Introduction:

Goodpasture´s syndrome is a rare autoimmune disease that is part of the lung-kidney syndrome’s sprectrum.

Objective:

To present the case of a pediatric patient with an atypical Goodpasture´s syndrome.

Case presentation:

Six years old female patient under follow-up in the Nephrology service. She was admitted when she was four years old by presenting macroscopic hematuria, respiratory symptoms and a history of high titers of anti-glomerular basement membrane antibodies. She was under research and was diagnosed with the Goodpasture´s symdrome. After being under immunosupressive treatment with cyclophosphamide and methylprednisolone followed by oral prednisone, the patient presented a decrease in the titers of anti-glomerular basement membrane antibodies, and an improvement of the respiratory symptoms; however, proteinuria kept increasing in the last months. For the diagnosis, there were taken into account the clinical manifestations of the girl, the high titers of anti-glomerular basement membrane antibodies and the confirmations of glumerulopathy by renal biopsy. The treatment was effective quickly with an inmmediate decrease of the titers of anti-glomerular basement membrane antibodies and an evident improvement of the clinical condition of the patient. This is the first pediatric case presenting Goodpasture´s syndrome that has been published in Cuba.

Conclusions:

As this is a rare entity in Pediatrics, it is required an early diagnosis and an aggressive treatment to improve the patient’s prognosis. In the follow-up are needed a prolonged pharmacological therapy, an adequate adherence to the proposed treatment and a close clinical and analytic monitoring from which will depend the progression of the lung and renal injury.

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