Pulmoner Hemoraji ile Gelen Granülomatöz Polianjitis Tanısı Alan Hastanın Yoğun Bakım Yönetimi
Özet
Amaç: Böbrek ve akciğer tutulumu ile seyreden ve pulmonorenal sendrom olarak tanımlanan hastalıklar sıklıkla altta yatan romatolojik patolojilerle ilişkilidir. En yaygın neden Goodpasture sendromu olmakla birlikte sistemik lupus eritematozus (SLE), granülomatöz polianjitis (GPA), eozinofilik granülomatöz polianjitis (EGPA) ve mikroskopik polianjitis (MPA) gibi daha nadir vaskülitler de sorumlu olabilir. ANCA ilişkili vaskülitler arasında en sık görüleni GPA’dır. Bu olgu sunumunda, GPA tanısı alan ve yoğun bakım yönetimi gerektiren bir hastanın klinik seyri ve tedavi yaklaşımı aktarılmaktadır.
Olgu: Romatoid artrit öyküsü olan 50 yaşındaki kadın hasta, akut böbrek yetmezliği nedeniyle izlemde iken ani gelişen nefes darlığı sonucu yoğun bakım ünitesine kabul edildi. Romatolojik testler ve böbrek biyopsisi ile GPA tanısı doğrulandı. Tip 1 solunum yetmezliği gelişmesi üzerine entübe edilen hastaya pulse metilprednizolon, siklofosfamid ve plazmaferez tedavileri başlandı. Tedavi sonrası oksijen gereksinimi ve solunum desteği kademeli olarak azalan hasta ekstübe edilerek yeniden nefroloji servisine devredildi.
Sonuç: Nadir görülen bir hastalık olan GPA, her olguda hematüri ve hemoptizi gibi tipik semptomlarla seyretmeyebilir. Atipik klinik bulgularla başvuran ve romatolojik test pozitifliği saptanan hastalarda erken tanı ve uygun tedavi yaklaşımları mortalite ve morbiditeyi belirgin ölçüde azaltabilir.
Objective: Pulmonary–renal syndrome, characterized by concurrent renal and pulmonary involvement, is frequently associated with underlying rheumatologic disorders. While Goodpasture’s syndrome is the most common cause, systemic lupus erythematosus (SLE), granulomatosis with polyangiitis (GPA), eosinophilic granulomatosis with polyangiitis (EGPA), and microscopic polyangiitis (MPA) represent additional, albeit less common, etiologies. Among antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides, GPA is the most prevalent. This report presents the clinical course and intensive care management of a patient diagnosed with GPA.
Case Report: A 50-year-old female patient with a history of rheumatoid arthritis was admitted to the intensive care unit following the acute onset of dyspnea during hospitalization for acute kidney injury. Rheumatologic serology and renal biopsy confirmed the diagnosis of GPA. The patient developed type 1 respiratory failure and was subsequently intubated. Pulse methylprednisolone, cyclophosphamide, and plasmapheresis were initiated. Following a favorable clinical response with reduced oxygen requirements and ventilatory support, the patient was extubated and transferred back to the nephrology service.
Conclusion: GPA, a rare clinical entity, may present with atypical manifestations rather than the classic symptoms of hematuria and hemoptysis. Prompt recognition and early initiation of appropriate immunosuppressive therapy in patients with atypical presentations and positive rheumatologic serology are critical for improving clinical outcomes.
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