisciplinary approach including intensive care unit team. The immediate aim of therapy is suppression of the severe hyper-inflammation, which can lead to multiple organ failure. Emergency treatment is currently based on etoposide (VP16), pending to the identification and treatment of underlying cause.