A prospective, open-label, non-comparative study of ambrisentan with anti-fibrotic agent combination therapy in the treatment of diffuse systemic sclerosis.

Background: Systemic Sclerosis is a multifactorial autoimmune rheumatic disease characterized by inflammation, fibrosis, immune dysregulation and vascular dysfunction.

Methods: An open label, prospective, non-comparative study evaluating ambrisentan with an antifibrotic agent in diffuse cutaneous systemic sclerosis (dcSSc). Recruited 15 consecutive patients with dcSSc who were already on a stable dose of an antifibrotic agent and if they met inclusion criteria they were initiated on ambrisentan 5 mg/day for 12 months.

Initial presentation of acute transverse myelitis in systemic lupus erythematosus: demographics, diagnosis, management and comparison to idiopathic cases.

To describe and compare the diagnosis, demographics and management of systemic lupus erythematosus (SLE) related versus idiopathic acute transverse myelitis during the initial presentation of the disease. We undertook a chart review of the hospital records of patients admitted to our hospital from 1994 until 2007 and had the diagnosis of SLE related and idiopathic acute transverse myelitis. Demographics, laboratory and imaging studies, diagnosis and treatment were recorded in both groups and analyzed in a case control fashion.

Primary amyloidosis causing diffuse alveolar hemorrhage.

Diffuse alveolar hemorrhage is a serious complication that has been described in various disease states including several vasculitic syndromes as well as conditions that may resemble vasculitis clinically. In this article, we present a case of 79-year-old man, who was admitted with productive cough, blood-tinged sputum, and a positive atypical antineutrophil cytoplasmic antibody pattern on indirect immunofluorescence microscopy. He subsequently developed frank hemoptysis and respiratory failure.

Gastric antral vascular ectasia in systemic sclerosis: demographics and disease predictors.

Objectives: To evaluate patients with systemic sclerosis (SSc) who have gastric antral vascular ectasia (GAVE), to further characterize this disease association, and to identify factors that may predict which patients with SSc are at greatest risk for the development of GAVE.

Methods: Patients with a diagnosis of both SSc and GAVE were identified from the Division of Rheumatology at Georgetown University and Thomas Jefferson University. A chart review was conducted to obtain the demographic data.

A prospective open-label study of mycophenolate mofetil for the treatment of diffuse systemic sclerosis.

Objective: To evaluate the efficacy and safety of mycophenolate mofetil for the treatment of SSc.

Methods: We recruited 15 patients with dcSSc to take part in an open-label study using mycophenolate mofetil to treat their disease over a 12-month period. The primary outcome measure was the modified Rodnan skin score (mRSS), whereas secondary outcomes included the Medsger severity score, pulmonary function studies, 2D echocardiograms and the Short Form Health Survey (SF)-36 questionnaire.

The use of mycophenolate mofetil for the treatment of systemic sclerosis.

Mycophenolate mofetil (MMF) is an inosine monophosphate dehydrogenase inhibitor, that inhibits the de novo pathway of guanosine nucleotide synthesis, the proliferative responses of T and B lymphocytes as well as antibody production by B-lymphocytes. It is indicated for the prophylaxis of organ rejection after allogeneic cardiac, hepatic and renal transplants . It has recently also been used with good success in patients with lupus nephritis .