Autoantibodies against small nucleolar ribonucleoprotein complexes and their clinical associations

Eenennaam, Hans van; Vogelzangs, Judith; Bisschops, Laurens L. A.; Boome, L.C.J. te; Seelig, H. P.; Renz, Manfred; Rooij, D. J. De; Brouwer, R.; Pluk, Helma; Pruijn, Ger J. M.; Venrooij, Walther J. van; Hoogen, F.H.J. van den

doi:10.1046/j.1365-2249.2002.01991.x

Cited by 73 publications

(60 citation statements)

References 47 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…A predominantly nucleolar pattern of ANAs is seen with anti-U3 RNP, anti-PM-Scl, and anti-Th/To antibodies (27,28). Nucleolar staining has been observed in 8-48% of SSc patient sera (6-10), 12-52% of which have been positive for anti-U3 RNP antibodies (6,9,12,13,27,29).…”

Section: Discussionmentioning

confidence: 99%

Anti–U3 RNP autoantibodies in systemic sclerosis

Aggarwal

Lucas

Fertig

et al. 2009

Arthritis & Rheumatism

134

View full text Add to dashboard Cite

Objective. To describe the classification, demographic and clinical features, and survival in anti-U3 RNP autoantibody-positive patients with systemic sclerosis (SSc).Methods. Results. The anti-U3 RNP-positive group had a higher proportion of African American patients (27% versus 5%; P < 0.001) and male patients (29% versus 19%; P ‫؍‬ 0.021), and was younger at the time of first physician diagnosis (mean age 42.8 years versus 47.4 years; P ‫؍‬ 0.001). The 2 groups had similar proportions of patients with diffuse cutaneous involvement (47% and 45% in those with and those without anti-U3 RNP, respectively). However, among patients with diffuse cutaneous involvement, the mean maximum modified Rodnan skin score was significantly lower in the anti-U3 RNP group (22.3 versus 27.9; P < 0.001). Skeletal muscle involvement was more frequent in anti-U3 RNP-positive patients (25% versus 14%; P ‫؍‬ 0.002), as was "intrinsic" pulmonary arterial hypertension (PAH) (31% versus 13%; P < 0.001). The frequency of gastrointestinal involvement, cardiac involvement, pulmonary fibrosis, and "renal crisis" did not differ significantly between the 2 groups. Survival was worse in the anti-U3 RNP-positive group (hazard ratio 1.38 [95% confidence interval 1.05-1.82]). PAH was the most common known cause of death in patients with anti-U3 RNP (30%, versus 10% in the anti-U3 RNP-negative group; P < 0.001).Conclusion. The present findings demonstrate that the frequencies of African American race and male sex are greater among SSc patients with anti-U3 RNP antibody than those without, and the former group is younger at SSc diagnosis. Anti-U3 RNP-positive patients have more frequent skeletal muscle involvement and PAH, the latter being the most common cause of death.

show abstract

Section: Discussionmentioning

confidence: 99%

Anti–U3 RNP autoantibodies in systemic sclerosis

Aggarwal

Lucas

Fertig

et al. 2009

Arthritis & Rheumatism

134

View full text Add to dashboard Cite

show abstract

“…Although these antibodies are considered specific for scleroderma, antinucleolar antibodies can occur in other autoimmune diseases, such as SLE, PM or dermatomyositis, and RA [17]. The first of the antinucleolar antibodies to be characterized in 1977 were the anti-polymyositis/scleroderma (anti-PM/ Scl) antibodies.…”

Section: Antinucleolar Antibodiesmentioning

confidence: 99%

“…The particles can be divided into three groups according to conserved RNA sequences-box ribonuclease multidrug resistance protein/ribonuclease P, box C/D, and box H/ ACA snoRNPs [17].…”

Section: Antibodies To Small Nucleolar Ribonucleoprotein Particlesmentioning

confidence: 99%

Antibodies in scleroderma: Direct pathogenicity and phenotypic associations

Chung

Utz²

2004

Curr Rheumatol Rep

View full text Add to dashboard Cite

Scleroderma is an autoimmune disease involving endothelial cell damage and fibroblast overproduction of extracellular matrix. Several autoantibodies present in the sera of patients with scleroderma, including anti-endothelial cell, antifibroblast, anti-matrix metalloproteinase, and antifibrillin-1 antibodies, may directly contribute to disease pathogenesis. Scleroderma also is characterized by the presence of antinuclear and antinucleolar antibodies, which correlate with particular phenotypes. These include antitopoisomerase-I, anticentromere, antihistone, anti-polymyositis/scleroderma, anti-Th/To, anti-U3-small nucleolar ribonucleoprotein particle, anti-U1-small nuclear ribonucleoprotein particle, anti-RNA polymerase, and anti-B23 antibodies. Other antibodies classically associated with other autoimmune diseases, such as antiphospholipid, antineutrophil cytoplasmic, and antimitochondrial antibodies, also have been described in patients with scleroderma. This review will summarize the various autoantibodies associated with scleroderma, their putative pathogenic roles, and their phenotypic correlations.

show abstract

“…They are present in a minority of patients with SSc (2-5 %) and seem to characterize a novel subset of patients with limited skin involvement (similar to ACA) but high risk for severe organ involvement and worse prognosis [17,42,60]. Severe pulmonary fibrosis preceding PAH in the same patient as well as renal crisis without interstitial lung disease may be associated with these antibodies [17,60,61].…”

Section: Anti-th/to Antibodiesmentioning

confidence: 99%

Autoantikörper bei systemischer Sklerodermie: Bedeutung für Diagnostik, Prognose und Pathogenese

Grassegger¹,

Pohla‐Gubo

Frauscher

et al. 2008

Wien Med Wochenschr

View full text Add to dashboard Cite

Systemic sclerosis is a generalized autoimmune connective tissue disease of unknown aetiology. Profound vascular and immunological dysregulations result in tissue fibrosis affecting the skin and internal organs. Currently, two main clinical subtypes are distinguished, i.e. limited and diffuse cutaneous systemic sclerosis, which differ significantly in the clinical course and prognosis. Autoantibodies against topoisomerase (Scl-70), centromere-associated proteins, and nucleolar antigens are important for the diagnosis of the disease and give clues for its clinical manifestations and prognosis (prognostic autoantibodies). For most of these antibodies, however, the role in pathogenesis is not established. Anti-centromere antibodies are associated with limited cutaneous involvement and risk for pulmonary hypertension, whereas anti-topoisomerase I is associated with diffuse progressive disease and severe interstitial lung disease. Anti-Th/To positivity is associated with limited skin involvement but a high risk for severe internal organ involvement (Kidneys, PAH, Lung fibrosis). Anti-RNA polymerase I/III antibodies are associated with a high risk for renal involvement. Autoantibodies against the PDGF receptor and fibrillin-1 seem to play important roles in the pathogenetic process of systemic sclerosis.

show abstract

Autoantibodies against small nucleolar ribonucleoprotein complexes and their clinical associations

Cited by 73 publications

References 47 publications

Anti–U3 RNP autoantibodies in systemic sclerosis

Anti–U3 RNP autoantibodies in systemic sclerosis

Antibodies in scleroderma: Direct pathogenicity and phenotypic associations

Autoantikörper bei systemischer Sklerodermie: Bedeutung für Diagnostik, Prognose und Pathogenese

Contact Info

Product

Resources

About