대한흉부영상의학회 Weekly Chest Cases

Case No : 1415 Date 2024-12-02

Courtesy of Jung Im Kim, Hye Young Choi / Kyunghee University Hospital at Gangdong
Age/Sex 56 / M
Chief ComplaintFever/cough, 2months ago

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Diagnosis With Brief Discussion

Diagnosis: PAP(pulmonary alveolar proteinosis) with secondary aspergillus Infection
Radiologic Findings: Fig 1. Chest PA shows diffuse GGO in both lungs and irregular consolidation in the RMLF and BLLFs.
Fig 2-3. CT scans reveals diffuse GGO with crazypaving pattern in both lungs.
Fig 4-5. Previous diffuse GGO with crazypaving pattern in both lungs were partially decreased and partially increased. In addition, subpleural or peribronchial consolidation with GGO in both lungs were newly developed. Subpleural consolidation in the LLL showed internal necrotic change.
Brief Review: The patient underwent wedge resection of the right upper and right lower lobe and pulmonary alveolar proteinosis was diagnosed in the right upper lobe specimen. In the right lower lobe specimen, fungal hyphae were found along with pulmonary alveolar proteinosis and confirmed as Aspergillosis. After antifungal treatment, consolidations with GGO were resolved and extent of GGOs with crazypaving pattern were decreased.
Pulmonary alveolar proteinosis (PAP) is a rare disease. The prevalence ranged from 4 to 40 cases in every 1 million population with an incidence of 0.2-0.42 cases for every 1 million population every year. In general, PAP is a condition with deposition of surfactant, a lipoprotein, in the alveolar space and can be accompanied by alveolar macrophage dysfunction. In patients with PAP, the gas exchange process is disrupted and causes various respiratory issues with the increased risk
of secondary infection.

PAP can be classified into three types based on its pathophysiology as primary, secondary, and congenital. In primary PAP, there is a disturbance of GM-CSF signaling, causing a reduction in surfactant clearance. GM-CSF is a cytokine with an important role in surfactant homeostasis and is secreted by many cells, including the type II alveolar epithelial, and can be classified further as autoimmune (increase in anti-GM-CSF antibody) or hereditary (CSF2RA or CSF2RB gene mutation). More than 90% of PAP cases are autoimmune. In secondary PAP, there is a decrease in the number and/or function of the alveolar macrophages due to certain health conditions or substance exposures, such as myelodysplastic syndrome, myeloid leukemia (acute and chronic), cigarette smoke exposure, and inhalation of silica or other mineral and metal particles. PAP can also be caused by the abnormality of surfactant production as in congenital PAP.
Secondary infection is the most common complication occurring in autoimmune PAP and is life-threatening. Around 5-13% of PAP patients have a secondary infection. Infections can be caused by general pathogens (Streptococcus, Haemophilus, and Enterobacter) and opportunistic pathogens (Mycobacterium, Nocardia, Actinomyces, Aspergillus, and Cryptococcus). From the many pathogens, Aspergillus, Mycobacterium, and Nocardia are the most common infection source in autoimmune PAP. From Japanese national PAP registry, most of the infections in autoimmune PAP are caused by Aspergillus spp.
References: 1. Jouneau S, Menard C, Lederlin M. Pulmonary alveolar proteinosis. Respirology 2020;25(8):816-826.
2. Trapnell BC, Nakata K, Bonella F, Campo I, Griese M, Hamilton J, et al. Pulmonary alveolar proteinosis. Nat Rev 2019;5(1):16.
3. Trapnell BC, Whitsett JA, Nakata K. Pulmonary alveolar proteinosis. N Engl J Med 2003;349(26):2527-2539
4. Borie R, Danel C, Debray MP, Taille C, Dombret MC, Aubier M, et al. Pulmonary alveolar proteinosis. Eur Respir Rev 2011;20(120):98-107
5. Fanny Fachrucha, et al.: PAP with Secondary Aspergillus Infection Journal of Health Sciences 2021;11(3):191-195
Keywords

No. of Applicants : 59

▶ Correct Answer : 1/59, 1.7%

- Saitama-Sekishinkai Hosptal , Japan MIHOKO YAMAZAKI

▶ Correct Answer as Differential Diagnosis : 6/59, 10.2%

- Healthy Longevity Medical Center , Japan SHIN-ICHI CHO
- Vita Hospital , Brazil DIOGO LAGO PINHEIRO
- The University of Tokyo Hospital , Japan TOSHIHIRO FURUTA
- , Japan YOSHIKI ISHII
- Yonsei University,Severance Hospital , Korea (South) SEO BUM CHO
- Toyota Kosei Hospital , Japan YUKI HAYASHI

▶ Semi-Correct Answer : 39/59, 66.1%

- , Japan SUZUNE TSUKAMOTO
- medical scanning , Japan HIROAKI ARAKAWA
- ZIGONG TCM HOSPITAL OF CHINA , China CAO CUN YOU
- Shiga University of Medical Science , Japan AKITOSHI INOUE
- Kyoto University , Japan AKIHIKO SAKATA
- McGill University Health Center , Canada ALEXANDRE SEMIONOV
- Kyoto City Hospital , Japan YUSUKE UTSUNOMIYA
- , Korea (South) DONG-HO BANG
- Other , Korea (South) SEONGSU KANG
- , Korea (South) JIN YOUNG LEE
- Other , Korea (South) DOWOO KIM
- , China FANG YING GEN
- Secomedic Hospital , Japan FUMINORI MIYOSHI
- Seoul Medical Center , Korea (South) HYUK GI HONG
- university of montreal , Canada Andrei Bogdan Gorgos I
- University of Yamanashi , Japan TAKAAKI HASHIMOTO
- Osaka University , Japan AKINORI HATA
- University of Yamanashi , Japan HIROAKI WATANABE
- Nankai medical center , Japan NORITAKA KAMEI
- Other , Korea (South) CHAEHUN LIM
- Other , Korea (South) MARI A
- Osaka Metropolitan University Hospital , Japan SHU MATSUSHITA
- , Myanmar MAW MAW OO
- , Japan SHUNJIRO NOGUCHI
- , Korea (South) HEONSEOK LEE
- Jichi Medical University, School of Medicine , Japan MITSURU MATSUKI
- University of Tsukuba, Dept of Radiology , Japan MANABU MINAMI
- Shiga General Hospital , Japan YUSAKU MORIBATA
- Mie university , Japan SHIKO OKABE
- GHICL , France MANUEL TOLEDANO
- Dokkyo Medical University , Japan HIROAKI ARAKAWA
- Holy Family Hospital , India RAJESH GOTHI
- Korea University Anam Hospital , Korea (South) KYU-CHONG LEE
- Paras hospital, Panchkula , India SHALEEN RANA
- The First Affiliated Hospital of Nanjing Medical University , China TENG ZHANG
- Kyoto University , Japan SHO KOYASU
- Fukuoka university , Japan KEISUKE SATO
- , China LI GUO
- Jiangsu province hospital , China WANGJIAN ZHA

대한흉부영상의학회 Korean Society of Thoracic Radiology

Archive of Old Cases

Case No : 1415 Date 2024-12-02

Discussion

No. of Applicants : 59