Dilated cardiomyopathy in a patient with antibody-negative Goodpasture's syndrome and pulmonary relapse.
The Goodpasture's syndrome, also known as anti-GBM disease, is an uncommon disease, responsible for 20% of all cases of rapidly progressive glomerulonephritis. Anti-GBM antibodies are directed against non-collagenous domain (NC-1) of the alpha-3 chain of type IV collagen. When conventional ELISA assays are used, these antibodies can be detected in almost all the patients. Nevertheless, some reports have described antibody-negative relapsing disease. Some aggravating factors, namely, smoking, pulmonary infection and hypervolemia, may expose embedded antigenic target and may be responsible for the relapse. In addition, these antibody-negative relapses also respond to standard treatment, which comprises of plasma exchange, pulse steroids and cyclophosphamide. Herein, we report a patient who presented at the Selcuk University Meram School of Medicine, Meram, Konya, Turkey, with the pulmonary-renal syndrome. He was also found to have idiopathic dilated cardiomyopathy (DCM). To our knowledge, this is the first report describing co-existence of DCM and anti-GBM disease. There is growing evidence showing strong relation of both DCM and anti-GBM disease with HLA. Although not proven, this might have occurred in our patient. In our opinion, volume overload was facilitated by anuria and DCM and led to an antibody-negative pulmonary relapse. The relapse was treated just as the first episode and the patient improved satisfactorily.
Department of Nephrology, Selcuk University, Meram School of Medicine, Meram, Konya, Turkey. Yalcinsolakmd@gmail.com
Yalcin
Y
Solak
Nedim Yilmaz
NY
Selcuk
Ilker
I
Polat
Huseyin
H
Atalay
Kultigin
K
Turkmen
D000293:Adolescent; D019867:Anti-Glomerular Basement Membrane Disease / Q000150:complications* / Q000000981:diagnostic imaging / Q000276:immunology / Q000628:therapy; D001323:Autoantibodies / Q000097:blood*; D002311:Cardiomyopathy, Dilated / Q000150:complications* / Q000175:diagnosis; D050533:Glomerular Basement Membrane / Q000276:immunology*; D006801:Humans; D007166:Immunosuppressive Agents / Q000008:administration & dosage; D008171:Lung Diseases / Q000000981:diagnostic imaging / Q000209:etiology* / Q000276:immunology / Q000628:therapy; D008297:Male; D010951:Plasma Exchange; D020551:Pulse Therapy, Drug; D012008:Recurrence; D013256:Steroids / Q000008:administration & dosage; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome
D001323:Autoantibodies; D007166:Immunosuppressive Agents; D013256:Steroids; C051951:antiglomerular basement membrane antibody