Macrophage activation syndrome, a rare complication of primary Sjögren’s syndrome: a case report
| العنوان: | Macrophage activation syndrome, a rare complication of primary Sjögren’s syndrome: a case report |
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| المؤلفون: | Souhaibou Ndongo, Baïdy Sy Kane, Abdoulaye Pouye, Boundia Djiba, Ngoné Diaba Diack, Maïmouna Sow, Mouhamed Dieng, Atoumane Faye, M. Niasse, Awa Cheikh Ndao, Nafissatou Diagne |
| المصدر: | Journal of Medical Case Reports, Vol 13, Iss 1, Pp 1-5 (2019) Journal of Medical Case Reports |
| بيانات النشر: | BMC, 2019. |
| سنة النشر: | 2019 |
| مصطلحات موضوعية: | Adult, medicine.medical_specialty, Fever, Anemia, lcsh:Medicine, Physical examination, Gastroenterology, 03 medical and health sciences, 0302 clinical medicine, Phagocytosis, 030202 anesthesiology, Internal medicine, Case report, medicine, Humans, Etoposide, Hypertriglyceridemia, 030203 arthritis & rheumatology, medicine.diagnostic_test, business.industry, lcsh:R, Interstitial lung disease, Hypergammaglobulinemia, General Medicine, medicine.disease, Antineoplastic Agents, Phytogenic, Sjogren's Syndrome, Africa south of the Sahara, Macrophage activation syndrome, Sjögren’s syndrome, Ferritins, Female, Polyarthritis, Hemophagocytosis, business, Complication |
| الوصف: | Background The association of macrophage activation syndrome and primary Sjögren’s syndrome has been rarely reported in the literature. We report the first observation of this association in Africa, south of the Sahara, and we discuss the diagnosis and therapeutic challenge. Case presentation A 26-year-old Mauritanian and Berber woman was followed for primary Sjögren’s syndrome. After a voluntary cessation of her usual background treatment, she was admitted to our department for an outbreak of her illness. A clinical examination revealed anemic syndrome, peripheral polyarthritis, coughing rales at both pulmonary bases, and fever at 39.5 °C. On biologic examination, there was bicytopenia with anemia at 5.70 g/dl, lymphopenia at 796/mm3, a biological inflammatory syndrome with a sedimentation rate at 137 mm in the first hour, C-reactive protein at 97 mg/l, hyperferritinemia at 1778 mg/l (9 normal value), and hypergammaglobulinemia at 20.7 g/l of polyclonal appearance. The triglycerides were 1.95 g/l (1.4 normal value) and the lactate dehydrogenase level was 491 IU/l (1.5 normal value). Cytological examination of a medullary puncture revealed an image of hemophagocytosis. An infectious screening was negative. Thoracic computed tomography showed non-specific interstitial lung disease. A diagnosis of macrophage activation syndrome complicating primary Sjögren’s syndrome was selected with a probability of 97.2%, according to H-Score. The evolution was favorable under a treatment including etoposide (VP-16). Conclusion Macrophage activation syndrome is a rare entity, rarely reported during primary Sjögren’s syndrome. Its spontaneous evolution is invariably fatal. There is no consensus on therapeutic treatment. Etoposide is a therapeutic option especially in forms refractory to corticosteroid therapy. |
| اللغة: | English |
| تدمد: | 1752-1947 |
| URL الوصول: | https://explore.openaire.eu/search/publication?articleId=doi_dedup___::2445eb49cf21b500a78843015a5c0fec http://link.springer.com/article/10.1186/s13256-019-2252-z |
| حقوق: | OPEN |
| رقم الأكسشن: | edsair.doi.dedup.....2445eb49cf21b500a78843015a5c0fec |
| قاعدة البيانات: | OpenAIRE |
Find this article in full text from Springer Verlag. | تدمد: | 17521947 |
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