Reduced CT-derived erector spinae muscle area: a poor prognostic factor for short- and long-term outcomes in idiopathic pulmonary fibrosis patients.

AIM: To assess the relationship between idiopathic pulmonary fibrosis (IPF) prognosis, baseline skeletal muscle mass, and attenuation on computed tomography (CT) and clinical parameters. MATERIAL AND METHODS: This retrospective cohort study enrolled 195 patients. The mean follow-up duration was 42.52 months. Erector spinae muscle area (ESMA), pectoralis muscle area (PMA), and the attenuation of the erector spinae muscle at the level of T12 vertebrae were measured. Muscle indexes were obtained by adjusting the measured muscle areas to the patients' heights. The relationship between baseline CT-derived muscle metrics and clinical parameters including short- and long-term mortality were evaluated. RESULTS: There was a moderate correlation between ESMA and PMA and pectoralis muscle index (PMI; r=0.536, p<0001 and r=0.403, p<0.001 respectively). ESMA correlated significantly with forced expiratory volume in 1 second (FEV1; hazard ratio [HR] = 0.488 p<0.001) and forced vital capacity (FVC; HR=0.501, p<0.001). Compared with PMA, ESMA was more strongly associated with 1- and 2-year mortality in patients with IPF (HR=0.957, p=0.022). The survival rate in male patients with sarcopenia was significantly worse (p=0.040). CONCLUSION: ESMA measurements obtained from CT correlated with clinical parameters in IPF patients and were also predictors of short- and long-term survival.

Clinical and immunological analysis of 23 adult patients with common variable immunodeficiency.

BACKGROUND: Common variable immunodeficiency (CVID) is characterized by hypogammaglobulinemia, defective antibody production, and recurrent upper and lower airway tract infections. OBJECTIVES: To reveal the clinical heterogeneity of this condition, analyze the high frequency of respiratory and gastrointestinal complications despite satisfactory trough immunoglobulin (Ig) G levels, and determine the main difficulties in management and treatment. METHODS: We performed a retrospective analysis of 23 patients (13 male and 10 female) diagnosed with CVID between 2001 and 2008. RESULTS: The median diagnostic delay for females and males was 15 years (range, 1-32 years) and 8 years (range, 1-31 years), respectively. Restrictive, obstructive, and combined pulmonary function defects were determined in 23%, 27%, and 14% of patients, respectively. The most frequent findings on the thoracic computed tomography scan were bronchiectasis, mediastinal lymphadenopathy, fibrosis, ground-glass patterns, mosaic oligemia, peribronchial cuffing, and parenchymal nodules. Giardiasis and duodenal lymphoid hyperplasia were detected in 52% and 42% of the patients, respectively, and Helicobacter pylori in 42%. Vitamin A levels were normal, although beta-carotene and/or vitamin E levels were decreased in patients presenting malabsorption-related symptoms. Malignancy was documented in 3 patients and decreased bone mineral density in 9 patients (3 had osteoporosis and 3 had osteomalacia). CONCLUSION: CVID is a multisystemic disease that should be managed by a multidisciplinary team. Intravenous immunoglobulin therapy and antibiotics do not seem to have a suppressive effect on granulomatous or inflammatory manifestations. More comprehensive studies based not only on peripheral blood but also on immunohistological analysis are necessary to shed light on the pathogenesis of these life-threatening complications.