Chest Wall Diseases: Respiratory Pathophysiology.

The chest wall consists of various structures that function in an integrated fashion to ventilate the lungs. Disorders affecting the bony structures or soft tissues of the chest wall may impose elastic loads by stiffening the chest wall and decreasing respiratory system compliance. These alterations increase the work of breathing and lead to hypoventilation and hypercapnia.

Complementary Role of CMR to Conventional Screening in the Diagnosis and Prognosis of Cardiac Sarcoidosis.

Objectives: The goal of this study was to assess the independent and collective diagnostic value of various modalities in cardiac sarcoidosis, delineate the role of cardiac magnetic resonance (CMR), and identify patients at risk.

Background: Cardiac sarcoidosis is associated with increased morbidity and mortality. CMR is a key modality in the evaluation of patients with cardiac symptoms, but the complementary role of CMR to conventional tests for the diagnosis of cardiac sarcoidosis is not fully defined.

Respiratory dysfunction in multiple sclerosis.

Respiratory dysfunction frequently occurs in patients with advanced multiple sclerosis (MS), and may manifest as acute or chronic respiratory failure, disordered control of breathing, respiratory muscle weakness, sleep disordered breathing, or neurogenic pulmonary edema. The underlying pathophysiology is related to demyelinating plaques involving the brain stem or spinal cord. Respiratory complications such as aspiration, lung infections and respiratory failure are typically seen in patients with long-standing MS.

Evolution of cardiac dysfunction in patients with antiphospholipid antibodies and/or antiphospholipid syndrome: a 10-year follow-up study.

Objectives: To describe the evolution of valve involvement and myocardial dysfunction over time in patients with systemic lupus erythematosus (SLE) with or without antiphospholipid antibodies (aPL) and/or antiphospholipid syndrome (APS).

Methods: From an initial cohort of 150 patients assessed by transthoracic echocardiography 10 years ago, 17 patients with primary APS (PAPS), 23 with SLE-associated APS (SLE/APS), 19 with SLE positive for aPL without APS, and 23 with SLE negative for aPL were re-evaluated in the present echocardiography study.

Results: Valvulopathy was detected in 65% of PAPS and 62% of SLE patients with or without aPL.

Elevated pulmonary arterial systolic pressure in patients with sarcoidosis: prevalence and risk factors.

Background: Sarcoidosis-related pulmonary hypertension (SRPH) is an entity associated with significant morbidity and mortality irrespective of disease severity, while the pathogenic mechanisms remain poorly understood.

Methods: This cross-sectional study included consecutive patients with biopsy-proven sarcoidosis (n = 313) who were followed up in an outpatient setting from October 2002 through June 2010. All patients underwent clinical and cardiopulmonary evaluation, including cardiac MRI, to assess prevalence of SRPH and identify possible underlying pathophysiological mechanisms.

Activin-A overexpression in the murine lung causes pathology that simulates acute respiratory distress syndrome.

Rationale: Activin-A is up-regulated in various respiratory disorders. However, its precise role in pulmonary pathophysiology has not been adequately substantiated in vivo.

Objectives: To investigate in vivo the consequences of dysregulated Activin-A expression in the lung and identify key Activin-A-induced processes that contribute to respiratory pathology.

Portopulmonary hypertension and serum endothelin levels in hospitalized patients with cirrhosis.

Background: Cirrhosis is associated with several extrahepatic manifestations including portopulmonary hypertension (PPHT). Recent data suggest that endothelins (ETs) are related to the pathophysiology of PPHT. The study aimed to measure serum ET levels in hospitalized cirrhotic patients and to determine their association with PPHT and patient outcome.

The many faces of scleroderma sine scleroderma: a literature review focusing on cardiopulmonary complications.

Scleroderma sine scleroderma (ssSSc) is an occult form of systemic sclerosis that may cause diagnostic difficulties due to the absence of skin involvement. Delays in the diagnosis of ssSSc means lost opportunites to address and treat the often lethal involvement of internal organs such as the lungs and heart. In this systemic review we collected all published cases of ssSSc using EMBASE, MEDLINE, PubMed, and Web of Science from 1950 to present.

Association of the shrinking lung syndrome in systemic lupus erythematosus with pleurisy: a systematic review.

Objectives: To report 2 patients with systemic lupus erythematosus and typical shrinking lung syndrome (SLS) in which pleuritic chest pain was the predominant symptom. In addition, to record the prevalence of pleuritic chest pain in all reported cases of patients with SLS and diaphragmatic dysfunction.

Methods: We conducted a comprehensive search of the English literature to record the association of pleurisy and SLS in all reported cases using the MEDLINE database from 1965 to present.

Cryptogenic organizing pneumonia associated with primary Sjogren's syndrome.

The association of cryptogenic organizing pneumonia (COP) with primary Sjogren's syndrome (PSS) is extremely rare. We report a case of simultaneous diagnosis of PSS and COP. A 70-year-old female presented with fever, non-productive cough and dyspnea of 2 months' duration.

Relative contributions of the ribcage and abdomen to lung volume displacement during speech production.

We tested the hypothesis that the pattern of chest wall configuration during speech production correlates with the pattern of chest wall motion during resting breathing. Twenty-one men (age 40 +/- 8 years) with ankylosing spondylitis and varied degrees of ribcage involvement participated in the study. None of the patients had an obvious speech abnormality.

Pattern and distribution of myocardial fibrosis in systemic sclerosis: a delayed enhanced magnetic resonance imaging study.

Objective: To assess the prevalence and pattern of myocardial fibrosis as detected by delayed enhanced magnetic resonance imaging (DE-MRI) in patients with systemic sclerosis (SSc), and to evaluate a possible association between myocardial fibrosis and cardiac arrhythmias.

Methods: Forty-one patients with SSc underwent 24-hour Holter monitoring, Doppler echocardiography, and DE-MRI following gadolinium administration.

Results: Technically acceptable DE-MRIs were obtained in 36 patients with SSc.

Chest CT findings in patients with inflammatory myopathy and Jo1 antibodies.

Thoracic high-resolution computed tomography scans (HRCT) of 17 patients with inflammatory muscle disorders (IMD) and positive Jo1 antibodies were retrospectively reviewed regarding presence, extension, and distribution of pathological findings. Abnormal findings were found in 14 (82.3%) patients.

Chest wall motion during speech production in patients with advanced ankylosing spondylitis.

Purpose: To test the hypothesis that ankylosing spondylitis (AS) alters the pattern of chest wall motion during speech production.

Method: The pattern of chest wall motion during speech was measured with respiratory inductive plethysmography in 6 participants with advanced AS (5 men, 1 woman, age 45+/-8 years, Schober test 1.45+/-1.

Determinants of pulmonary arterial hypertension in scleroderma.

Objective: To define risk factors associated with pulmonary arterial hypertension (PAH) in a large cohort of patients with systemic sclerosis (SSc).

Methods: SSc patients undergoing screening for PAH by means of Doppler echocardiography were identified and their charts were retrospectively reviewed. In all patients, we recorded systolic pulmonary artery pressure along with pulmonary function testing, clinical, and laboratory data.

Inter-session reproducibility of peak expiratory flow with standardized expiratory maneuvers.

Background: In adults performing forceful expiratory maneuvers, the length of post-inspiratory pause prior to forced expiration may influence the subsequently measured peak expiratory flow (PEF) and increase its variability. We investigated the effects of two different lengths of breath-hold at total lung capacity (TLC) on the short-term reproducibility of PEF in healthy volunteers.

Methods: Forty-six healthy volunteers (age 34.

Scleroderma lung: initial forced vital capacity as predictor of pulmonary function decline.

Objective: To determine the ability of initial forced vital capacity (FVC) of patients with scleroderma to predict subsequent pulmonary function deterioration.

Methods: Data on 78 patients with scleroderma were retrospectively collected and analyzed. FVC (percent predicted), diffusing capacity for carbon monoxide (percent predicted), and various clinical and laboratory parameters were recorded.

Effects of theophylline on pulmonary function in patients with traumatic tetraplegia.

Background/objectives: To assess the effects of theophylline on pulmonary function in patients with chronic traumatic tetraplegia, we conducted a double-blind placebo-controlled crossover study in 10 patients.

Methods: The patients (age: 41 +/- 3 years; time from injury: 16 +/- 3 years; neurological levels: C3 to C7-T1) were randomized to receive oral theophylline or placebo for 6 weeks. After 2 months of washout, the patients received the medication not taken in the first trial for an additional 6 weeks.