A Rare Case of Anti-RNA Polymerase III Autoantibody-Associated Interstitial Lung Disease in Systemic Sclerosis Sine Scleroderma

2019 ◽  
Author(s):  
W.S. Tam ◽  
A.M. Nambiar
Keyword(s):  
Systemic Sclerosis ◽  
Interstitial Lung Disease ◽  
Rna Polymerase ◽  
Lung Disease ◽  
Rare Case ◽  
Rna Polymerase Iii ◽  
Polymerase Iii

Systemic Sclerosis Sine Scleroderma: A Multicenter Study of 1417 Subjects

2014 ◽  
Vol 41 (11) ◽  
pp. 2179-2185 ◽  
Author(s):  
Sehriban Diab ◽  
Nathaniel Dostrovsky ◽  
Marie Hudson ◽  
Solène Tatibouet ◽  
Marvin J. Fritzler ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Interstitial Lung Disease ◽  
Rna Polymerase ◽  
Lung Disease ◽  
Multicenter Study ◽  
Research Group ◽  
Rna Polymerase Iii ◽  
Skin Involvement ◽  
Anticentromere Antibodies ◽  
Clinical Profiles

Objective.To describe the clinical and serological features of systemic sclerosis sine scleroderma (ssSSc) in a multicentered SSc cohort.Methods.Data from 1417 subjects in the Canadian Scleroderma Research Group registry were extracted to identify subjects with ssSSc, defined as SSc diagnosed by an expert rheumatologist, but without any sclerodactyly or skin involvement prior to baseline study visit or during followup. Clinical and serological features of ssSSc subjects were compared to limited (lcSSc) and diffuse cutaneous SSc (dcSSc) subjects.Results.At the first registry visit, only 57 subjects (4.0%) were identified as having ssSSc. Of these, 30 (2.1%) were reclassified as lcSSc within 1.9 years. Thus, only 27 ssSSc subjects (1.9%) remained, with mean followup of 2.4 years. Clinical profiles of ssSSc were generally similar or milder compared to lcSSc, and milder than dcSSc, including rates of interstitial lung disease (25.9% ssSSc, 25.4% lcSSc, 40.3% dcSSc). Patients with ssSSc had serological profiles similar to those with lcSSc, including high rates of anticentromere antibodies (50.0% ssSSc, 47.5% lcSSc, 12.1% dcSSc), and low rates of antitopoisomerase I (16.7% ssSSc, 7.0% lcSSc, 21.8% dcSSc) and anti-RNA polymerase III (0 ssSSc, 11.1% lcSSc, 34.9% dcSSc).Conclusion.The condition ssSSc is rare and resembles lcSSc. These observations suggest that ssSSc is most likely a forme fruste of lcSSc, and that the absence of skin involvement may in part be related to misclassification arising from early or subtle skin involvement. There is little evidence to consider ssSSc as a distinct clinical or serological subset of SSc.

Association of anti-RNA polymerase III antibody and silicone breast implants in patients with systemic sclerosis

2016 ◽  
Vol 43 (7) ◽  
pp. 808-810 ◽  
Author(s):  
Ryosuke Saigusa ◽  
Yoshihide Asano ◽  
Kouki Nakamura ◽  
Takashi Yamashita ◽  
Yohei Ichimura ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Rna Polymerase ◽  
Rna Polymerase Iii ◽  
Breast Implants ◽  
Silicone Breast Implants ◽  
Polymerase Iii

scholarly journals A longitudinal study of anti-RNA polymerase III antibody levels in systemic sclerosis

Rheumatology ◽  
2009 ◽  
Vol 48 (10) ◽  
pp. 1218-1221 ◽  
Author(s):  
S. I. Nihtyanova ◽  
J. C. Parker ◽  
C. M. Black ◽  
C. C. Bunn ◽  
C. P. Denton
Keyword(s):  
Longitudinal Study ◽  
Systemic Sclerosis ◽  
Rna Polymerase ◽  
Rna Polymerase Iii ◽  
Polymerase Iii ◽  
Antibody Levels

scholarly journals AB0668 ASSOCIATION OF ANTI-RNA POLYMERASE III ANTIBODY AND BREAST IMPLANTS RUPTURE IN ITALIAN PATIENTS WITH SYSTEMIC SCLEROSIS

2019 ◽  
Author(s):  
Maria Grazia Lazzaroni ◽  
Cristian Caimmi ◽  
Eugenia Bertoldo ◽  
Franco Franceschini ◽  
Angela Tincani ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Rna Polymerase ◽  
Rna Polymerase Iii ◽  
Breast Implants ◽  
Polymerase Iii

Analysis of Anti-RNA Polymerase III Antibody-positive Systemic Sclerosis and Altered GPATCH2L and CTNND2 Expression in Scleroderma Renal Crisis

2020 ◽  
Vol 47 (11) ◽  
pp. 1668-1677
Author(s):  
Edward P. Stern ◽  
Sandra G. Guerra ◽  
Harry Chinque ◽  
Vanessa Acquaah ◽  
David González-Serna ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Rna Polymerase ◽  
Renal Biopsy ◽  
Genetic Susceptibility ◽  
Rna Polymerase Iii ◽  
Human Kidney ◽  
Scleroderma Renal Crisis ◽  
Nucleotide Polymorphisms ◽  
Polymerase Iii ◽  
Renal Crisis

ObjectiveScleroderma renal crisis (SRC) is a life-threatening complication of systemic sclerosis (SSc) strongly associated with anti-RNA polymerase III antibody (ARA) autoantibodies. We investigated genetic susceptibility and altered protein expression in renal biopsy specimens in ARA-positive patients with SRC.MethodsARA-positive patients (n = 99) with at least 5 years’ follow-up (49% with a history of SRC) were selected from a well characterized SSc cohort (n = 2254). Cases were genotyped using the Illumina Human Omni-express chip. Based on initial regression analysis, 9 single-nucleotide polymorphisms (SNP) were chosen for validation in a separate cohort of 256 ARA-positive patients (40 with SRC). Immunostaining of tissue sections from SRC or control kidney was used to quantify expression of candidate proteins based upon genetic analysis of the discovery cohort.ResultsAnalysis of 641,489 SNP suggested association of POU2F1 (rs2093658; P = 1.98 × 10−5), CTNND2 (rs1859082; P = 5.58 × 10−5), HECW2 (rs16849716; P = 1.2 × 10−4), and GPATCH2L (rs935332; P = 4.92 × 10−5) with SRC. Further, the validation cohort showed an association between rs935332 within the GPATCH2L region, with SRC (P = 0.025). Immunostaining of renal biopsy sections showed increased tubular expression of GPATCH2L (P = 0.026) and glomerular expression of CTNND2 (P = 0.026) in SRC samples (n = 8) compared with normal human kidney controls (n = 8), despite absence of any genetic replication for the associated SNP.ConclusionIncreased expression of 2 candidate proteins, GPATCH2L and CTNND2, in SRC compared with control kidney suggests a potential role in pathogenesis of SRC. For GPATCH2L, this may reflect genetic susceptibility in ARA-positive patients with SSc based upon 2 independent cohorts.

scholarly journals Malignancies in Italian Patients with Systemic Sclerosis Positive for Anti-RNA Polymerase III Antibodies

2011 ◽  
Vol 38 (7) ◽  
pp. 1329-1334 ◽  
Author(s):  
PAOLO AIRO’ ◽  
ANGELA CERIBELLI ◽  
ILARIA CAVAZZANA ◽  
MARA TARABORELLI ◽  
STEFANIA ZINGARELLI ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Rna Polymerase ◽  
Temporal Relationship ◽  
Rna Polymerase Iii ◽  
Patients With Cancer ◽  
Polymerase Iii ◽  
Number Of Patients ◽  
I 11 ◽  
Anticentromere Antibodies ◽  
Close Temporal Relationship

Objective.To evaluate the frequency of malignancies in Italian patients with systemic sclerosis (SSc) and anti-RNA polymerase III (RNAP III), antitopoisomerase I (topo I), or anticentromere antibodies (ACA); and to characterize the temporal relationship between the 2 diseases, in order to confirm data suggesting a close temporal relationship between the onset of SSc and malignancy in American patients with anti-RNAP III antibodies.Methods.From a cohort of 466 consecutive SSc patients, 360 Italians with isolated positivity for anti-RNAP III (n = 16), anti-topo I (n = 101), or ACA (n = 243) were identified. Malignancy cases were divided according to their relationship with SSc onset into 3 categories: preceding, synchronous with, or metachronous to the onset of SSc (diagnosed more than 6 months before; 6 months before to 12 months after; and more than 12 months after onset of SSc, respectively).Results.Malignancies were more frequent in the anti-RNAP III group (7/16 patients), than in the anti-topo I (11/101) and ACA groups (21/243) (p < 0.001). This difference was accounted for by the number of patients with cancer synchronous to the onset of SSc (3/16 in the anti-RNAP III group vs 0/101 in the anti-topo I and 1/243 in the ACA group; p < 0.001), whereas neither the number of malignancies preceding nor those metachronous to the onset of SSc was significantly different between the groups.Conclusion.In a cohort of Italian patients with SSc we observed a significant association between malignancies synchronous to SSc onset and positivity for anti-RNAP III antibodies, similar to that described in American patients with SSc.

scholarly journals Anti-Centromere Antibody Positivity in a Patient with Generalized Morphea

2018 ◽  
Vol 10 (3) ◽  
pp. 226-230
Author(s):  
Fumi Miyagawa ◽  
Anna Nakajima ◽  
Yasuhiro Akai ◽  
Hideo Asada
Keyword(s):  
Systemic Sclerosis ◽  
Rna Polymerase ◽  
Rna Polymerase Iii ◽  
Skin Lesions ◽  
Histological Findings ◽  
Skin Manifestations ◽  
Polymerase Iii ◽  
Oral Steroids ◽  
Antibody Positivity

We report the case of a 45-year-old female with generalized morphea (GM), who exhibited positivity for the anti-centromere antibody (Ab). She frequently developed multiple sclerotic skin lesions, whose histological findings were compatible with morphea. She demonstrated favorable responses to topical and oral steroids. Cases of GM associated with systemic sclerosis (SSc)-specific Abs (anti-Scl-70 Ab, anti-centromere Ab, and anti-RNA polymerase III Ab) have rarely been reported. The previously reported GM cases involving anti-SSc-specific Abs exhibited some skin manifestations of SSc, such as nailfold capillary changes. However, our case did not show any signs of SSc or limited cutaneous SSc. More cases are needed to clarify whether GM with SSc-specific Abs leads to SSc.

Sign in / Sign up

Export Citation Format

Share Document