ABSTRACT
The combined pulmonary fibrosis and emphysema (CPFE) syndrome is a unique and an under-recognized disorder characterized by emphysema in the upper lobes and interstitial fibrosis in the lower lobes of the lung. It occurs predominantly in males and almost exclusively in smokers. This rare combination of a restrictive and an obstructive mechanical defect carries a poorer prognosis than either of the two components. We present a case of CPFE syndrome in a non-smoker female patient who developed lower lobe emphysema subsequent to development of interstitial fibrosis. The case was remarkable for the extreme rarity of several presenting features, namely, a lower lobe occurrence of emphysema subsequent to pre-existent interstitial fibrosis, female gender and absence of a history of smoking.
Subject(s)
Cough/diagnosis , Dyspnea/diagnosis , Lung Diseases, Interstitial/diagnostic imaging , Lung/diagnostic imaging , Pulmonary Emphysema/complications , Pulmonary Fibrosis/complications , Cough/etiology , Dyspnea/etiology , Fatal Outcome , Female , Forced Expiratory Volume/physiology , Humans , Hypoxia/physiopathology , Lung/physiopathology , Lung Diseases, Interstitial/drug therapy , Middle Aged , Pulmonary Emphysema/diagnostic imaging , Pulmonary Emphysema/physiopathology , Pulmonary Fibrosis/diagnostic imaging , Pulmonary Fibrosis/physiopathology , Rare Diseases , Respiratory Function Tests/methods , Respiratory Tract Infections/complications , Smoking/trends , Tomography, X-Ray Computed/methods , Vital Capacity/physiologyABSTRACT
Cavitation in pulmonary sarcoidosis is rather unusual and even rarer in the alveolar form of the disease. A review of the literature revealed only 13 documented patients with cavitating alveolar sarcoidosis. Of the 13 patients, clinical information was available in only eight cases. Aspergilloma complicating cavitary alveolar sarcoidosis has been documented only once before. A 38-year-old woman with progressive dyspnoea for 3 years presented owing to a bout of haemoptysis. Imaging prior to presentation demonstrated mediastinal lymphadenopathy and coalescent parenchymal alveolar opacities along with air bronchograms, suggestive of alveolar sarcoidosis. On presentation, cavitations were visible in both lungs within the consolidation along with an intracavitary mass in the left upper lobe, which on evaluation was recognised as an aspergilloma. Bronchoscopy disclosed non-caseating granulomas on histopathology confirming pulmonary sarcoidosis. Cavitary alveolar sarcoidosis with aspergilloma formation can result in a diagnostic dilemma, especially in endemic tuberculous areas.
