Idiopathic pleuroparenchymal fibroelastosis is characterized by an elevated serum level of surfactant protein-D, but Not Krebs von den Lungen-6.

PURPOSE: Idiopathic pleuroparenchymal fibroelastosis (IPPFE) is a recently reported rare disease entity characterized by fibrotic thickening of the pleural and subpleural parenchyma predominantly in the upper lobes in idiopathic interstitial pneumonias (IIPs). Because the clinical features of this rare disease are not fully elucidated, we examined the clinical characteristics of IPPFE, especially for serum interstitial biomarkers, surfactant protein-D (SP-D), and Krebs von den Lungen-6 (KL-6). METHODS AND RESULTS: Four consecutive cases of IPPFE who fulfilled the diagnostic criteria were studied. All cases were more than 60 years of age, and were classified as underweight by body mass index. A severe restrictive ventilatory defect was found in all cases on admission. High-resolution computed tomography showed intense pleural thickening associated with fibrosis predominant in upper lobes. Histopathological findings were also confirmed in three out of four cases. Interestingly, the serum level of SP-D was markedly elevated in all cases, while KL-6 was within normal range in three out of four cases. As compared with major IIPs such as idiopathic pulmonary fibrosis and fibrotic nonspecific interstitial pneumonia, IPPFE significantly showed higher frequency of cases with a unique pattern of serum biomarkers, which is characterized by an elevated level of SP-D with a normal range of KL-6. CONCLUSIONS: In IPPFE, SP-D might tend to be elevated, while KL-6 was within a normal range. Further study is required to determine the pathogenesis and clinical significance of the elevated SP-D in IPPFE.

[A case of severe pulmonary tuberculosis with septic shock and ARDS].

A 69-year-old man visited another hospital due to dyspnea and bloody sputum. A diagnosis of pulmonary tuberculosis was given because of multiple nodular shadows and consolidation on chest radiography and positive testing for acid-fast bacilli in his sputum; he was then referred to our hospital for treatment. Despite antituberculosis chemotherapy with isoniazid, rifampicin and ethambutol, his general condition worsened. On the 10th hospital day he needed mechanical ventilation and circulatory control in the intensive care unit. We diagnosed pulmonary tuberculosis complicated with septic shock, acute respiratory distress syndrome and disseminated intravascular coagulation based on his clinical course and laboratory data. After he recovered from shock, the antituberculosis chemotherapy was restarted. Intensive care resulted in the improvement of his general condition and the reduction of his chest abnormal shadows, and he was discharged 8 months after admission. No obvious recurrence was observed even after the cessation of antituberculosis chemotherapy, although pulmonary nodules on chest radiography remained.