Wojciech J. Piotrowski et al., Diagnosis and treatment of idiopathic pulmonary fibrosis 43 www.journals.viamedica.pl

IntroductionSarcoidosis is a systemic granulomatous disease which predominantly affects the lungs, although granulomas can also involve all other organs, including the heart. Cardiac sarcoidosis (CS) may occur at any stage of the disease and may be the cause of sudden cardiac death, even in a previously asymptomatic patient. The aim of this study was to evaluate the incidence of CS in a large group of patients diagnosed or followed up due to sarcoidosis.MethodsWe performed a retrospective analysis of patients at our institution discharged with the final diagnosis “sarcoidosis” (ICD-10: D86) from January 2008 to October 2012. Only those with biopsy (from respiratory tract or lymph nodes) confirmed diagnosis of sarcoidosis were included. We then selected the subset of patients with cardiac involvement due to sarcoidosis confirmed by positive magnetic resonance imaging.ResultsThe study covered 1375 consecutive sarcoidosis patients (51 % men), who were hospitalized during 5 years. Multiorgan disease was detected in 160 cases (11.7 %), and cardiac involvement was found in 64 patients (4.7 % of all), 70.3 % of whom were men. Twelve of those with CS were in stage I, 48 in stage II, and four in stage III. The odds ratio for having cardiac involvement in men compared to women was 2.3 (95 % CI 1.36–4.0, p = 0.002).ConclusionsCardiac involvement in sarcoidosis was diagnosed in the similar percentage as in previously published data but was significantly more frequently in men.

BackgroundCryptogenic organizing pneumonia (COP) is a clinicopathological syndrome of unknown origin. Corticosteroids are the standard treatment, but clarithromycin (CAM) is also effective. The aim of this observational retrospective study was to compare the results of CAM versus prednisone (PRE) treatment in patients with biopsy-proven OP without respiratory insufficiency.Material and methodsIn a 15-year period, 40 patients were treated with CAM (500 mg twice daily orally for 3 months) and 22 with PRE (mean initial dose of 0.67 ± 0.24 mg/kg/d for a mean of 8.59 ± 3.05 months).ResultsThe clinical presentation, laboratory, and radiological findings did not differ markedly between patients treated with CAM and PRE, with the exception of a higher frequency of sweats (55% vs. 23%; p < 0.015), ground glass opacities (95% vs. 50%; p <0.0001) and nodular lesions (45% vs. 18%; p = 0.036) in the CAM group. A complete response was achieved in 35(88%) patients treated with CAM and in all treated with PRE. Patients treated with PRE relapsed more frequently than those treated with CAM (54.5% vs. 10%; p < 0.0001). Corticosteroid-related adverse events were noticed in 8(6.5%) patients (with one death), but CAM caused only one (2.5%) allergic reaction. A FVC >80% identified patients who might be successfully treated with CAM with a sensitivity of 60% and a specificity of 88.57% (AUC 0.869; 95% CI 0.684–1; p = 0.008); the figures for the FEV1 were >70%, a sensitivity of 60%, and a specificity of 91.43% (AUC 0.809; 95%CI 0.609–1; p = 0.027).ConclusionsCAM can be used to treat COP patients in whom the pulmonary function parameters are within normal limits. Such therapy is shorter, better tolerated, and associated with fewer adverse events and relapses than is PRE. However, the therapy is ineffective in some patients.

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Serum VEGF-D is the useful biomarker of LAM extension, and might also prove predictive towards therapeutic decision.