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Background: The aim of this study was to report difficulty in establishing a diagnosis of diffuse alveolar hemorrhage (DAH) in patients with granulomatosis with polyangiitis (GPA) and report an outcome of treatment requiring intensive care unit support. Methods: Four (3 women, 1 man, mean age 55) patients with GPA were diagnosed and treated with rescue administration of cyclophosphamide for DAH at our ICU in 2013-2018. Results: The main symptoms was dyspnea, fever, cough and chest pain. The preliminary diagnosis of hospital admission was – abscess or TBC in 1 case, generalized tumor in 1 case, bilateral pneumonia with no ATB effect in 2 cases. In all cases, we diagnosed the vasculitis when admitting to ICU with clinical features typical for DAH. 2 patients had typical ORL symptoms, 1 skin symptoms. All patients had respiratory failure, 2 corresponding with ARDS, 2 needed OTI, 2 NIV. 2 patient had renal failure requiring dialysis. All patients had high inflammatory markers, anemia, 3 patients had proteinuria. All patients were treated with cyclophosphamide, methylprednisolone, 3 patients with plasmapheresis. Follow-up was with improvement of overall status and image of CT scans in all cases. One patient died after 21months for cardiac failure, one had a long weaning period, one had recurring tracheal stenosis. Conclusions: DAH is a syndrome of heterogeneous etiology, relatively rare. Patients with DAH may be easily misdiagnosed as a pneumonia, abscesses, tumors or even TB. In our four cases, the underlying disease was vasculitis. The prognoses of the patients depend on the fast diagnose establishment and quickness of suitable treatment administered. |
