The Experts below are selected from a list of 5271 Experts worldwide ranked by ideXlab platform
Brad A Pauley - One of the best experts on this subject based on the ideXlab platform.
-
anti mj nxp 2 autoantibody specificity in a cohort of adult italian patients with polymyositis dermatomyositis
Arthritis Research & Therapy, 2012Co-Authors: Angela Ceribelli, Jason Y F Chan, Steven J Ross, Ilaria Cavazzana, Micaela Fredi, M Taraborelli, F Franceschini, M Quinzanini, A Tincani, Brad A PauleyAbstract:Autoantibodies in patients with polymyositis/dermatomyositis (PM/DM) are associated with unique subsets, clinical course and outcome. Anti-MJ antibodies, which recognize the Nuclear protein NXP-2/MORC3, are reported in ~25% of juvenile DM. Prevalence and clinical significance of anti-MJ antibodies in adult Italian PM/DM patients were studied. Sera from 58 consecutive adult Italian PM/DM patients were analyzed by immunoprecipitation of 35S-labeled K562 cells extract, ELISA (anti-MJ, Jo-1), Western blot and indirect immunofluorescence. Clinical associations were analyzed using information from medical charts. Anti-MJ antibodies were the most prevalent specificity (17%) found mainly in DM (30%, 8 cases) vs 8% of PM (2 cases, P = 0.02). Comparing 10 anti-MJ (+) vs 48 anti-MJ (-) cases, DM was more common (P = 0.03), and age at onset was younger in anti-MJ (+) (P = 0.0006). In anti-MJ (+), heliotrope rash (P = 0.01) and calcinosis (P = 0.09) were more frequent. None of them had heart or lung involvement, or malignancy. Myopathy in anti-MJ (+) patients responded well to therapy and none of them had elevated CPK at last visit (0% vs 25% in anti-MJ (-)). Only 60% of anti-MJ (+) showed immunofluorescent Nuclear Dots staining, despite PML localization of NXP-2/MORC3. Anti-MJ antibodies are the most frequent specificity in our cohort of adult Italian PM/DM. Anti-MJ (+) were associated with young onset DM, calcinosis, no internal organ involvement and good response of myopathy to therapy. Anti-MJ reported in juvenile DM is also found in adult PM/DM, and could be a new useful biomarker.
-
anti mj nxp 2 autoantibody specificity in a cohort of adult italian patients with polymyositis dermatomyositis
Arthritis Research & Therapy, 2012Co-Authors: Angela Ceribelli, Jason Y F Chan, Steven J Ross, Ilaria Cavazzana, Franco Franceschini, Micaela Fredi, M Taraborelli, M Quinzanini, A Tincani, Brad A PauleyAbstract:Introduction: Autoantibodies in patients with polymyositis/dermatomyositis (PM/DM) are associated with unique subsets, clinical course and outcome. Anti-MJ antibodies, which recognize the Nuclear protein NXP-2/MORC3, are reported in ~25% of juvenile DM. Prevalence and clinical significance of anti-MJ antibodies in adult Italian PM/DM patients were studied. Methods: Sera from 58 consecutive adult Italian PM/DM patients were analyzed by immunoprecipitation of 35 Slabeled K562 cells extract, ELISA (anti-MJ, Jo-1), Western blot and indirect immunofluorescence. Clinical associations were analyzed using information from medical charts. Results: Anti-MJ antibodies were the most prevalent specificity (17%) found mainly in DM (30%, 8 cases) vs 8% of PM (2 cases, P = 0.02). Comparing 10 anti-MJ (+) vs 48 anti-MJ (-) cases, DM was more common (P = 0.03), and age at onset was younger in anti-MJ (+) (P = 0.0006). In anti-MJ (+), heliotrope rash (P = 0.01) and calcinosis (P = 0.09) were more frequent. None of them had heart or lung involvement, or malignancy. Myopathy in anti-MJ (+) patients responded well to therapy and none of them had elevated CPK at last visit (0% vs 25% in anti-MJ (-)). Only 60% of anti-MJ (+) showed immunofluorescent Nuclear Dots staining, despite PML localization of NXP-2/ MORC3. Conclusions: Anti-MJ antibodies are the most frequent specificity in our cohort of adult Italian PM/DM. Anti-MJ (+) were associated with young onset DM, calcinosis, no internal organ involvement and good response of myopathy to therapy. Anti-MJ reported in juvenile DM is also found in adult PM/DM, and could be a new useful biomarker.
Angela Ceribelli - One of the best experts on this subject based on the ideXlab platform.
-
anti mj nxp 2 autoantibody specificity in a cohort of adult italian patients with polymyositis dermatomyositis
Arthritis Research & Therapy, 2012Co-Authors: Angela Ceribelli, Jason Y F Chan, Steven J Ross, Ilaria Cavazzana, Franco Franceschini, Micaela Fredi, M Taraborelli, M Quinzanini, A Tincani, Brad A PauleyAbstract:Introduction: Autoantibodies in patients with polymyositis/dermatomyositis (PM/DM) are associated with unique subsets, clinical course and outcome. Anti-MJ antibodies, which recognize the Nuclear protein NXP-2/MORC3, are reported in ~25% of juvenile DM. Prevalence and clinical significance of anti-MJ antibodies in adult Italian PM/DM patients were studied. Methods: Sera from 58 consecutive adult Italian PM/DM patients were analyzed by immunoprecipitation of 35 Slabeled K562 cells extract, ELISA (anti-MJ, Jo-1), Western blot and indirect immunofluorescence. Clinical associations were analyzed using information from medical charts. Results: Anti-MJ antibodies were the most prevalent specificity (17%) found mainly in DM (30%, 8 cases) vs 8% of PM (2 cases, P = 0.02). Comparing 10 anti-MJ (+) vs 48 anti-MJ (-) cases, DM was more common (P = 0.03), and age at onset was younger in anti-MJ (+) (P = 0.0006). In anti-MJ (+), heliotrope rash (P = 0.01) and calcinosis (P = 0.09) were more frequent. None of them had heart or lung involvement, or malignancy. Myopathy in anti-MJ (+) patients responded well to therapy and none of them had elevated CPK at last visit (0% vs 25% in anti-MJ (-)). Only 60% of anti-MJ (+) showed immunofluorescent Nuclear Dots staining, despite PML localization of NXP-2/ MORC3. Conclusions: Anti-MJ antibodies are the most frequent specificity in our cohort of adult Italian PM/DM. Anti-MJ (+) were associated with young onset DM, calcinosis, no internal organ involvement and good response of myopathy to therapy. Anti-MJ reported in juvenile DM is also found in adult PM/DM, and could be a new useful biomarker.
-
anti mj nxp 2 autoantibody specificity in a cohort of adult italian patients with polymyositis dermatomyositis
Arthritis Research & Therapy, 2012Co-Authors: Angela Ceribelli, Jason Y F Chan, Steven J Ross, Ilaria Cavazzana, Micaela Fredi, M Taraborelli, F Franceschini, M Quinzanini, A Tincani, Brad A PauleyAbstract:Autoantibodies in patients with polymyositis/dermatomyositis (PM/DM) are associated with unique subsets, clinical course and outcome. Anti-MJ antibodies, which recognize the Nuclear protein NXP-2/MORC3, are reported in ~25% of juvenile DM. Prevalence and clinical significance of anti-MJ antibodies in adult Italian PM/DM patients were studied. Sera from 58 consecutive adult Italian PM/DM patients were analyzed by immunoprecipitation of 35S-labeled K562 cells extract, ELISA (anti-MJ, Jo-1), Western blot and indirect immunofluorescence. Clinical associations were analyzed using information from medical charts. Anti-MJ antibodies were the most prevalent specificity (17%) found mainly in DM (30%, 8 cases) vs 8% of PM (2 cases, P = 0.02). Comparing 10 anti-MJ (+) vs 48 anti-MJ (-) cases, DM was more common (P = 0.03), and age at onset was younger in anti-MJ (+) (P = 0.0006). In anti-MJ (+), heliotrope rash (P = 0.01) and calcinosis (P = 0.09) were more frequent. None of them had heart or lung involvement, or malignancy. Myopathy in anti-MJ (+) patients responded well to therapy and none of them had elevated CPK at last visit (0% vs 25% in anti-MJ (-)). Only 60% of anti-MJ (+) showed immunofluorescent Nuclear Dots staining, despite PML localization of NXP-2/MORC3. Anti-MJ antibodies are the most frequent specificity in our cohort of adult Italian PM/DM. Anti-MJ (+) were associated with young onset DM, calcinosis, no internal organ involvement and good response of myopathy to therapy. Anti-MJ reported in juvenile DM is also found in adult PM/DM, and could be a new useful biomarker.
-
autoantibodies to survival of motor neuron complex in patients with polymyositis immunoprecipitation of d e f and g proteins without other components of small Nuclear ribonucleoproteins
Arthritis & Rheumatism, 2011Co-Authors: Minoru Satoh, Jason Y F Chan, Steven J Ross, Angela Ceribelli, Ilaria Cavazzana, Franco Franceschini, Westley H Reeves, Eric S Sobel, Edward K L ChanAbstract:Specific autoantibodies in systemic rheumatic diseases are useful biomarkers associated with diagnosis and also often with unique clinical manifestations (1). Small Nuclear ribonucleoproteins (snRNPs) are one of the most common targets of autoantibodies found in SLE and other rheumatic diseases. Antibodies to U1snRNPs (U1RNP) is the most common anti-snRNPs specificity, seen in 30–40% of SLE and less frequently in other systemic rheumatic diseases. In contrast, anti-Sm antibodies that recognize U1, U2, U4-6, and U5snRNPs are specific for SLE found in ~10–15% of patients. Antibodies to U1RNP or Sm immunoprecipitate characteristic set of proteins U1-70k, A, B’/B, C, D1/D2/D3, E, F, and G, which can be easily identified by protein immunoprecipitation (2). While screening autoantibodies in human sera, unusual sera that appeared to immunoprecipitate D, E, F, and G but not other common component of the snRNPs, were noticed. Although there are reports on less common anti-snRNPs autoantibodies including anti-U2RNP, U4-6RNP, and U5RNP (2), the pattern does not match with any known UsnRNPs. Thus, it was considered that these sera had antibodies that bind pre-assembly complex prior to form the Sm core particle or snRNPs. Target antigen of this previously unreported autoantibodies has been identified as survival of motor neuron (SMN) complex that is present in Nuclear Dots structure Cajal body and play a critical role in assembly of the snRNPs (3). Deletion or mutation of SMN is known to cause spinal muscular atrophy (3). Interestingly, all 3 patients with anti-SMN had polymyositis, another disease that involves muscle pathology.
Steven J Ross - One of the best experts on this subject based on the ideXlab platform.
-
anti mj nxp 2 autoantibody specificity in a cohort of adult italian patients with polymyositis dermatomyositis
Arthritis Research & Therapy, 2012Co-Authors: Angela Ceribelli, Jason Y F Chan, Steven J Ross, Ilaria Cavazzana, Franco Franceschini, Micaela Fredi, M Taraborelli, M Quinzanini, A Tincani, Brad A PauleyAbstract:Introduction: Autoantibodies in patients with polymyositis/dermatomyositis (PM/DM) are associated with unique subsets, clinical course and outcome. Anti-MJ antibodies, which recognize the Nuclear protein NXP-2/MORC3, are reported in ~25% of juvenile DM. Prevalence and clinical significance of anti-MJ antibodies in adult Italian PM/DM patients were studied. Methods: Sera from 58 consecutive adult Italian PM/DM patients were analyzed by immunoprecipitation of 35 Slabeled K562 cells extract, ELISA (anti-MJ, Jo-1), Western blot and indirect immunofluorescence. Clinical associations were analyzed using information from medical charts. Results: Anti-MJ antibodies were the most prevalent specificity (17%) found mainly in DM (30%, 8 cases) vs 8% of PM (2 cases, P = 0.02). Comparing 10 anti-MJ (+) vs 48 anti-MJ (-) cases, DM was more common (P = 0.03), and age at onset was younger in anti-MJ (+) (P = 0.0006). In anti-MJ (+), heliotrope rash (P = 0.01) and calcinosis (P = 0.09) were more frequent. None of them had heart or lung involvement, or malignancy. Myopathy in anti-MJ (+) patients responded well to therapy and none of them had elevated CPK at last visit (0% vs 25% in anti-MJ (-)). Only 60% of anti-MJ (+) showed immunofluorescent Nuclear Dots staining, despite PML localization of NXP-2/ MORC3. Conclusions: Anti-MJ antibodies are the most frequent specificity in our cohort of adult Italian PM/DM. Anti-MJ (+) were associated with young onset DM, calcinosis, no internal organ involvement and good response of myopathy to therapy. Anti-MJ reported in juvenile DM is also found in adult PM/DM, and could be a new useful biomarker.
-
anti mj nxp 2 autoantibody specificity in a cohort of adult italian patients with polymyositis dermatomyositis
Arthritis Research & Therapy, 2012Co-Authors: Angela Ceribelli, Jason Y F Chan, Steven J Ross, Ilaria Cavazzana, Micaela Fredi, M Taraborelli, F Franceschini, M Quinzanini, A Tincani, Brad A PauleyAbstract:Autoantibodies in patients with polymyositis/dermatomyositis (PM/DM) are associated with unique subsets, clinical course and outcome. Anti-MJ antibodies, which recognize the Nuclear protein NXP-2/MORC3, are reported in ~25% of juvenile DM. Prevalence and clinical significance of anti-MJ antibodies in adult Italian PM/DM patients were studied. Sera from 58 consecutive adult Italian PM/DM patients were analyzed by immunoprecipitation of 35S-labeled K562 cells extract, ELISA (anti-MJ, Jo-1), Western blot and indirect immunofluorescence. Clinical associations were analyzed using information from medical charts. Anti-MJ antibodies were the most prevalent specificity (17%) found mainly in DM (30%, 8 cases) vs 8% of PM (2 cases, P = 0.02). Comparing 10 anti-MJ (+) vs 48 anti-MJ (-) cases, DM was more common (P = 0.03), and age at onset was younger in anti-MJ (+) (P = 0.0006). In anti-MJ (+), heliotrope rash (P = 0.01) and calcinosis (P = 0.09) were more frequent. None of them had heart or lung involvement, or malignancy. Myopathy in anti-MJ (+) patients responded well to therapy and none of them had elevated CPK at last visit (0% vs 25% in anti-MJ (-)). Only 60% of anti-MJ (+) showed immunofluorescent Nuclear Dots staining, despite PML localization of NXP-2/MORC3. Anti-MJ antibodies are the most frequent specificity in our cohort of adult Italian PM/DM. Anti-MJ (+) were associated with young onset DM, calcinosis, no internal organ involvement and good response of myopathy to therapy. Anti-MJ reported in juvenile DM is also found in adult PM/DM, and could be a new useful biomarker.
-
autoantibodies to survival of motor neuron complex in patients with polymyositis immunoprecipitation of d e f and g proteins without other components of small Nuclear ribonucleoproteins
Arthritis & Rheumatism, 2011Co-Authors: Minoru Satoh, Jason Y F Chan, Steven J Ross, Angela Ceribelli, Ilaria Cavazzana, Franco Franceschini, Westley H Reeves, Eric S Sobel, Edward K L ChanAbstract:Specific autoantibodies in systemic rheumatic diseases are useful biomarkers associated with diagnosis and also often with unique clinical manifestations (1). Small Nuclear ribonucleoproteins (snRNPs) are one of the most common targets of autoantibodies found in SLE and other rheumatic diseases. Antibodies to U1snRNPs (U1RNP) is the most common anti-snRNPs specificity, seen in 30–40% of SLE and less frequently in other systemic rheumatic diseases. In contrast, anti-Sm antibodies that recognize U1, U2, U4-6, and U5snRNPs are specific for SLE found in ~10–15% of patients. Antibodies to U1RNP or Sm immunoprecipitate characteristic set of proteins U1-70k, A, B’/B, C, D1/D2/D3, E, F, and G, which can be easily identified by protein immunoprecipitation (2). While screening autoantibodies in human sera, unusual sera that appeared to immunoprecipitate D, E, F, and G but not other common component of the snRNPs, were noticed. Although there are reports on less common anti-snRNPs autoantibodies including anti-U2RNP, U4-6RNP, and U5RNP (2), the pattern does not match with any known UsnRNPs. Thus, it was considered that these sera had antibodies that bind pre-assembly complex prior to form the Sm core particle or snRNPs. Target antigen of this previously unreported autoantibodies has been identified as survival of motor neuron (SMN) complex that is present in Nuclear Dots structure Cajal body and play a critical role in assembly of the snRNPs (3). Deletion or mutation of SMN is known to cause spinal muscular atrophy (3). Interestingly, all 3 patients with anti-SMN had polymyositis, another disease that involves muscle pathology.
Jason Y F Chan - One of the best experts on this subject based on the ideXlab platform.
-
anti mj nxp 2 autoantibody specificity in a cohort of adult italian patients with polymyositis dermatomyositis
Arthritis Research & Therapy, 2012Co-Authors: Angela Ceribelli, Jason Y F Chan, Steven J Ross, Ilaria Cavazzana, Franco Franceschini, Micaela Fredi, M Taraborelli, M Quinzanini, A Tincani, Brad A PauleyAbstract:Introduction: Autoantibodies in patients with polymyositis/dermatomyositis (PM/DM) are associated with unique subsets, clinical course and outcome. Anti-MJ antibodies, which recognize the Nuclear protein NXP-2/MORC3, are reported in ~25% of juvenile DM. Prevalence and clinical significance of anti-MJ antibodies in adult Italian PM/DM patients were studied. Methods: Sera from 58 consecutive adult Italian PM/DM patients were analyzed by immunoprecipitation of 35 Slabeled K562 cells extract, ELISA (anti-MJ, Jo-1), Western blot and indirect immunofluorescence. Clinical associations were analyzed using information from medical charts. Results: Anti-MJ antibodies were the most prevalent specificity (17%) found mainly in DM (30%, 8 cases) vs 8% of PM (2 cases, P = 0.02). Comparing 10 anti-MJ (+) vs 48 anti-MJ (-) cases, DM was more common (P = 0.03), and age at onset was younger in anti-MJ (+) (P = 0.0006). In anti-MJ (+), heliotrope rash (P = 0.01) and calcinosis (P = 0.09) were more frequent. None of them had heart or lung involvement, or malignancy. Myopathy in anti-MJ (+) patients responded well to therapy and none of them had elevated CPK at last visit (0% vs 25% in anti-MJ (-)). Only 60% of anti-MJ (+) showed immunofluorescent Nuclear Dots staining, despite PML localization of NXP-2/ MORC3. Conclusions: Anti-MJ antibodies are the most frequent specificity in our cohort of adult Italian PM/DM. Anti-MJ (+) were associated with young onset DM, calcinosis, no internal organ involvement and good response of myopathy to therapy. Anti-MJ reported in juvenile DM is also found in adult PM/DM, and could be a new useful biomarker.
-
anti mj nxp 2 autoantibody specificity in a cohort of adult italian patients with polymyositis dermatomyositis
Arthritis Research & Therapy, 2012Co-Authors: Angela Ceribelli, Jason Y F Chan, Steven J Ross, Ilaria Cavazzana, Micaela Fredi, M Taraborelli, F Franceschini, M Quinzanini, A Tincani, Brad A PauleyAbstract:Autoantibodies in patients with polymyositis/dermatomyositis (PM/DM) are associated with unique subsets, clinical course and outcome. Anti-MJ antibodies, which recognize the Nuclear protein NXP-2/MORC3, are reported in ~25% of juvenile DM. Prevalence and clinical significance of anti-MJ antibodies in adult Italian PM/DM patients were studied. Sera from 58 consecutive adult Italian PM/DM patients were analyzed by immunoprecipitation of 35S-labeled K562 cells extract, ELISA (anti-MJ, Jo-1), Western blot and indirect immunofluorescence. Clinical associations were analyzed using information from medical charts. Anti-MJ antibodies were the most prevalent specificity (17%) found mainly in DM (30%, 8 cases) vs 8% of PM (2 cases, P = 0.02). Comparing 10 anti-MJ (+) vs 48 anti-MJ (-) cases, DM was more common (P = 0.03), and age at onset was younger in anti-MJ (+) (P = 0.0006). In anti-MJ (+), heliotrope rash (P = 0.01) and calcinosis (P = 0.09) were more frequent. None of them had heart or lung involvement, or malignancy. Myopathy in anti-MJ (+) patients responded well to therapy and none of them had elevated CPK at last visit (0% vs 25% in anti-MJ (-)). Only 60% of anti-MJ (+) showed immunofluorescent Nuclear Dots staining, despite PML localization of NXP-2/MORC3. Anti-MJ antibodies are the most frequent specificity in our cohort of adult Italian PM/DM. Anti-MJ (+) were associated with young onset DM, calcinosis, no internal organ involvement and good response of myopathy to therapy. Anti-MJ reported in juvenile DM is also found in adult PM/DM, and could be a new useful biomarker.
-
autoantibodies to survival of motor neuron complex in patients with polymyositis immunoprecipitation of d e f and g proteins without other components of small Nuclear ribonucleoproteins
Arthritis & Rheumatism, 2011Co-Authors: Minoru Satoh, Jason Y F Chan, Steven J Ross, Angela Ceribelli, Ilaria Cavazzana, Franco Franceschini, Westley H Reeves, Eric S Sobel, Edward K L ChanAbstract:Specific autoantibodies in systemic rheumatic diseases are useful biomarkers associated with diagnosis and also often with unique clinical manifestations (1). Small Nuclear ribonucleoproteins (snRNPs) are one of the most common targets of autoantibodies found in SLE and other rheumatic diseases. Antibodies to U1snRNPs (U1RNP) is the most common anti-snRNPs specificity, seen in 30–40% of SLE and less frequently in other systemic rheumatic diseases. In contrast, anti-Sm antibodies that recognize U1, U2, U4-6, and U5snRNPs are specific for SLE found in ~10–15% of patients. Antibodies to U1RNP or Sm immunoprecipitate characteristic set of proteins U1-70k, A, B’/B, C, D1/D2/D3, E, F, and G, which can be easily identified by protein immunoprecipitation (2). While screening autoantibodies in human sera, unusual sera that appeared to immunoprecipitate D, E, F, and G but not other common component of the snRNPs, were noticed. Although there are reports on less common anti-snRNPs autoantibodies including anti-U2RNP, U4-6RNP, and U5RNP (2), the pattern does not match with any known UsnRNPs. Thus, it was considered that these sera had antibodies that bind pre-assembly complex prior to form the Sm core particle or snRNPs. Target antigen of this previously unreported autoantibodies has been identified as survival of motor neuron (SMN) complex that is present in Nuclear Dots structure Cajal body and play a critical role in assembly of the snRNPs (3). Deletion or mutation of SMN is known to cause spinal muscular atrophy (3). Interestingly, all 3 patients with anti-SMN had polymyositis, another disease that involves muscle pathology.
Ilaria Cavazzana - One of the best experts on this subject based on the ideXlab platform.
-
anti mj nxp 2 autoantibody specificity in a cohort of adult italian patients with polymyositis dermatomyositis
Arthritis Research & Therapy, 2012Co-Authors: Angela Ceribelli, Jason Y F Chan, Steven J Ross, Ilaria Cavazzana, Franco Franceschini, Micaela Fredi, M Taraborelli, M Quinzanini, A Tincani, Brad A PauleyAbstract:Introduction: Autoantibodies in patients with polymyositis/dermatomyositis (PM/DM) are associated with unique subsets, clinical course and outcome. Anti-MJ antibodies, which recognize the Nuclear protein NXP-2/MORC3, are reported in ~25% of juvenile DM. Prevalence and clinical significance of anti-MJ antibodies in adult Italian PM/DM patients were studied. Methods: Sera from 58 consecutive adult Italian PM/DM patients were analyzed by immunoprecipitation of 35 Slabeled K562 cells extract, ELISA (anti-MJ, Jo-1), Western blot and indirect immunofluorescence. Clinical associations were analyzed using information from medical charts. Results: Anti-MJ antibodies were the most prevalent specificity (17%) found mainly in DM (30%, 8 cases) vs 8% of PM (2 cases, P = 0.02). Comparing 10 anti-MJ (+) vs 48 anti-MJ (-) cases, DM was more common (P = 0.03), and age at onset was younger in anti-MJ (+) (P = 0.0006). In anti-MJ (+), heliotrope rash (P = 0.01) and calcinosis (P = 0.09) were more frequent. None of them had heart or lung involvement, or malignancy. Myopathy in anti-MJ (+) patients responded well to therapy and none of them had elevated CPK at last visit (0% vs 25% in anti-MJ (-)). Only 60% of anti-MJ (+) showed immunofluorescent Nuclear Dots staining, despite PML localization of NXP-2/ MORC3. Conclusions: Anti-MJ antibodies are the most frequent specificity in our cohort of adult Italian PM/DM. Anti-MJ (+) were associated with young onset DM, calcinosis, no internal organ involvement and good response of myopathy to therapy. Anti-MJ reported in juvenile DM is also found in adult PM/DM, and could be a new useful biomarker.
-
anti mj nxp 2 autoantibody specificity in a cohort of adult italian patients with polymyositis dermatomyositis
Arthritis Research & Therapy, 2012Co-Authors: Angela Ceribelli, Jason Y F Chan, Steven J Ross, Ilaria Cavazzana, Micaela Fredi, M Taraborelli, F Franceschini, M Quinzanini, A Tincani, Brad A PauleyAbstract:Autoantibodies in patients with polymyositis/dermatomyositis (PM/DM) are associated with unique subsets, clinical course and outcome. Anti-MJ antibodies, which recognize the Nuclear protein NXP-2/MORC3, are reported in ~25% of juvenile DM. Prevalence and clinical significance of anti-MJ antibodies in adult Italian PM/DM patients were studied. Sera from 58 consecutive adult Italian PM/DM patients were analyzed by immunoprecipitation of 35S-labeled K562 cells extract, ELISA (anti-MJ, Jo-1), Western blot and indirect immunofluorescence. Clinical associations were analyzed using information from medical charts. Anti-MJ antibodies were the most prevalent specificity (17%) found mainly in DM (30%, 8 cases) vs 8% of PM (2 cases, P = 0.02). Comparing 10 anti-MJ (+) vs 48 anti-MJ (-) cases, DM was more common (P = 0.03), and age at onset was younger in anti-MJ (+) (P = 0.0006). In anti-MJ (+), heliotrope rash (P = 0.01) and calcinosis (P = 0.09) were more frequent. None of them had heart or lung involvement, or malignancy. Myopathy in anti-MJ (+) patients responded well to therapy and none of them had elevated CPK at last visit (0% vs 25% in anti-MJ (-)). Only 60% of anti-MJ (+) showed immunofluorescent Nuclear Dots staining, despite PML localization of NXP-2/MORC3. Anti-MJ antibodies are the most frequent specificity in our cohort of adult Italian PM/DM. Anti-MJ (+) were associated with young onset DM, calcinosis, no internal organ involvement and good response of myopathy to therapy. Anti-MJ reported in juvenile DM is also found in adult PM/DM, and could be a new useful biomarker.
-
autoantibodies to survival of motor neuron complex in patients with polymyositis immunoprecipitation of d e f and g proteins without other components of small Nuclear ribonucleoproteins
Arthritis & Rheumatism, 2011Co-Authors: Minoru Satoh, Jason Y F Chan, Steven J Ross, Angela Ceribelli, Ilaria Cavazzana, Franco Franceschini, Westley H Reeves, Eric S Sobel, Edward K L ChanAbstract:Specific autoantibodies in systemic rheumatic diseases are useful biomarkers associated with diagnosis and also often with unique clinical manifestations (1). Small Nuclear ribonucleoproteins (snRNPs) are one of the most common targets of autoantibodies found in SLE and other rheumatic diseases. Antibodies to U1snRNPs (U1RNP) is the most common anti-snRNPs specificity, seen in 30–40% of SLE and less frequently in other systemic rheumatic diseases. In contrast, anti-Sm antibodies that recognize U1, U2, U4-6, and U5snRNPs are specific for SLE found in ~10–15% of patients. Antibodies to U1RNP or Sm immunoprecipitate characteristic set of proteins U1-70k, A, B’/B, C, D1/D2/D3, E, F, and G, which can be easily identified by protein immunoprecipitation (2). While screening autoantibodies in human sera, unusual sera that appeared to immunoprecipitate D, E, F, and G but not other common component of the snRNPs, were noticed. Although there are reports on less common anti-snRNPs autoantibodies including anti-U2RNP, U4-6RNP, and U5RNP (2), the pattern does not match with any known UsnRNPs. Thus, it was considered that these sera had antibodies that bind pre-assembly complex prior to form the Sm core particle or snRNPs. Target antigen of this previously unreported autoantibodies has been identified as survival of motor neuron (SMN) complex that is present in Nuclear Dots structure Cajal body and play a critical role in assembly of the snRNPs (3). Deletion or mutation of SMN is known to cause spinal muscular atrophy (3). Interestingly, all 3 patients with anti-SMN had polymyositis, another disease that involves muscle pathology.
