대한흉부영상의학회 Weekly Chest Cases

Case No : 505 Date 2007-07-02

Courtesy of Ho Yun Lee, MD. / Seoul National University College of Medicine, Korea
Age/Sex 15 / F
Chief ComplaintGradual onset of cough and dyspnea

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Diagnosis With Brief Discussion

Diagnosis: Pulmonary Alveolar Proteinosis
Radiologic Findings: Chest PA shows bilateral patchy haziness in both lungs with slightly lower and peripheral predominance.
Chest CT shows patchy ground-glass opacities and internal reticular opacities of subpleural portion of both lungs.
Brief Review: Pulmonary alveolar proteinosis (PAP) is a rare lung disorder of unknown etiology characterized by alveolar filling with floccular material that stains positive using the periodic acid-Schiff (PAS) method and is derived from surfactant phospholipids and protein components.
Two forms are recognized, (1) primary (idiopathic) and (2) secondary (due to lung infections; hematologic malignancies; and inhalation of mineral dusts such as silica, titanium oxide, aluminum, and insecticides). Incidence of PAP is increased in patients with hematologic malignancies and AIDS, suggesting a relationship with immune dysfunction.
Patients with pulmonary alveolar proteinosis (PAP) typically present with a gradual onset of symptoms. As many as 30% of patients are asymptomatic, even with diffuse chest radiograph (CXR) abnormalities. Most common symptoms include Persistent dry cough (or scant sputum production) or Persistent dry cough (or scant sputum production).
Serologic studies are generally not useful. Flexible bronchoscopy with bronchoalveolar lavage (BAL) remains the criterion standard. Elevated levels of the proteins SP-A and SP-D in serum and BAL fluid may be useful. Elevated titer of neutralizing autoantibody against GM-CSF (immunoglobulin G [IgG] isotype) in serum and BAL fluid may be useful. Recent studies have proposed that deficiency of GM-CSF causes pulmonary alveolar proteinosis (PAP); all patients studied had the antibody to GM-CSF. Serum lactate dehydrogenase (LDH) level is usually elevated, but this finding is nonspecific.
The diagnosis can be made by BAL only if PAS staining is requested. Therefore, PAP is probably underdiagnosed.
CXR shows bilateral perihilar infiltrates with consolidation in a "bat-wing" configuration, which may mimic pulmonary edema, although with a typical absence of cardiomegaly or pleural effusion. Unilateral involvement occurs occasionally, and lymphadenopathy is rarely present. Typically, changes progress over weeks to months into a diffuse reticulogranular pattern. High-resolution computed tomography (HRCT) scan of the chest demonstrates areas of patchy ground-glass opacification with smooth interlobular septal thickening and intralobular interstitial thickening, which produces a polygonal pattern referred to as "crazy paving".
Management of pulmonary alveolar proteinosis (PAP) depends on the progression of the illness, coexisting infections, and degree of physiological impairment. The standard of care for PAP is mechanical removal of the lipoproteinaceous material by whole-lung lavage.
In secondary PAP, appropriate treatment of the underlying cause also is warranted.
GM-CSF has been shown to improve PAP in several patients and is being investigated.
Lung transplantation is the treatment of choice in patients with congenital PAP and in adult patients with end-stage interstitial fibrosis and cor pulmonale.
References: Am J Respir Crit Care Med 2002; 166: 215?35
Keywords: Lung, Interstitium, Metabolic and storage lung disesae,

No. of Applicants : 51

▶ Correct Answer : 32/51, 62.7%

- Dept. of radiology, Inje University Ilsan Paik Hospital, Korea Bae Geun Oh
- Jiangsu Province Official Hospital, Nanjing, Jiangsu Province, China Dong Sheng Jin
- CHRU Lille hopital calmette, France Toledano Manuel
- Apollo clinic, Kandivali, Mumbai, Malad, Mumbai, India Jeshil R. Shah
- Dept. of Radiodiagnosis and Imaging, PGIMER, Chandigarh, India Ram Prakash Galwa
- Hospital Sao Paulo, Brazil Israel Missrie
- Asan Medical Center, Korea Eun Jin Chae
- Aditya Imaging centre, Sayajigunj, Vadodara, Gujarat, India Vivek Patel
- Maimonides Medical Center; Brooklyn, N.Y., U.S.A. Naomi Twersky
- Escorts heart institute new delhi, India Monika Aggarwal
- Regional Imaging Riverina, Australia Rashid Hashmi
- Yashodha Hospital, Hyderabad, Andhra Pradesh, India Satish Babu, Maddkuri
- Osaka University, Japan Osamu Honda
- University of Colorado Health Sciences Center, Denver, CO, USA Seth Kligerman
- NUH, nottingham, UK Rohit Kochhar
- Cotia Sade, Brazil Jos Luiz Masson
- Dept. of radiology, Zhongshan Hospital, Fudan University, Shanghai, China. Sheng-xiang Rao
- Seoul National University Hospital, Korea Gyoung Min Kim
- Hangang Sacred Heart Hospital, Korea Eil Seong Lee
- Annemasse, Polyclinique De Savoie, France Gay-Depassier Philippe
- Dong-A university hospital, Busan, Korea Park Seong Hoon
- HIS Hospitals, Site d'Ixelles, Brussels, Belgium Emmanuel Agneessens
- CHU Grenoble, France Bing Fabrice
- Annecy hospital, France Gilles Genin
- IRSA, La Rochelle, France Denis Chabassiere
- National Taiwan University Hospital, Taipei, Taiwan Chin Chung Shu
- Yongsan Hospital, College of Medicine Chung-Ang University, Korea Jae Seung Seo
- Jackson Memorial Hospital, Miami, Florida, USA N.B.S.Mani
- China Medical University Hospital, Taiwan Jun-Jun Yeh
- E-Da hospital, Taiwan Yu-Feng Wei
- Max Hospital, New Delhi, India Vickrant Malhotra
- Aims, New Delhi, India Ashish Gupta

▶ Correct Answer as Differential Diagnosis : 11/51, 21.6%

- Registrar in Radiology, UHL NHS Trust, UK. A. Donuru
- Saint-etienne, France Antoine Marie
- Mumbai , India Rahul B Deshmukh
- Mackay Memorial Hospital, Taipei, Taiwan Chia-Fu Tsai
- S S G Hospital, Medical College, Vadodara (GUJ), India M P Swami
- Nour El-Islam Radiology Center Egypt .Alexandria Elsayed Mousa
- Annecy Hospital, France Olivier Segall
- Hospital Sotiria, Athens, Greece Vasilios Tzilas
- Kazipet, Warangal, AP, India Pavankumar Vodithala
- Vital imaging centre, Mumbai, India Ganesh Agrawal
- Nassau, Bahamas Trupti Dabholkar

▶ Semi-Correct Answer : 4/51, 7.8%

- Dept. of radiology, Changhua Christian Hospital, Taiwan Chiung-Ying, Liao
- Samsung Medical Center, Korea Chin A Yi
- Homs National Hospital, Homs - Syria Rami Abou Zalaf
- KAUMS, Kashan, Iran Ebrahim Razi

대한흉부영상의학회 Korean Society of Thoracic Radiology

Archive of Old Cases

Case No : 505 Date 2007-07-02

Discussion

No. of Applicants : 51