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Weekly Chest CasesArchive of Old Cases

Case No : 1200 Date 2020-10-19

  • Courtesy of Jin Young Yoo, Sung Jin Kim / Chungbuk National University Hospital
  • Age/Sex 62 / M
  • Chief ComplaintDyspnea for 1 month
  • Figure 1
  • Figure 2
  • Figure 3
  • Figure 4
  • Figure 5
  • Figure 6

Diagnosis With Brief Discussion

Diagnosis
Pulmonary Alveolar Proteinosis (PAP)
Radiologic Findings
Figure 1. Chest PA shows bilateral widespread and symmetric parenchymal opacities
Figure 2-6. CT scans reveals diffuse widespread ground glass opacities with superimposed smooth interlobular septal thickening giving a typical appearance called "crazy paving pattern”. There is subpleural and equivocal costophrenic recess sparing.
Brief Review
Pulmonary alveolar proteinosis (PAP) is a rare respiratory syndrome characterized by the accumulation of surfactant lipoproteins within the alveoli, caused by an impairment of surfactant clearance or abnormal surfactant production, leading to variable impairment of the respiratory function. Diagnosis of PAP is initiated by computed tomography (CT) scan and confirmed by staining of bronchoalveolar lavage fluid (BALF). PAP is classified, according to various pathogenetic mechanisms, as primary, secondary or congenital; primary autoimmune form represents 90% of all PAP cases.
Chest radiography commonly demonstrates diffuse bilateral symmetrical infiltrates in a perihilar distribution, which can progress to confluent infiltrates involving all the five lobes. High-resolution computed tomography (HRCT) of the chest is mandatory to identify the peculiar radiological pattern that is characterized by interlobular septal thickening associated with patchy ground glass, known as “crazy paving”, referring to the polygonal appearance of the secondary pulmonary lobules within interspersed ground-glass attenuation. Areas of consolidation with air bronchograms can be present in addition to the ground-glass opacification. Fibrosis on HRCT at baseline or at follow-up predicts a poor prognosis. Pleural effusion, enlargement of mediastinal lymph nodes and evidence of air trapping are not characteristic, and should lead to alternate or concomitant diagnosis. Pulmonary nodules are also atypical and should be assessed for malignancy or infection. BALF staining is required for the diagnosis of PAP. When performed in a diseased lung area, BALF typically has a milky appearance but might appear abnormal or normal if performed in a healthy zone with a weak amount of lipoproteinaceous material.

Whole-lung lavage is the current gold standard of care of PAP; however, the therapeutic approach is strictly related to the pathogenic form and disease severity, including granulocyte–macrophage colony-stimulating factor augmentation strategies in autoimmune PAP and other promising new treatments.
Please refer to
Case 198, Case 275, Case 404, Case 496, Case 505, Case 525, Case 624, Case 670, Case 692, Case 818, Case 843, Case 880, Case 883, Case 1033,
KSTR Symposium 1998  Case 6 ,
KSTR Imaging Conference 2006 Summer  Case 8 ,
KSTR Imaging Conference 2009 Spring  Case 1 ,
KSTR Imaging Conference 2015 Spring  Case 9 ,
KSTR imaging conference 2017 Spring  Case 13,
References
1. Elena Salvaterra, Ilaria Campo.Pulmonary alveolar proteinosis: from classification to therapy. Breathe Jun 2020, 16 (2) 200018; DOI: 10.1183/20734735.0018-2020
Keywords
lung, Pulomary Alveolar Proteinosis ,

No. of Applicants : 57

▶ Correct Answer : 48/57,  84.2%
  • - Mayo Clinic , Japan HIROAKI TAKAHASHI
  • - College of Medicine, CHA University , Korea (South) PARK JONG WON
  • - Chonnam National University Hospital , Korea (South) SEUNGYOON CHAE
  • - Yeungnam University Medical Center , Korea (South) JONGSOO PARK
  • - Jiangsu province hospital , China WANGJIAN ZHA
  • - Chungbuk National University Hospital , Korea (South) JUNGHWAN KIM
  • - NIMS, HYDERABAD , India BHASKAR K
  • - university of montreal , Canada Andrei Bogdan Gorgos I
  • - Mayo Clinic , United States AKITOSHI INOUE
  • - Soonchunhyang University Hospital Bucheon , Korea (South) SEONGHWAN BYUN
  • - Chonnam National University Hwasun Hospital , Korea (South) WONGI JEONG
  • - DELTA CARE HOSPITAL, THANJAVUR, INDIA , India SIVARAJA SUBRAMANIAM
  • - Tiger Gate Hospital , Japan SHIN-ICHI CHO
  • - Diskapi Yildirim Beyazit Hospital, Ankara , Turkey MERIC TUZUN
  • - , Japan HIROAKI ARAKAWA
  • - University of Tsukuba Hospital , Japan SODAI HOSHIAI
  • - Seoul Medical Center , Korea (South) HYUN JI LIM
  • - Cabinet Capricorne, clinique sainte-clotilde , Reunion FABIEN HO
  • - Asan Medical Center , Korea (South) EUNJI CHOI
  • - , India SHYJUMON GEORGE
  • - Beaulieu clinic Geneva , Switzerland GILLES GENIN
  • - The University of Tokyo Hospital , Japan RYO KUROKAWA
  • - National Center for Global Health and Medicine , Japan HIROSHI TAKUMIDA
  • - Narayana Multispeciality Hospital Jaipur Rajasthan , India JAINENDRA JAIN
  • - Vita Hospital , Brazil DIOGO LAGO PINHEIRO
  • - University of Tsukuba, Dept of Radiology , Japan MANABU MINAMI
  • - Osaka City General Hospital , Japan SHU MATSUSHITA
  • - GEMS, Srikakulam, Andhra Pradesh , India RAMU C
  • - Chonnam National University Hwasun Hospital , Korea (South) JEONG YEOP LEE
  • - Primus , India ABHISHEK BHASKAR YASHOD
  • - Other , Korea (South) MINSU KIM
  • - The University of Tokyo Hospital , Japan TOSHIHIRO FURUTA
  • - , China QING WEN YU
  • - Ondokuz Mayis University , Turkey CETIN CELENK
  • - , Korea (South) YEONSOO KIM
  • - Ichinomiya Nishi Hospital , Japan Takao Kiguchi
  • - CH de la Cote Basque , France PAUL ARDILOUZE
  • - , Korea (South) HYEWON CHOI
  • - , Korea (South) JIN YOUNG LEE
  • - Saitama-Sekishinkai Hosptal , Japan MIHOKO YAMAZAKI
  • - National Center for Global Health and Medicine , Japan MASATOSHI HOTTA
  • - Chonnam National University Hwasun Hospital , Korea (South) SEONGWOO CHO
  • - Showa University Fujigaoka Hospital , Japan KYOKO NAGAI
  • - Chonbuk National University Hospital , Korea (South) YOUNGKWANG LEE
  • - the first affiliatited hospital of nanjing medical univercity , China HAI XU
  • - , Senegal AXEL MBUYU KAYEMBE
  • - Kinki University Faculty of Medicine, , Japan MITSURU MATSUKI
  • - Hamamatsu University Hospital , Japan YUKI HAYASHI
▶ Correct Answer as Differential Diagnosis : 9/57,  15.8%
  • - , Turkey MURAT TEPE
  • - McGill University Health Center , Canada Alexandre Semionov
  • - Other , Korea (South) KYU-CHONG LEE
  • - Lilavati Hospital , India DHRUV UMESH SHARMA
  • - Eskisehir Sehir Hastanesi , Turkey MURAT SAHIN
  • - Holy Family Hopsital , India RAJESH GOTHI
  • - Fortis hospital , Mohali , India SHALEEN RANA
  • - Diagnose.me (BV) / Royal Perth Hospital , Australia YURANGA WEERAKKODY
  • - Gifu University Hospital , Japan Yo Kaneko
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