Autoimmune pulmonary alveolar proteinosis developed during treatment for systemic sclerosis: a case report
Abstract Background Reports of autoimmune diseases coexisting with autoimmune pulmonary alveolar proteinosis (autoimmune PAP; APAP) are extremely rare. APAP coexisting with autoimmune diseases may often be misdiagnosed as connective tissue disease–associated interstitial lung disease (ILD). Here, we...
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BMC
2025-01-01
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| Online Access: | https://doi.org/10.1186/s12890-025-03489-0 |
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| author | Tomoki Kozono Kentaro Tanaka Tomoko Yagi Kazuto Kamikawaji Masaki Watanabe Azusa Iwanaga Minako Hamada Yoshifusa Koreeda Hiromasa Inoue Ikkou Higashimoto |
| author_facet | Tomoki Kozono Kentaro Tanaka Tomoko Yagi Kazuto Kamikawaji Masaki Watanabe Azusa Iwanaga Minako Hamada Yoshifusa Koreeda Hiromasa Inoue Ikkou Higashimoto |
| author_sort | Tomoki Kozono |
| collection | DOAJ |
| description | Abstract Background Reports of autoimmune diseases coexisting with autoimmune pulmonary alveolar proteinosis (autoimmune PAP; APAP) are extremely rare. APAP coexisting with autoimmune diseases may often be misdiagnosed as connective tissue disease–associated interstitial lung disease (ILD). Here, we describe a rare case of a patient with systemic sclerosis who was diagnosed with APAP after the exacerbation of lung opacities during treatment with immunosuppressive agents. Case presentation A 72-year-old woman was diagnosed with systemic sclerosis (SSc) at the age of 68, and initiated treatment with prednisolone (PSL). At the age of 70, she was diagnosed with ILD associated with SSc. Despite intravenous cyclophosphamide (IVCY), no improvement was observed. A significant elevation of Krebs von den Lungen-6 (KL-6) and a crazy-paving pattern on chest computed tomography (CT) are observed. Bronchoscopy showed milky white bronchoalveolar lavage fluid (BALF) and histology of periodic acid-Schiff (PAS) stain-positive eosinophilic granular material. Serum anti granulocyte–macrophage colony-stimulating factor (GM-CSF) antibodies were measured, and the result was positive, leading to the diagnosis of APAP. Conclusion In patients with interstitial lung shadows who do not improve with immunosuppressive treatment, PAP is one of the differential diagnoses that should be considered. All physicians should be aware that the appropriate diagnosis of PAP and the measurement of serum anti-GM-CSF antibodies will critically affect patient outcomes. |
| format | Article |
| id | doaj-art-3cba159167674524a9b02cc4027fd626 |
| institution | Kabale University |
| issn | 1471-2466 |
| language | English |
| publishDate | 2025-01-01 |
| publisher | BMC |
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| series | BMC Pulmonary Medicine |
| spelling | doaj-art-3cba159167674524a9b02cc4027fd6262025-01-26T12:13:07ZengBMCBMC Pulmonary Medicine1471-24662025-01-012511610.1186/s12890-025-03489-0Autoimmune pulmonary alveolar proteinosis developed during treatment for systemic sclerosis: a case reportTomoki Kozono0Kentaro Tanaka1Tomoko Yagi2Kazuto Kamikawaji3Masaki Watanabe4Azusa Iwanaga5Minako Hamada6Yoshifusa Koreeda7Hiromasa Inoue8Ikkou Higashimoto9Department of Pulmonary Medicine, National Hospital Organization MinamiKyushu HospitalDepartment of Pulmonary Medicine Graduate School of Medical and Dental Sciences, Kagoshima UniversityDepartment of Pulmonary Medicine, National Hospital Organization MinamiKyushu HospitalDepartment of Pulmonary Medicine, National Hospital Organization MinamiKyushu HospitalDepartment of Pulmonary Medicine, National Hospital Organization MinamiKyushu HospitalDepartment of Pulmonary Medicine, National Hospital Organization MinamiKyushu HospitalDepartment of Pulmonary Medicine, National Hospital Organization MinamiKyushu HospitalDepartment of Pulmonary Medicine, National Hospital Organization MinamiKyushu HospitalDepartment of Pulmonary Medicine Graduate School of Medical and Dental Sciences, Kagoshima UniversityDepartment of Pulmonary Medicine, National Hospital Organization MinamiKyushu HospitalAbstract Background Reports of autoimmune diseases coexisting with autoimmune pulmonary alveolar proteinosis (autoimmune PAP; APAP) are extremely rare. APAP coexisting with autoimmune diseases may often be misdiagnosed as connective tissue disease–associated interstitial lung disease (ILD). Here, we describe a rare case of a patient with systemic sclerosis who was diagnosed with APAP after the exacerbation of lung opacities during treatment with immunosuppressive agents. Case presentation A 72-year-old woman was diagnosed with systemic sclerosis (SSc) at the age of 68, and initiated treatment with prednisolone (PSL). At the age of 70, she was diagnosed with ILD associated with SSc. Despite intravenous cyclophosphamide (IVCY), no improvement was observed. A significant elevation of Krebs von den Lungen-6 (KL-6) and a crazy-paving pattern on chest computed tomography (CT) are observed. Bronchoscopy showed milky white bronchoalveolar lavage fluid (BALF) and histology of periodic acid-Schiff (PAS) stain-positive eosinophilic granular material. Serum anti granulocyte–macrophage colony-stimulating factor (GM-CSF) antibodies were measured, and the result was positive, leading to the diagnosis of APAP. Conclusion In patients with interstitial lung shadows who do not improve with immunosuppressive treatment, PAP is one of the differential diagnoses that should be considered. All physicians should be aware that the appropriate diagnosis of PAP and the measurement of serum anti-GM-CSF antibodies will critically affect patient outcomes.https://doi.org/10.1186/s12890-025-03489-0Autoimmune pulmonary alveolar proteinosisAutoimmune diseasesSargramostim inhalationAnti-GM-CSF antibodies |
| spellingShingle | Tomoki Kozono Kentaro Tanaka Tomoko Yagi Kazuto Kamikawaji Masaki Watanabe Azusa Iwanaga Minako Hamada Yoshifusa Koreeda Hiromasa Inoue Ikkou Higashimoto Autoimmune pulmonary alveolar proteinosis developed during treatment for systemic sclerosis: a case report BMC Pulmonary Medicine Autoimmune pulmonary alveolar proteinosis Autoimmune diseases Sargramostim inhalation Anti-GM-CSF antibodies |
| title | Autoimmune pulmonary alveolar proteinosis developed during treatment for systemic sclerosis: a case report |
| title_full | Autoimmune pulmonary alveolar proteinosis developed during treatment for systemic sclerosis: a case report |
| title_fullStr | Autoimmune pulmonary alveolar proteinosis developed during treatment for systemic sclerosis: a case report |
| title_full_unstemmed | Autoimmune pulmonary alveolar proteinosis developed during treatment for systemic sclerosis: a case report |
| title_short | Autoimmune pulmonary alveolar proteinosis developed during treatment for systemic sclerosis: a case report |
| title_sort | autoimmune pulmonary alveolar proteinosis developed during treatment for systemic sclerosis a case report |
| topic | Autoimmune pulmonary alveolar proteinosis Autoimmune diseases Sargramostim inhalation Anti-GM-CSF antibodies |
| url | https://doi.org/10.1186/s12890-025-03489-0 |
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