Saudi Journal of Kidney Diseases and Transplantation

CASE REPORT
Year
: 2010  |  Volume : 21  |  Issue : 2  |  Page : 332--336

Dilated cardiomyopathy in a patient with antibody-negative Goodpasture俟Q製 syndrome and pulmonary relapse


Yalcin Solak1, Nedim Yilmaz Selcuk1, Ilker Polat2, Huseyin Atalay1, Kultigin Turkmen1 
1 Department of Nephrology, Selcuk University, Meram School of Medicine, Meram, Konya, Turkey
2 Department of Internal Medicine, Selcuk University, Meram School of Medicine, Meram, Konya, Turkey

Correspondence Address:
Yalcin Solak
Department of Nephrology, Selcuk University, Meram School of Medicine, Meram, Konya
Turkey

The Goodpasture俟Q製 syndrome, also konwn 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要egative 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 Univer貞ity 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.


How to cite this article:
Solak Y, Selcuk NY, Polat I, Atalay H, Turkmen K. Dilated cardiomyopathy in a patient with antibody-negative Goodpasture's syndrome and pulmonary relapse.Saudi J Kidney Dis Transpl 2010;21:332-336


How to cite this URL:
Solak Y, Selcuk NY, Polat I, Atalay H, Turkmen K. Dilated cardiomyopathy in a patient with antibody-negative Goodpasture's syndrome and pulmonary relapse. Saudi J Kidney Dis Transpl [serial online] 2010 [cited 2020 Oct 1 ];21:332-336
Available from: http://www.sjkdt.org/article.asp?issn=1319-2442;year=2010;volume=21;issue=2;spage=332;epage=336;aulast=Solak;type=0