scholarly journals Upfront Combination Therapy With Rituximab and Mycophenolate Mofetil for Progressive Systemic Sclerosis

2020 ◽  
pp. jrheum.200484
Author(s):  
Doron Rimar ◽  
Itzhak Rosner ◽  
Gleb Slobodin

Systemic sclerosis (SSc) is a complex disease involving multiple pathophysiological pathways: autoimmunity, vasculopathy, and fibrosis, all of which are interrelated. Most of the damage consists of skin and lung fibrosis, and is accumulated within the first 2 years of disease in rapidly progressive patients with a serology of anti-SCL-70 or anti–RNA polymerase III (RNAP3)1.

2016 ◽  
Vol 43 (7) ◽  
pp. 808-810 ◽  
Author(s):  
Ryosuke Saigusa ◽  
Yoshihide Asano ◽  
Kouki Nakamura ◽  
Takashi Yamashita ◽  
Yohei Ichimura ◽  
...  

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

Author(s):  
Maria Grazia Lazzaroni ◽  
Cristian Caimmi ◽  
Eugenia Bertoldo ◽  
Franco Franceschini ◽  
Angela Tincani ◽  
...  

2020 ◽  
Vol 47 (11) ◽  
pp. 1668-1677
Author(s):  
Edward P. Stern ◽  
Sandra G. Guerra ◽  
Harry Chinque ◽  
Vanessa Acquaah ◽  
David González-Serna ◽  
...  

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.


2011 ◽  
Vol 38 (7) ◽  
pp. 1329-1334 ◽  
Author(s):  
PAOLO AIRO’ ◽  
ANGELA CERIBELLI ◽  
ILARIA CAVAZZANA ◽  
MARA TARABORELLI ◽  
STEFANIA ZINGARELLI ◽  
...  

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.


2018 ◽  
Vol 10 (3) ◽  
pp. 226-230
Author(s):  
Fumi Miyagawa ◽  
Anna Nakajima ◽  
Yasuhiro Akai ◽  
Hideo Asada

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.


2015 ◽  
Vol 74 (Suppl 2) ◽  
pp. 88.1-88
Author(s):  
M.G. Lazzaroni ◽  
E. Colombo ◽  
I. Cavazzana ◽  
O. Distler ◽  
R. Hesselstrand ◽  
...  

Sign in / Sign up

Export Citation Format

Share Document