RESUMEN
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.
Asunto(s)
Síndrome de Activación Macrofágica/diagnóstico , Síndrome de Sjögren/complicaciones , Adulto , África del Sur del Sahara , Antineoplásicos Fitogénicos/uso terapéutico , Etopósido/uso terapéutico , Femenino , Ferritinas/sangre , Fiebre/etiología , Humanos , Hipertrigliceridemia/etiología , Síndrome de Activación Macrofágica/complicaciones , Síndrome de Activación Macrofágica/tratamiento farmacológico , FagocitosisRESUMEN
Reports of late-onset spondyloarthritis in sub-Saharan Africa are sparse. This series allows us to describe the characteristics of this disease in Senegal. This is a retrospective study conducted in the Rheumatology Department of the Dantec University Hospital (Dakar) where we reviewed records of spondyloarthritis cases. Its diagnosis met the modified ASAS and New York criteria. Late-onset was defined as after the age of 55 years. During the study period, the department managed 133 late-onset patients, or 38% of all spondyloarthritis cases (350). Age ranged from 65 to 74 years, with a mean of 66. HLA B 27 phenotyping was performed in 89 patients; 39 patients were HLA B27 positive (29%). The treatments were based on DMARDs (salazopyrin and methotrexate).