Sarcoidosis of the upper and lower airways.

Sarcoidosis is a systemic granulomatous disease of undetermined etiology characterized by a variable clinical presentation and disease course. Although clinical granulomatous inflammation may occur within any organ system, more than 90% of sarcoidosis patients have lung disease. Sarcoidosis is considered an interstitial lung disease that is frequently characterized by restrictive physiologic dysfunction on pulmonary function tests.

Sarcoidosis diagnosed after September 11, 2001, among adults exposed to the World Trade Center disaster.

Objective: Explore relationships between World Trade Center (WTC) exposures and sarcoidosis.

Methods: Sarcoidosis has been reported after exposure to the WTC disaster. We ascertained biopsy-proven post-9/11 sarcoidosis among WTC Health Registry enrollees.

"Sarcoid like" granulomatous pulmonary disease in World Trade Center disaster responders.

Background: More than 20,000 responders have been examined through the World Trade Center (WTC) Medical Monitoring and Treatment Program since September 11, 2001. Studies on WTC firefighters have shown elevated rates of sarcoidosis. The main objective of this study was to report the incidence of "sarcoid like" granulomatous pulmonary disease in other WTC responders.

Primary prevention of sudden cardiac death in silent cardiac sarcoidosis: role of programmed ventricular stimulation.

Background: Cardiac involvement in sarcoidosis is often silent and may lead to sudden death. This study was designed to assess the value of programmed electric stimulation of the ventricle (PES) for risk stratification in patients with sarcoidosis and evidence of preclinical cardiac involvement on imaging studies.

Methods And Results: Patients with biopsy-proven systemic sarcoidosis but without cardiac symptoms who had evidence of cardiac sarcoidosis on positron emission tomography (PET) or cardiac MRI (CMR) were included.

Pulmonary manifestations of light chain deposition disease.

Light chain deposition disease (LCDD) is a rare condition characterized by extracellular light chain deposition in tissues. Patients commonly have an underlying plasma cell dyscrasia, and produce excess levels of monoclonal light chains. Renal involvement is the most common clinical manifestation.

"End-stage" pulmonary fibrosis in sarcoidosis.

Pulmonary fibrosis is an unusual "end stage" in patients with sarcoidosis. Fibrosis occurs in a minority of patients, and presents with a unique physiologic combination of airways dysfunction (obstruction) superimposed on the more common restrictive dysfunction. Imagin techniques are essential to the diagnosis, assessment and treatment of pulmonary fibrosis.

Fluoro-deoxy-glucose positron emission tomography for evaluation of indeterminate lung nodules: assigning a probability of malignancy may be preferable to binary readings.

Objective: To assess the diagnostic value of fluorine-18 fluoro-2-deoxy-D-glucose positron emission tomography (FDG-PET) using standard uptake values (SUV) in the differential diagnoses of indeterminate pulmonary nodules. Specifically, we assessed the probability of malignancy for various SUV ranges, and compared the diagnostic efficacy of SUV with and without correction for partial volume effects on the basis of lesion size.

Methods: The FDG-PET scans performed on 158 patients with biopsy-proven pulmonary lesions seen on computed tomography (CT) scan were retrospectively reviewed.

Chronic facial sarcoidosis including lupus pernio: clinical description and proposed scoring systems.

Background: Facial lesions including lupus pernio are often a form of chronic cutaneous sarcoidosis.

Objective: To evaluate the intra- and inter-observer consistency of objective measures of chronic facial lesions.

Method: This was a retrospective study of patients with chronic cutaneous facial lesions including lupus pernio.

Cardiac involvement in patients with sarcoidosis: diagnostic and prognostic value of outpatient testing.

Background: Cardiac sarcoidosis (CS) causes substantial morbidity and sudden death. Early diagnosis and risk stratification are warranted.

Methods: Ambulatory patients with sarcoidosis were interviewed to determine whether they experienced palpitations, syncope, or presyncope, and were evaluated with ECG, Holter monitoring, and echocardiography (transthoracic echocardiogram [TTE]).

A double-blinded, randomized, placebo-controlled trial of infliximab in subjects with active pulmonary sarcoidosis.

Study Objectives: To evaluate the safety and tolerability of infliximab in the treatment of active pulmonary sarcoidosis, and to provide an initial assessment of the efficacy of infliximab in the treatment of active pulmonary sarcoidosis.

Design: Double-blind, randomized, placebo-controlled phase II study.

Setting: Multicenter.

Results of 188 whole-body fluorodeoxyglucose positron emission tomography scans in 137 patients with sarcoidosis.

Background: To study the role of whole-body 18F-fluorodeoxyglucose (FDG) positron emission tomography (PET) scans in the identification of occult biopsy sites and reversible granulomatous disease in patients with sarcoidosis.

Methods: A retrospective review was undertaken of 188 FDG PET scans performed in 137 patients with proven sarcoidosis. All patients had given a complete medical history and undergone a physical examination, standard chest radiograph, spirometry, diffusing capacity determination, and measurement of serum angiotensin-converting enzymes levels.

Late recurrent pulmonary typical carcinoid tumor: case report and review of the literature.

Carcinoid tumors are uncommon pulmonary neoplasms. They are classified histologically as either atypical or typical. Atypical carcinoids are aggressive malignancies that require radical surgical resection and have a guarded prognosis with a propensity to metastasize and recur.

Comparison of sarcoidosis phenotypes among affected African-American siblings.

Study Objective: To test the hypothesis that sibling pairs, who share genes and environmental exposures, might have similar phenotypic expressions of sarcoidosis beyond what would be expected by chance alone.

Design: Multicenter family study with study subjects recruited from 11 clinical centers.

Subjects: Subjects were African-American sibling pairs with sarcoidosis.

Distinctive clinical, radiographic, and functional characteristics of patients with sarcoidosis-related pulmonary hypertension.

Study Objective: To differentiate the clinical, radiographic, and physiologic profile in patients with sarcoidosis with and without pulmonary hypertension.

Design: Retrospective survey.

Setting: Tertiary care center.

Neuromuscular sarcoidosis.

Neuromuscular sarcoidosis is recognized with increasing frequency. Although reported to occur in only 5% of patients with sarcoidosis, major centers are reporting up to 20% neuromuscular involvement. While the majority of patients with neurologic involvement present with 7 (th) nerve palsy, the reported frequency of sarcoidosis of the brain, meninges, spinal cord, and muscles is rising.

Pulmonary and psychosocial findings at enrollment in the ACCESS study.

Aim: To assess lung involvement and the association of demographic and psychosocial factors with respiratory health in 736 persons with sarcoidosis at enrollment in A Case Control Etiologic Study of Sarcoidosis (ACCESS).

Methods: 736 patients with biopsy diagnosis of sarcoidosis within 6 months of enrollment were studied at 10 US centers. Lung involvement was evaluated by chest radiography, spirometry and dyspnea questionnaire.

The spectrum of biopsy sites for the diagnosis of sarcoidosis.

Background: The diagnosis of sarcoidosis is most secure when supported by a tissue biopsy exhibiting noncaseating epithelioid granulomas with absence of known granulomagenic agents in a patient with multi-organ disease. Clinicians must decide which site offers the best chance of achieving a diagnostic biopsy with the least patient risk and discomfort.

Methods: 736 cases were enrolled in the NHLBI supported A Case Controlled Etiologic Study of Sarcoidosis (ACCESS) from November 1996 to June 1999.

A sarcoidosis genetic linkage consortium: the sarcoidosis genetic analysis (SAGA) study.

Background: Sarcoidosis, a systemic granulomatous disease of unknown etiology, likely results from an environmental insult in a genetically susceptible host. In the United States of America, African Americans have a higher sarcoidosis incidence and suffer greater morbidity than Caucasians.

Methods: A sarcoidosis genetic linkage study consortium was established to recruit African-American affected sib pair (ASP) families to identify chromosomal regions that may harbor sarcoidosis susceptibility genes and to determine if environmental factors modify any genetic effects.

Usefulness of programmed ventricular stimulation in predicting future arrhythmic events in patients with cardiac sarcoidosis.

The utility of programmed ventricular stimulation to predict future arrhythmic events in patients with cardiac sarcoidosis is unknown. Similarly, the long-term benefit of implantable cardioverter-defibrillators (ICDs) in cardiac sarcoidosis has not been established. Thirty-two consecutive patients with cardiac sarcoidosis underwent programmed ventricular stimulation.

MRI of sarcoidosis patients with musculoskeletal symptoms.

Objective: Our objective was to determine MRI findings in sarcoidosis patients with musculoskeletal symptoms.

Conclusion: In sarcoidosis patients with musculoskeletal complaints, MRI reveals marrow and soft-tissue lesions that are occult or underestimated on radiographs. Axial and large-bone lesions may resemble osseous metastases on MRI.