Background: Hermansky-Pudlak syndrome (HPS) is an extremely rare disease with pulmonary fibrosis (PF),\noculocutaneous albinism, induced platelet dysfunction, and granulomatous colitis. Although patients with HPSassociated\nPF (HPS-PF) often receive treatment with anti-fibrotic agents, including pirfenidone, many HPS-PF cases\nare progressive. The development of pneumothorax is known to be rare in HPS-PF. Pneumothorax development is\ngenerally important for prognosis in patients with interstitial pneumonia. However, there are few reports regarding\nthe development of pneumothorax in patients with HPS-PF.........................
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