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Eosinophilic granulomatosis with polyangiitis (EGPA), formerly named Churg-Strauss syndrome, is a rare systemic small- and medium-sized-vessel vasculitis, characterized by the presence of severe asthma as well as blood and tissue eosinophilia. Gastrointestinal (GI) symptoms, like diarrhea and
OBJECTIVE
The aim of this study was to describe the use of mycophenolate mofetil (MMF) in the treatment of patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis with ocular involvement.
METHODS
A retrospective review was performed. Ocular and systemic manifestations, history
Thrombotic thrombocytopenic purpura (TTP) rarely occurs with systemic vasculitis. A 17-year-old girl presented with non-bloody diarrhea, menorrhagia, and syncope. She had severe anemia (hemoglobin = 3.8 g/dl), thrombocytopenia (platelet = 7,000/mm(3)), and acute kidney injury (serum creatinine, Cr =
Rituximab is recommended to induce remission of severe granulomatosis with polyangiitis (GPA). Plasma exchange (PE) may be considered in the setting of rapidly progressive glomerulonephritis (RPGN) with a serum creatinine increase of more than 5.6 mg/dl or diffuse alveolar hemorrhage Eosinophilic granulomatosis with polyangiitis (EGPA), or Churg-Strauss syndrome, is a rare disease characterized by disseminated necrotizing vasculitis with extravascular granulomas occurring among patients with asthma and tissue eosinophilia. Clinically, it presents in various ways and progresses
We reviewed the medical records of 62 patients with systemic small and medium-sized vessel vasculitides and gastrointestinal tract involvement followed at our institution between 1981 and 2002. This group included 46 men and 16 women (male:female ratio, 2.9), with a mean age of 48 +/- 18 years.
Wegener granulomatosis (WG) is an idiopathic small vessel vasculitis involving primarily airway and kidneys. Intestinal involvement of WG is rare and usually occurs after several years of WG and its treatment. We report a case of WG, presented initially as rapid-onset hemorrhagic pancolitis without
Leflunomide, a new oral immunomodulatory agent, is effective for the treatment of rheumatoid arthritis. Its mechanism of action in suppressing inflammation is based in its inhibition of dihydroorotate dehydrogenase, an enzyme responsible for de novo synthesis of pyrimidine containing
BACKGROUND Pyogenic arthritis, pyoderma gangrenosum (PG), acne, and suppurative hidradenitis (PA-PASH) syndrome has been linked to an auto-inflammatory pathway. We report a case that is an atypical presentation of a rare syndrome, which supports literature suggesting that different phenotypes of
This study describes the pharmacokinetics of mycophenolate mofetil (MMF) in 15 pediatric patients with vasculitis and connective tissue disease involving the kidney. Patients included 10 with systemic lupus erythematosus (SLE), 1 with antiphospholipid antibody syndrome, 2 with Wegener
Wegener's granulomatosis, also known as anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis, is a small vessel vasculitis with primarily pulmonary, renal, and sinus disease manifestations. The prevalence of Wegener's granulomatosis is three cases per 100,000 patients. Cardiovascular,
In an autoimmune disease, the immune system attacks and harms the body's own tissues. The systemic autoimmune diseases include collagen vascular diseases, the systemic vasculitides, Wegener granulomatosis, and Churg-Strauss syndrome. These disorders can involve any part of the gastrointestinal
Background: Whether patients with autoimmune rheumatic diseases (ARD) have a higher risk for SARS-CoV-2 infection (COVID-19) and how SARS-CoV-2 pandemic impacts on adherence to therapy has not been fully elucidated. We assessed the rate
We report a 16-year-old previously healthy boy who was admitted to hospital with fever, constitutional symptoms, purpura, additive arthritis, dysentery, rapid progressive renal failure, resembling Henoch- Schuenlein purpura, accompanied with retropharyngeal abscess. Kidney biopsy revealed rapid