| Popis: |
Introduction: Pulmonary-renal syndrome (PRS) refers to the combination of acute kidney injury (AKI) caused by rapidly progressive glomerulonephritis, and lung involvement with severe respiratory failure, which often involves diffuse alveolar haemorrhage. It implies different rare conditions such as various forms of systemic vasculitis, Goodpasteur syndrome and systemic lupus erythematosus. Nevertheless, nonspecific conditions without vasculitis can also cause PRS. Material and methods: We describe a child with unknown cause of PRS who was successfully treated with immunosuppressive therapy. Results: A 16- year-old girl presented with abdominal pain, vomiting, high fever, cervical lymphadenopathy and splenomegaly. Initially, her laboratory findings indicated systemic inflammation, with increased inflammatory markers and transaminases. Urinalysis was positive for leukocytes, erythrocytes, nitrites and proteins with active sediment and significant number of E. coli in microbiology. Subsequently, nephrotic range proteinuria and AKI were observed. Seven days later she developed dyspnoea with respiratory insufficiency necessitating mechanical ventilation. Computed lung tomography revealed ground-glass opacities and crazy paving pattern. The broad differential diagnosis of PRS was considered, with alternative complement pathway activation, negative ANCA, antiGBM and ASO titre. Renal biopsy was suggestive of possible infectious cause, while microbiology revealed high circulating EBV DNA load with positive EBV serology. Steroid pulse therapy led to prompt improvement of lung and kidney function, with further amelioration and no relapses during the six months follow-up. Conclusions: Even though PRS is mostly caused by specific well defined immunological disorders, unknown and atypical causes can also lead to this life-threatening condition. Therefore, early clinical recognition and prompt immunosuppressive treatment are of greater importance than defining the cause which might remain inconclusive long after the full remission has been achieved. |
