Rituximab for remission induction and maintenance in refractory granulomatosis with polyangiitis (Wegener's): ten-year experience at a single center.

Objective: This study was conducted to evaluate the efficacy and safety of repeated and prolonged B cell depletion with rituximab (RTX) for the maintenance of long-term remission in patients with chronic relapsing granulomatosis with polyangiitis (Wegener's) (GPA).

Methods: We conducted a single-center observational study of all patients with chronic relapsing GPA treated with at least 2 courses of RTX between January 1, 2000 and May 31, 2010. Participants in the Rituximab in ANCA-Associated Vasculitis (RAVE) trial were excluded from this analysis.

Intrathoracic manifestations of Rosai-Dorfman disease.

Introduction: Rosai-Dorfman Disease (RDD), also known as Sinus Histiocytosis with Massive Lymphadenopathy (SHML), is a rare monocyte/macrophage proliferative disorder of varied biological behavior. Although cutaneous and lymph node involvement are relatively well-described, intrathoracic manifestations of RDD have only occasionally been reported.

Methods: We conducted a retrospective computer-assisted search of the Mayo Clinic record from 1976 to 2005 for patients with histopathologic evidence of RDD on organ biopsy.

Obstructive sleep apnea, cardiovascular disease, and pulmonary hypertension.

With the growing epidemic of obesity in an aging population, obstructive sleep apnea (OSA) is increasingly encountered in clinical practice. Given the acute cardiopulmonary stressors consequent to repetitive upper airway collapse, as well as evidence for cardiovascular homeostatic dysregulation in subjects with sleep apnea, there is ample biologic plausibility that OSA imparts increased cardiovascular risk, independent of comorbid disease. Indeed, observational studies have suggested strong associations with multiple disorders, such as systemic hypertension, heart failure, cardiac arrhythmias, and pulmonary hypertension.

Airway involvement in Wegener's granulomatosis.

Wegener's granulomatosis is characterized by necrotizing granulomatous inflammation and necrotizing vasculitis affecting predominantly small arteries, arterioles, capillaries, and venules. In contrast to the well-described pulmonary parenchymal involvement of Wegener's granulomatosis, the lower airway (tracheobronchial) disease manifestations are less well recognized by clinicians. Consequently, mild disease of the airways is easily missed.

Targeting B lymphocytes as therapy for ANCA-associated vasculitis.

This article focuses on the initial results achieved with the more selective immunosuppressive approach of B-lymphocyte depletion in patients who fail cyclophosphamide or have contraindications for its use in the treatment of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). This novel approach has sparked hope for patients and physicians in their search for effective, well-tolerated therapy for AAV. B-cell depletion is now undergoing rigorous investigation in randomized clinical trials.

Causes and presenting features in 85 consecutive patients with hypersensitivity pneumonitis.

Objective: To assess the current spectrum of causes and clinical features associated with hypersensitivity pneumonitis (HP).

Patients And Methods: We studied consecutive patients with HP diagnosed at the Mayo Clinic in Rochester, Minn, from January 1, 1997, through December 31, 2002. Diagnostic criteria for HP included the following: (1) presence of respiratory symptoms, (2) radiologic evidence of diffuse lung disease, (3) known exposure or a positive serologic test result to an inciting antigen, and (4) no other identifiable cause for the lung disease.

Moving beyond empiric continuous positive airway pressure (CPAP) trials for central sleep apnea: a multi-modality titration study.

There is no universally accepted method to determine effective therapy for central sleep apnea (CSA). Continuous positive airway pressure (CPAP) applied acutely most often does not eliminate apneas and hypopneas. We hypothesized that the application of two or more therapeutic modalities after the diagnostic phase of polysomnography, a multi-modality titration study (MMTS), would identify a successful CSA treatment more often than a standard split-night study (SNS) and obviate the need for additional polysomnograms to determine a successful therapy.

Portopulmonary hypertension.

As a result of the success of orthotopic liver transplantation, there has been increasing interest in the diagnosis and therapeutic options for the pulmonary vascular complications of hepatic disease. These pulmonary vascular complications range from the hepatopulmonary syndrome, which is characterized by intrapulmonary vascular dilatations, to portopulmonary hypertension (POPH), which is characterized by an elevated pulmonary vascular resistance as a consequence of obstruction to pulmonary arterial blood flow. This review concentrates on POPH.