Read by QxMD icon Read

juvenile granulomatosis with polyangiitis

Saara Rawn, Paivi Miettunen, Holly A Brown, Heinrike Schmeling
UNLABELLED: We present a case of a 14-year-old girl who had a severe form of granulomatosis with polyangiitis (GPA) with extensive dermatological involvement, whose initial presentation was nonspecific leading to diagnostic confusion and initial consideration of infectious and other vasculitis causes. The patient presented with fever, congestion, malaise, and sinus pain. She was diagnosed with bacterial sinusitis and treated with antibiotics. Within weeks, she developed abdominal pain, hematuria, migratory arthritis, and palpable purpura and was diagnosed with Henoch-Schonlein purpura...
December 2014: European Journal of Pediatrics
L Allard, J-L Giniès, G Champion, A Chevailler, J-F Subra
BACKGROUND: Antineutrophil cytoplasmic antibodies (ANCA) are usually considered as serological markers of vasculitis (microscopic polyangiitis, granulomatosis with polyangiitis, and Churg-Strauss syndrome), but they have also been described in other diseases. They are rarely observed in children. Therefore, this study aims to describe the clinical spectrum associated with positive ANCA in children. PATIENTS AND METHODS: All the children below 15 years of age, admitted to the Angers University Hospital between June 2004 and June 2010 presenting with an ANCA-positive test by indirect immunofluorescence were included in this study...
March 2013: Archives de Pédiatrie: Organe Officiel de la Sociéte Française de Pédiatrie
Elisabett Miserocchi, Giulio Modorati
Rituximab (RTX) is a monoclonal antibody directed against the CD20 antigen expressed on B cells. This drug has been successfully employed in the treatment of non-Hodgkin's lymphoma and different systemic autoimmune diseases such as rheumatoid arthritis, systemic lupus erythematosus, granulomatosis with polyangiitis (Wegener's) and anti-neutrophil cytoplasmic antibody-associated vasculitis. At present, RTX may be used in patients with rheumatoid arthritis who qualify for treatment with tumor necrosis factor blockers and have had an inadequate response or intolerance to one or more of these agents...
2012: Developments in Ophthalmology
Juan Jakez-Ocampo, Jacqueline Ramírez, Carmen María Paulín-Vera, Luis Felipe Flores-Suárez, Edgardo Reyes, Luis Llorente
We report two cases of long-term juvenile idiopathic arthritis which developed recurrent subglottic stenosis secondary to localized Wegener's granulomatosis. In both cases, the biopsies taken from the affected region shown granulomatous vasculitis with multinucleated giant cells. Both patients presented positive serology for antineutrophil cytoplasmic antibodies by immunofluorescence (c-ANCA) and ELISA (PR3). The involvement or other organs was ruled out at the time of Wegener's granulomatosis diagnosis and during follow-up...
July 2010: Joint, Bone, Spine: Revue du Rhumatisme
Francesco Zulian, Marianna Sari, Cosimo de Filippis
Rheumatic diseases represent a group of autoimmune conditions which primarily affect the musculo-skeletal system but can also involve other internal organs such as the auditory and the respiratory systems. Among the rheumatic diseases of children those which present an otolaryngological involvement at disease onset or during their course are essentially juvenile idiopathic arthritis (JIA), Cogan syndrome (CS), relapsing polycondritis (RPC) and Wegener granulomatosis (WG). In this section, we will review the main characteristics of these conditions with the attempt to propose a few elements for an easy differential diagnosis which might help for an early diagnosis and a more appropriate treatment...
December 2009: International Journal of Pediatric Otorhinolaryngology
Patricia Woo
PURPOSE OF REVIEW: The clinical practice of introducing anti-inflammatory therapies in paediatric autoimmune disorders has changed substantially in the last two decades. This is partly due to the fact that we are able to put disease into remission with potent drugs, and so the issue of when to introduce these drugs is important. This review will seek to highlight the consequences of chronic inflammation and the change to outcomes if adequate or 'aggressive' treatment is given early to induce remission...
September 2009: Current Opinion in Rheumatology
J Brunner, M Freund, M Prelog, E Binder, M Sailer-Hoeck, T Jungraithmayr, C Huemer, C Sergi, L B Zimmerhackl
Microscopic polyangiitis (MPA) previously called hypersensitivity angiitis is a systemic necrotizing vasculitis affecting predominantly small vessels. MPA involves multiple organ systems including the lung, the kidneys, the joints, and the skin. MPA mostly affects adults in their fourth and fifth decade of life. MPA and Wegener;s granulomatosis are grouped together as ANCA-associated vasculitis. MPA is associated with high titre of myeloperoxidase antineutrophil cytoplasmic antibodies (MPO)-ANCA. We present a 14-year-old female patient presented with MPA...
August 2009: Clinical Rheumatology
D A Akkad, P Jagiello, P Szyld, R Goedde, S Wieczorek, W L Gross, J T Epplen
We analysed whether the single nucleotide polymorphism (SNP) rs3087456 in the promoter of the MHC class II transactivator (MHC2TA) gene is associated with manifestation of rheumatoid arthritis, multiple sclerosis, narcolepsy and Wegener granulomatosis. The recently reported association in a northern population of the MHC2TA variation with these autoimmune diseases is not evident in the German population.
February 2006: International Journal of Immunogenetics
Mark C Genovese, Joel M Kremer
Etanercept is effective in the treatment of rheumatoid arthritis (RA)when used as monotherapy and in combination with methotrexate(MTX). Radiographic progression of disease was slowed significantly when the drug was used for a 24-month period and was statistically significantly better than MTX. In addition to its use in RA,etanercept is approved by the U.S. Food and Drug Administration for other rheumatologic conditions, including psoriatic arthritis,ankylosing spondylitis, and juvenile chronic arthritis.
May 2004: Rheumatic Diseases Clinics of North America
Marianne Frieri
There are several rheumatologic and autoimmune disorders that can masquerade as allergic disease. Identification of these conditions in an office setting can be a challenge for the practicing allergist-immunologist. These conditions include rheumatoid and juvenile arthritis, Sjogren's syndrome, systemic lupus erythematosus, Behcet's and antiphospholipid syndromes, systemic sclerosis, vasculitis, sarcoidosis, chronic fatigue syndrome, and fibromyalgia. The article will address these topics and include clinical uses of immunologic tests for diagnosis...
November 2003: Allergy and Asthma Proceedings:
Fetch more papers »
Fetching more papers... Fetching...
Read by QxMD. Sign in or create an account to discover new knowledge that matter to you.
Remove bar
Read by QxMD icon Read

Search Tips

Use Boolean operators: AND/OR

diabetic AND foot
diabetes OR diabetic

Exclude a word using the 'minus' sign

Virchow -triad

Use Parentheses

water AND (cup OR glass)

Add an asterisk (*) at end of a word to include word stems

Neuro* will search for Neurology, Neuroscientist, Neurological, and so on

Use quotes to search for an exact phrase

"primary prevention of cancer"
(heart or cardiac or cardio*) AND arrest -"American Heart Association"