PubMed Health⌕ Search
Home/Archive/PubMed

PubMed · 16012875

Scleroderma associated with ANCA-associated vasculitis.

Young Hee Rho·Seong Jae Choi·Young Ho Lee·Jong Dae Ji·Gwan Gyu Song
View original source ↗Export metadata ↓

Abstract

Scleroderma and ANCA-associated vasculitides (AAV), such as microscopic polyangiitis, are distinct disease entities, but are rarely known to coexist with each other. We have reported on two cases of scleroderma patients for the first time in Korea, and these patients were initially known to have only limited type scleroderma with pulmonary fibrosis, but eventually they were found to be ANCA-positive with the associated clinical features of vasculitis. Both were treated with high-dose steroids and cyclophosphamide and remitted without major sequelae. When scleroderma patients exhibit atypical features such as normotensive renal failure with signs of active inflammation, the possibility of AAV should always be considered.

Explore related subjects

Antibodies, Antineutrophil CytoplasmicCyclophosphamideFemaleHumansImmunosuppressive AgentsMiddle AgedScleroderma, SystemicVasculitisCase ReportsJournal Article

Keep this discovery

Explore connections, maps & timelines

BibTeXRIS

Young Hee Rho, Seong Jae Choi, Young Ho Lee, Jong Dae Ji, Gwan Gyu Song. 2005-07-13. Scleroderma associated with ANCA-associated vasculitis.. https://doi.org/10.1007/s00296-005-0003-5

Cite the original work for its findings. Save a collection to share your selection of sources.

KEEP EXPLORING

Related citations

PubMed2007

The prevalence of genetic and serologic markers in an unselected European population-based cohort of IBD patients.

BACKGROUND AND AIM: The aetiology of inflammatory bowel disease (IBD) is unknown, but it has become evident that genetic factors are involved in disease susceptibility. Studies have suggested a north-south gradient in the incidence of IBD, raising the question whether this difference is caused by genetic heterogeneity. We aimed to investigate the prevalence of polymorphisms in CARD15 and TLR4 and occurrence of anti-Saccharomyces cerevisiae (ASCA) and antineutrophil cytoplasmic antibodies (pANCA) in a European population-based IBD cohort. METHODS: Individuals from the incident cohort were genotyped for three mutations in CARD15 and the Asp299gly mutation in TLR4. Levels of ASCA and pANCA were assessed. Disease location and behaviour at time of diagnosis was obtained from patient files. RESULTS: Overall CARD15 mutation rate was 23.9% for CD and 9.6% for UC patients (P < 0.001). Mutations were less present in the Scandinavian countries (12.1%) versus the rest of Europe (32.8%) (P < 0.001). Overall population attributable risk was 11.2%. TLR4 mutation rate was 7.6% in CD, 6.7% in UC patients and 12.3% in healthy controls (HC), highest among South European CD patients and HC. ASCA was seen in 28.5% of CD patients with no north-south difference, and was associated with complicated disease. pANCA was most common in North European UC patients and not associated with disease phenotype. CONCLUSION: The prevalence of mutations in CARD15 varied across Europe, and was not correlated to the incidence of CD. There was no association between mutations in TLR4 and IBD. The prevalence of ASCA was relatively low; however related to severe CD.

Antibodies, Antineutrophil Cytoplasmic↗
PubMed2007

The challenge of managing patients with polyarteritis nodosa.

PURPOSE OF REVIEW: In this short review we focus on the problems faced by clinicians caused by the changing definitions of polyarteritis nodosa. RECENT FINDINGS: The term polyarteritis nodosa has been used for more than 100 years as a diagnostic term for patients with systemic vasculitis; however, specific vasculitides have been singled out like branches being chopped off a tree. Now, so little is left of the trunk of that tree that it is questionable to what extent we can trust older literature with respect to clinical features, natural history and response to treatment. Many authors of case reports, as well as authors of reviews and book chapters, claim they adhere to the Chapel Hill Consensus Conference definition of polyarteritis nodosa, yet still cite almost exclusively studies using older definitions without highlighting this dilemma. In the past year, two proposals affecting classification have been published: one stating that cutaneous polyarteritis nodosa and hepatitis-associated polyarteritis nodosa are diseases distinct from classical polyarteritis nodosa, and one providing an algorithm to separate microscopic polyangiitis from classical polyarteritis nodosa. SUMMARY: There is hope that a wide acceptance of the new classification principles will lead to a more uniform way to diagnose classical polyarteritis nodosa, which will facilitate clinical studies and eventually improve management.

Antibodies, Antineutrophil Cytoplasmic↗
PubMed2007

Controversies in small vessel vasculitis--comparing the rheumatology and nephrology views.

Over the past 20 years, remarkable progress has been made in understanding the pathogenesis and treatment of patients with small vessel vasculitis. Nephrologists and rheumatologists play primary roles in the care of these patients. Whilst there are many areas of agreement between these specialists with respect to understanding and practical care of these patients, there are a number of important areas that differ in the practice patterns of nephrologic and rheumatologic practice. These areas include the nomenclature of these diseases, the role of antineutrophil cytoplasmic antibodies in the pathogenesis, the route and duration of initial cyclophosphamide administration, and, most importantly, the total duration of maintenance therapy following disease remission. Two experienced clinicians, a rheumatologist and a nephrologist, spar off in support of their opinions.

Antibodies, Antineutrophil Cytoplasmic↗