Résumé
Systemic lupus erythematosus (SLE) is an autoimmune disease characterized by the production of diverse autoantibodies with various systemic organ involvements. In patients with SLE, autoantibodies, such as antinuclear antibody (ANA) and anti-dsDNA antibody, play an important role not only in diagnosing the disease, but also representing the pathogenesis of the disease. ANA is the main screening tool in diagnosis and serum complement levels and anti-dsDNA antibody level are closely related to the disease activities. Nevertheless, exceptionally, some patients represent with negative ANA and/or anti-dsDNA antibody leading to difficulties in diagnosing the disease. Here, we report a case of 37-year old female SLE patient with negative ANA, negative anti-dsDNA antibody, and positive anti-Ro/SSA antibody, which manifested with nephrotic syndrome.
Sujets)
Femelle , Humains , Anticorps antinucléaires , Autoanticorps , Maladies auto-immunes , Protéines du système du complément , Glomérulonéphrite extra-membraneuse , Lupus érythémateux disséminé , Dépistage de masse , Syndrome néphrotiqueRésumé
Neonatal lupus erythematosus (NLE) is an uncommon passive autoimmune disease in which there is a transplacental passage of anti-Ro or anti-La maternal autoantibodies. The cutaneous lupus lesions are commonly observed and usually resolve spontaneously. The cardiac involvement which sometimes needs the permanent pacemaker, however, is the most clinically significant manifestation of NLE. In addition, the hepatic and hematologic abnormalities are observed in approximately 10% of infants with NLE. Therefore, in the way of the evaluation of hematologic disorders in neonate, NLE should be included. We present a case of NLE in an infant born to a mother with anti-Ro, and he had skin rash, pancytopenia and mildly abnormal liver functions without cardiac manifestation.