Dilated cardiomyopathy in a patient with antibody-negative Goodpasture's syndrome and pulmonary relapse

Abstract EN

The Goodpasture's 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 antibodynegative 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.