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Alveolar proteinosis in Behçet's disease
Cuneyt Tetikkurt1, Seza Tetikkurt, Imran Ozdemir
1Pulmonary Diseases Department, Cerrahpasa Medical Faculty, Istanbul University, Turkey. docmct@superonline.com.
Multidisciplinary Respiratory Medicine
|September 11, 2012
Summary
This study reports a rare case of pulmonary alveolar proteinosis in a patient with Behçet's disease. The condition showed spontaneous remission, highlighting a unique clinical presentation and outcome.
Area of Science:
- Pulmonology
- Rheumatology
- Pathology
Background:
- Behçet's disease is a multisystem vasculitis with rare pulmonary manifestations.
- Pulmonary alveolar proteinosis (PAP) is characterized by the accumulation of lipoproteinaceous material in the alveoli.
- The association between Behçet's disease and PAP is not well-established.
Purpose of the Study:
- To report a unique case of secondary pulmonary alveolar proteinosis in a patient with Behçet's disease.
- To investigate the clinical, radiological, and pathological features of this rare association.
- To describe the outcome of spontaneous remission.
Main Methods:
- Case report of a 51-year-old male with Behçet's disease presenting with respiratory symptoms.
- Diagnostic workup included chest CT, bronchoalveolar lavage (BAL), and transbronchial biopsy.
- Laboratory tests included serum anti-granulocyte-macrophage colony stimulating factor (GM-CSF) antibody levels.
Main Results:
- Chest CT revealed bilateral ground glass opacities and interstitial septal thickening.
- BAL and biopsy confirmed the presence of amorphous, lipoproteinaceous material consistent with PAP.
- Serum anti-GM-CSF antibody was negative.
- The patient experienced spontaneous remission of symptoms and radiological findings.
Conclusions:
- This case highlights a rare presentation of pulmonary alveolar proteinosis secondary to Behçet's disease.
- The absence of anti-GM-CSF antibodies in this case suggests a potential distinction from idiopathic PAP.
- Spontaneous remission in this context warrants further investigation and may offer insights into PAP pathogenesis.
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