ABSTRACT
Sarcoidosis is systematic granulomatous disease of unknown etiology which can affect any organ. Sarcoidosis belongs to diseases called interstitial lung diseases. Our study is a retrospective analysis of 169 patients (100 females and 69 males), whom we diagnosed sarcoidosis at our pulmonary department in years 2005-2010. Aim of the analysis was to find out prognostic factors and to describe the course of disease. Median age of sarcoidosis patients was 48 years (20-79). Females : males ratio was 1.5 : 1. Non smoker : smoker (or former smoker) ratio was 2.2 : 1. Familial occurrence was observed in 4 patients (2.4%). At diagnosis, stage 0 was present in 6 (3.5%) patients, stage I in 58 (34%) patients, stage II in 84 (49.5%) patients, stage III in 18 (11%) patients, and stage IV in 3 (2%) patients. Diagnosis was confirmed by histology in 111 patients. In 76 patients there was extrapulmonary sarcoidosis. The coincidence of sarcoidosis with autoimmune diseases was observed in 10 patients; 6 patients developed trombembolic disease. One patient suffered from sarcoidosis with cystic fibrosis. Spontaneous resolution was seen in 65 (38.5%) patients; 37 (64%) stage I patients, 26 (31%) stage II patients, and 2 (11%) stage III patients. One hundred one patients (60%) received corticosteroids. Adverse events of corticosteroid therapy were observed in 28 (26%) patients. In sarcoidosis patients with spontaneous resolution, no relapse of disease was observed. On the other hand, eleven (11%) patients treated with glucocorticosteroids relapsed. Median time to sarcoidosis relapse was 6 months (2-34). The age under 40 years, the X-ray stage I or II, the high CD4/CD8 ratio in bronchoalveolar fluid, pulmonary involvement, and therapy need for a period shorter than 2 years were assessed as a significant good prognostic factors. Observed lethality of our patient cohort was 1.2% (2 patients; both deaths related to sarcoidosis).
Subject(s)
Sarcoidosis, Pulmonary/diagnosis , Sarcoidosis, Pulmonary/drug therapy , Adult , Aged , Female , Humans , Male , Middle Aged , Prognosis , Sarcoidosis, Pulmonary/pathology , Young AdultABSTRACT
We describe a case of an adult patient, whom sarcoidosis was diagnosed at first and cystic fibrosis was also discovered 2 years later on. Cystic fibrosis and sarcoidosis are uncommon diseases that only rarely occur together. On the other hand, the coincidence of sarcoidosis and cystic fibrosis is possible, and, moreover, one of the diseases can remain undiagnosed long time. Relationship between sarcoidosis and cystic fibrosis seems not to be probable, in accordance with recent medical reports, and the coincidence of both conditions appears as sporadic.
Subject(s)
Cystic Fibrosis/complications , Sarcoidosis/complications , Adult , Cystic Fibrosis/diagnosis , Humans , Male , Sarcoidosis/diagnosisABSTRACT
INTRODUCTION: Most of cystic fibrosis (CF) patients survive now into adulthood and they are transferred to pulmonologist care. AIMS: An overview of progress in care for CF adults in Czech Republic and evaluation of relationship of pulmonary function, nutritional status and airway colonization. METHODS: All adult CF patients followed in pulmonary departments from December 1987 to December 2007 were included into study. Data about survival status, pulmonary function, nutritional status, airway colonization and other pulmonary and extrapulmonary manifestations of CF were collected from patients' records. RESULTS: Total of 206 patients (96 females) were followed. Pancreatic insufficiency was present in 175 (85.0%), liver disease in 61 (29.6%) and insulin treatment in 58 (28.2%) patients. Bone disease was found in 70 (46.7%) from 150 examined patients. Sixty-two patients (23 females) died at mean age 25.4 +/- 5.5 years (median 24.3 years). Worse survival was recorded in patients with Burkholderia cepacia complex (BCC) airway colonization (24.4 +/- 4.0 vs 28.5 +/- 7.0 years, p = 0.012). One hundred forty-four living patients were followed to date of the 31st December 2007 with mean age 27.5 +/- 6.5 years (median 26.5 years), FEV1 64.4 +/- 28.5% pred. and BMI 20.9 +/- 3.1 kg/m2. Worse pulmonary function was present in patients with BCC colonization (FEV1 58.8 +/- 21.9 vs 67.8 +/- 27.3% pred., p = 0.041) and in malnourished patients (FEv1 49.5 +/- 18.5 vs 69.7 +/- 25.9% pred., p < 0.0001). BCC colonization was found in 54 (37.5%), Pseudomonas aeruginosa (PA) colonization in 92 (63.9%) and colonization without BCC or PA in 40 (27.8%) patients, respectively. Malnutrition (BMI < 19.0 kg/m2) was recorded in 38 (26.4%) patients. CONCLUSION: This study confirms growing number of CF adults in Czech Republic, close relationship of pulmonary function and nutritional status and also unfavourable influence of BCC colonization.
