Smoking in young men may trigger
anti-GBM disease manifesting with
hemoptysis. We present a male adolescent in whom
hemoptysis was mistaken to be a sign of airway
infection for several months and who later on underwent an unusual antibody-negative relapse. The 16-year-old patient had a history of smoking and
therapy-refractant
hemoptysis and, later, acute macrohematuria with
renal insufficiency necessitating
hemodialysis (initial
creatinine 4.2 mg/ dl). Chest X-ray showed diffuse lung infiltration. Renal biopsy revealed linear
IgG deposits along the glomerular basement membrane (GBM) and cellular crescents in 13/16 glomeruli, simultaneously increased
anti-GBM antibodies were detected. Thus,
anti-GBM glomerulonephritis was diagnosed.
After treatment with
prednisone, oral
cyclophosphamide and
plasmapheresis, chest X-ray and
hemoptysis improved, but
renal failure persisted.
Anti-GBM antibodies were negative. 4 weeks later, the patient presented again with a clinical relapse of severe
hemoptysis and
respiratory insufficiency after
smoke exposition. Despite negative
anti-GBM antibodies, he was treated similarly to a relapse and after the second course of
plasmapheresis the patients' general condition improved and
hemoptysis subsided. During the next 10 months the patient was stable with negative
antibodies. He was under intermittent
hemodialysis until laboratory measurements showed improved renal function. Now, 30 months after the acute episode, the patient is off dialysis for 17 months with stable
creatinine values of 1.9 - 2.4 mg/dl, and is currently being treated with
antihypertensive medicaments,
calcitriol, calciumacetate, natriumhydrogencarbonate and
allopurinol. The prognosis of
anti-GBM glomerulonephritis depends on serum
creatinine and the need of dialysis at initial presentation. In these patients, one-year survival rate is 67% and 5% for kidney function. Of note, despite the unfavorable prognosis in our patient, renal function recovered after 1 year of
hemodialysis treatment. It is important to consider that in patients with
anti-GBM disease antibody-negative relapses are possible.