Thrombotic microangiopathy complicating newly diagnosed Sjögren's syndrome in a dialysis patient.
Ren Fail
; 36(7): 1162-5, 2014 Aug.
Article
de En
| MEDLINE
| ID: mdl-24828887
ABSTRACT
Thrombotic microangiopathy (TMA) is rarely associated with Sjögren's syndrome (SS). This is the first documented case of a patient undergoing chronic hemodialysis with SS who developed TMA. TMA is an infrequent, life-threatening multisystem disorder characterized by microangiopathic hemolytic anemia and thrombocytopenia, accompanied by microvascular thrombosis that causes variable degrees of tissue ischemia and infarction. It is important to make a quick diagnosis of TMA to cure the reported case as early as possible. The patients with TMA should be diagnosed quickly, and in this case plasma exchange and corticosteroids in combination with cyclophosphamide have been associated with a recurrence free period. Cyclophosphamide has led to the development of treatment protocols using alternative immunosuppressive agents in patients with SS showing a poor response to plasmapheresis and potentially life-threatening manifestations. Further research is required to ascertain the sensitivity, specificity, efficacy, timing, cost-benefit ratio, and necessity of cyclophosphamide in the setting of TMA complicating SS.
Mots clés
Texte intégral:
1
Collection:
01-internacional
Base de données:
MEDLINE
Sujet principal:
Syndrome de Gougerot-Sjögren
/
Microangiopathies thrombotiques
/
Défaillance rénale chronique
Type d'étude:
Diagnostic_studies
/
Guideline
Limites:
Female
/
Humans
/
Middle aged
Langue:
En
Journal:
Ren Fail
Sujet du journal:
NEFROLOGIA
Année:
2014
Type de document:
Article
Pays d'affiliation:
Chine