Un cas exceptionnel de Sarcoïdose multiviscérale

Cette thèse rapporte un cas de sarcoïdose sévère, suivi pendant 10 ans. A notre connaissance, aucun cas clinique n'a été décrit dans la littérature. Il s'agit d'une sarcoïdose multiviscérale, atteignant un nombre majeur de localisations (10 au total), dont certaines ont menacé le pronostic (atteinte neurologique et cardiaque), alors que certaines localisations dites " classiques " n'ont pas été observées comme les atteintes oculaire, articulaire, et la glandes exocrines. Au sein de chaque atteinte, certaines atypies ont été notées, telles que l'existence d'une atteinte péricardique sans atteinte myocardique, d'une atteinte pleurale alors que l'atteinte médiastino-pulmonaire est peu évolutive, d'une atteinte neurologique très polymorphe touchant l'ensemble des structures du système nerveux hormis les nerfs crâniens, alors que ceux-ci sont habituellement touchés chez les sujets de race noire. Une évolution particulièrement longue (10 ans), alors que la majorité des sarcoïdoses régresse spontanément en moins de 2 ans, est marquée par l'apparition successive de nouvelles localisations et de rechutes d'anciennes localisations malgré la corticothérapie, voire d'autres immunosuppresseurs. Une corticorésistance voire une " immunorésistance " imposant une escalade thérapeutique avec l'utilisation successive et associée de différents immunosuppresseurs. Toutes le atypies cliniques, radiologiques et évolutives ont conduit à une recherche permanente d'une autre pathologie ou de l'association de deux maladies, notamment c-tuberculose et sarcoïdose-lymphome.PARIS7-Villemin (751102101) / SudocPARIS-BIUM (751062103) / SudocSudocFranceF

Cardiac sarcoidosis: A long term follow up study.

BackgroundPrognostic factors are lacking in cardiac sarcoidosis (CS), and the effects of immunosuppressive treatments are unclear.ObjectivesTo identify prognostic factors and to assess the effects of immunosuppressive drugs on relapse risk in patients presenting with CS.MethodsFrom a cohort of 157 patients with CS with a median follow-up of 7 years, we analysed all cardiac and extra-cardiac data and treatments, and assessed relapse-free and overall survival.ResultsThe 10-year survival rate was 90% (95% CI, 84-96). Baseline factors associated with mortality were the presence of high degree atrioventricular block (HR, 5.56, 95% CI 1.7-18.2, p = 0.005), left ventricular ejection fraction below 40% (HR, 4.88, 95% CI 1.26-18.9, p = 0.022), hypertension (HR, 4.79, 95% CI 1.06-21.7, p = 0.042), abnormal pulmonary function test (HR, 3.27, 95% CI 1.07-10.0, p = 0.038), areas of late gadolinium enhancement on cardiac magnetic resonance (HR, 2.26, 95% CI 0.25-20.4, p = 0.003), and older age (HR per 10 years 1.69, 95% CI 1.13-2.52, p = 0.01). The 10-year relapse-free survival rate for cardiac relapses was 53% (95% CI, 44-63). Baseline factors that were independently associated with cardiac relapse were kidney involvement (HR, 3.35, 95% CI 1.39-8.07, p = 0.007), wall motion abnormalities (HR, 2.30, 95% CI 1.22-4.32, p = 0.010), and left heart failure (HR 2.23, 95% CI 1.12-4.45, p = 0.023). After adjustment for cardiac involvement severity, treatment with intravenous cyclophosphamide was associated with a lower risk of cardiac relapse (HR 0.16, 95% CI 0.033-0.78, p = 0.024).ConclusionsOur study identifies putative factors affecting morbidity and mortality in cardiac sarcoidosis patients. Intravenous cyclophosphamide is associated with lower relapse rates

Association of Prognostic Factors and Immunosuppressive Treatment With Long-term Outcomes in Neurosarcoidosis

International audiencePrognostic factors are lacking in neurosarcoidosis (NS), and the association of immunosuppressive treatments with outcomes are unclear

Prevalence and clinical features of COVID-19 in a large cohort of 199 patients with sarcoidosis.

International audienceNo abstract availabl

Additional file 1: of G908R NOD2 variant in a family with sarcoidosis

Contains supplementary data for the Material and Methods section. (DOC 64 kb

Osseous sarcoidosis: a multicenter case-control study

International audienceObjective: To describe the clinical presentation, distribution of lesions, treatment, and outcomes of osseous sarcoidosis.Methods: A French retrospective multicenter study of patients with biopsy-proven sarcoidosis analyzed patients with 1) a biopsy-proven granuloma without caseous necrosis, and either 2) osseous clinical manifestations, or 3) abnormal osseous imaging. Sarcoidosis patients with osseous involvement (cases) were compared with 264 age- and sex-matched sarcoidosis patients with no osseous manifestations (controls).Results: In the osseous sarcoidosis group (n = 88), forty-two (48%) patients had osseous-related symptoms involving the axial (69%) and/or appendicular (58%) skeleton. On imaging, the most commonly affected bones were in the spine (52%), pelvis (42%), hands (22%) and femur (19%). Compared with controls, cases had higher rates of mediastinal (93% vs. 47%) and extra-thoracic lymph node involvement (66% vs. 21%), pulmonary (90% vs. 65%) and cutaneous involvement (44% vs. 23%) (all P < 0.0001), and hypercalcemia (8.5% vs. 2%, P = 0.014). Spleen/liver and gastrointestinal involvement were less frequent in the osseous sarcoidosis group (29% vs. 45%, and 1% vs. 17%, respectively, P < 0.0001). Response rates to with glucocorticoids alone, glucocorticoids plus methotrexate or glucocorticoids plus hydroxychloroquine were 23/44 (52%), 9/13 (69%) and 4/6 (67%), respectively.Conclusion: In patients with osseous sarcoidosis the spine and pelvis were the most commonly affected bones. Compared with controls, cases with osseous sarcoidosis have higher rates of thoracic and extra-thoracic lymph node involvement, pulmonary and cutaneous involvement, and hypercalcemia. Most patients with osseous sarcoidosis had a good response to glucocorticoids in combination with methotrexate or hydroxychloroquine

Efficacy and Tolerance of Anti–Tumor Necrosis Factor α Agents in Cutaneous Sarcoidosis

International audienceEvidence for the long-term efficacy and safety of anti-tumor necrosis factor α agents (anti-TNF) in treating cutaneous sarcoidosis is lacking