RESUMEN
Pauci-immune glomerulonephritis in systemic vasculitides usually have anti-neuthrophil cytoplasmic antibodies (ANCA). However, vasculitides of large vessels such as Takayasu’s and giant cell (temporal) arteritis do not. Exceptionally ANCA(+) small vessel vasculitides are associated with large vessel vasculitis. It may be a coincidence or both vasculitides have a common pathogenesis. We report a 30 years old woman on hemodialysis due to a chronic glomerulonephritis ANCA(+) diagnosed nine years ago. Eight years later, she presented with an aortitis with severe stenosis of distal aorta and vasculitis of left subclavian artery. She was treated with adrenal steroids and cyclophosphamide. During the ensuing five years she has been stable and without signs of reactivation of the disease.
Asunto(s)
Adulto , Femenino , Humanos , Vasculitis Asociada a Anticuerpos Citoplasmáticos Antineutrófilos/tratamiento farmacológico , Estenosis de la Válvula Aórtica/tratamiento farmacológico , Aortitis/tratamiento farmacológico , Ciclofosfamida/uso terapéutico , Esteroides/uso terapéutico , Vasculitis Asociada a Anticuerpos Citoplasmáticos Antineutrófilos/complicaciones , Estenosis de la Válvula Aórtica/diagnóstico , Aortitis/diagnóstico , Insuficiencia Renal/complicaciones , Arteritis de Takayasu/tratamiento farmacológicoRESUMEN
Due to its multisystem involvement, IgG4 -related disease should be considered in the differential diagnosis of medical conditions such as lymphadenopathies, aortitis, serositis and retroperitoneal fibrosis. It shares features with other entities historically described as "great mimickers" such as syphilis, tuberculosis, sarcoidosis, and systemic lupus erythematosus. We report a 40 year-old male with recurrent effusive - constrictive pericarditis, lymphadenopathy and aortitis. The study revealed an inactive tuberculosis with negative cultures for acid fast bacilli. The patient had high serum levels of IgG4 and a mediastinal lymph node biopsy was consistent with IgG4 -related disease. The patient was treated with prednisone 40 mg/day with an excellent response.
Asunto(s)
Adulto , Humanos , Masculino , Aortitis/diagnóstico , Inmunoglobulina G/sangre , Enfermedades Linfáticas/diagnóstico , Aortitis/sangre , Biopsia , Diagnóstico Diferencial , Enfermedades Linfáticas/sangre , Tomografía Computarizada por Rayos XRESUMEN
Six patients (2 male, 4 female; age range 24 to 36 years) with aortoarteritis involving the supra-aortic trunks were investigated and surgically managed by bypass graft with either preclotted dacron or polytetrafluoroethylene (PTFE) in an urban institute. There has been no mortality and post-operative functional recovery has been satisfactory in all the cases.