Introduction:
Pulmonary renal syndrome is associated with vasculitis, Lupus nephritis and anti GBM disease. It usually presents with rapidly progressive glomerulonephritis and pulmonary hemorrhages. It is important to diagnose this at earliest as delay in treatment is associated with irreversible damage to kidneys and high mortality due to pulmonary hemorrhages. There are rare case report of pulmonary hemorrhage with Ig A nephropathy. Therefore we report this case where a young female with Ig A nephropathy developed pulmonary hemorrhages.
Methods:
A teen age girl presented with fatigue and nausea. She was diagnosed with renal failure, anemia and hypertension at other hospital and she was referred to our tertiary care center. She had no earlier history suggestive of chronic kidney disease and connective tissue disorder. She was investigated with this background.
Results:
Eighteen years old girl came with advance renal failure. She had Blood urea and s. creatinine of 182 mg% and 7.4 mg%. She serum sodium, potassium, calcium and phosphorus were 138 meq/l,5.6 meq/l, 8.6 mg% and 6.3 mg%. Her hemoglobin was 9.8 gm % and TLC was 6700. Her urine showed protein of ++ and 30- 35 red blood cell per high power field. Her BP was 150 /92 mm Hg. She had 24 hour urine protein excretion of 1.8 gram. Ultrasound abdomen revealed kidney size of 9.1 (right) and 9.3 cm (left). Chest skiagram was normal. She was worked up as a case of rapidly progressive glomerulonephritis because she had normal size kidneys and active urinary sediment. Anti-nuclear antibodies and anti neutrophil cytoplasmic antibodies were negative. Her anti GBM antibodies titre was also negative. She was started on tablet amlodepin 5 mg , Injection erythropoietin 10000 u s/c weekly and other supportive treatment. She was also started on hemodialysis as her investigations revealed advance renal failure and metabolic acidosis. She received 3 dialysis sessions. She also received 1 unit of packed RBC infusion for optimization. She underwent percutaneous ultrasound guided renal biopsy. Her clinical course remained uncomplicated after renal biopsy. She developed sudden onset of dyspnoea 3 after biopsy. She was taken for urgent hemodialysis with good amount of ultrafiltration but she did show much improvement. She was put on CPAP and Chest Xray was obtained. It showed bilateral fluffy shadows. Her TLC was 8500 and hemoglobin was 9.7 gm%. An echocardiogram was also done which was normal. She also underwent carbon mono oxide transfer factor (DLCO) study. It showed increased diffusion capacity for carbon mono oxide which was suggestive of pulmonary hemorrhage. She deteriorated and was put on ventilator support. She succumbed to illness after few hours. Her renal biopsy report showed Ig A nephropathy with significant interstitial fibrosis and no crescents were observed in renal histopathology.
There has been few case report of pulmonary hemorrhage with Ig A nephropathy and some of them had been described with henoch schonlein purpura. There has been suggestion of this in present case as there was no rash, arthralgia or pain abdomen.
Conclusions:
A case of suspected Ig A nephropathy rarely can develop pulmonary hemorrhage. If there is unexplained anemia or sudden development of unexplained breathlessness then there should be strong suspicion towards pulmonary renal syndrome. Early detection and timely management could improve outcome in such patients.
I have no potential conflict of interest to disclose.
I did not use generative AI and AI-assisted technologies in the writing process.