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Objectives: NSİP is an interstitial lung disease (İLD) is classified under idiopathic subgroup of diffuse parenchymal lung diseases. But it can also arise in the background of CTDs, environmental and occupational exposure, drugs, immune deficiency, infections etc. In this study we aimed primarily to invastigate clinical relationship of NSIP with CTDs, enviromental exposures and drug reaction and also effects on treatment response and mortality as well within patients diagnosed as NSIP and followed up for 5 years in our clinic.
Methods: 79 patients (36 female, 43 male) were included in this study. The data about NSİP patients were reached with J84.1 ICD code via hospital database (HBYS) and 175 patients’ histories were read. Data were analyzed with SPSS statistics version 24.0.
Results: Patients were between 41-87 ages, the disease is mostly seen in 7th, 8th decade. Long term dry cough was the most frequent complaint (n=54, 68%). At first 13 patients were diagnosed with a rheumatologic disease (16%), 11 patients were diagnosed afterwards too. RA (n=7) and Sjögren Syndrome (n=7) was most frequent ones. 34 patients (%43) had rheumatologic symptoms. The most frequent were the joint pain and decrease in RAM (n=25 31.6%). NSİP is usually diagnosed with clinical history and radiology in our clinic. BAL was done to 17 patients in whom other diseases cannot be ruled out. Biopsy was taken from ten of them. 58 patients (73.4%) took treatment through their follow up. 34 (43%) of them took steroid treatment. 4 of them (5%) showed good prognosis, 12 of them (15%) remained stable and 13 of them (16%) developed mortality. PFTs were normal in 66 patients who were compliant for the tests. Rest of them had the obstructive pattern and the restriction pattern was not seen. 43 (69%) patients had diffusion restriction out of 62 patients who DLco When clinical progression was assessed, 6 patients (7%) showed recovery and were taken to a follow up program with treatment. 21 stable patients (26,5%) are still coming to their routine follow up appointments. 13 patients (21%) died of disease. Mean survival time of patients was 66 months with Kaplan Meier analysis. There was no statistically significant relationship between survival rates and hypoxemia levels, lung volumes, respiratory function tests and diffusion capacities.
Conclusion: DLCO must be made and if necessary HRCT or thorax CT must be ordered for ILD in patients with long term dry cough and dyspnea who have otherwise normal PFT.