Autoimmune Pulmonary Alveolar Proteinosis Developed throughout Treatment for Systemic Sclerosis: A Case Report (2025) > 자유게시판

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Autoimmune Pulmonary Alveolar Proteinosis Developed throughout Treatme…

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작성자 Christena 작성일25-08-13 06:36

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In May 2023, a 72-year-outdated feminine affected person was referred to our division as a result of exertional dyspnea. On the age of 68, she began to notice swelling within the hands and toes. Based on the findings of skin hardening extending past the fingers and toes, together with constant histopathologic findings from a skin biopsy, the rheumatology department of her previous hospital diagnosed her as having SSc, even though numerous autoantibodies characteristic of scleroderma were adverse. Steroid therapy (PSL 20mg/day) was initiated, and the dosage was gradually tapered off as the cutaneous signs improved. Because the initiation of PSL therapy, the affected person had been experiencing exertional dyspnea without apparent lung shadows (Fig.1a). Two years later, an elevated Krebs von den Lungen-6 (KL-6, 2257 U/mL) and diffuse ground-glass opacities within the bilateral lung fields on chest computed tomography (CT) led to a analysis of interstitial lung disease (ILD) related to SSc (Fig.1b). Despite repeated IVCY and steroids, there was no improvement in subjective symptoms or imaging findings (Fig.1c); at this point, she was referred to us. Chest CT progress. No opacities at analysis of SSc (a), mild reticular opacities and ground-glass opacities at diagnosis of ILD associated with SSc (b), reticular opacities and ground-glass opacities with no improvement after IVCY (c), progressive reticular opacities and ground-glass opacities upon admission to our division (d,e), mild enchancment at 7months after initiating oxygen therapy (f). Reticular opacities and floor-glass opacities, which weren't observed at the time of SSc prognosis, appeared over time and worsened with little enchancment famous with IVCY. Saturation of percutaneous oxygen (BloodVitals SPO2) on room air was round 90% and dropped easily into the 70% vary with mild exertion. Arterial blood fuel evaluation revealed an arterial oxygen partial stress of 56.0Torr and an arterial carbon dioxide partial strain of 34.0Torr, indicating kind I respiratory failure. Respiratory sounds had been heard as intensive nice crackles in the bilateral lung fields.



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