Patients with anti-PM/Scl-positive and idiopathic inflammatory myopathy resemble antisynthetase syndrome

doi:10.21203/rs.3.rs-5388447/v1

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Patients with anti-PM/Scl-positive and idiopathic inflammatory myopathy resemble antisynthetase syndrome

https://doi.org/10.21203/rs.3.rs-5388447/v1

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Background. Anti-PM/Scl autoantibody has been associated with an overlap between polymyositis (PM) and systemic sclerosis (SSc). However, owing to few studies, we analyzed the relevance of this autoantibody in patients with idiopathic inflammatory myopathies (IIMs) without SSc.

Methods. This single-center retrospective cohort study was conducted between 2004 and 2024. Of these, 93 adult patients with IIMs (66 with dermatomyositis and 27 with PM - EULAR/ACR 2017) without SSc were included: 16 anti-PM/Scl(+) and 77 anti-PM/Scl(-). We excluded patients with other types of IIMs, cancer-associated myositis, or overlap myositis, including SSc, as well as those with other myositis-specific and/or myositis-associated autoantibodies.

Results. The median age, sex distribution, and white ethnicity, and median follow-up duration were comparable between the anti-PM/Scl(+) and anti-PM/Scl(-) groups. There were no differences in clinical and laboratory characteristics, except for a higher frequency of lung involvement, joint involvement, “mechanics’ hand,” “hiker’s feet,” and Raynaud’s phenomenon, in contrast to a lower frequency of facial rash and “V”-neck sign in patients with anti-PM/Scl(+) than in those with anti-PM/Scl(-) (all p<0.05). Furthermore, patients with anti-PM/Scl(+) presented a higher frequency of disease relapse (68.8% vs. 33.8%), disease activity (50.0% vs. 24.7%), and immunosuppressant use at the last medical evaluation (all p<0.05). Severe infection and death rates were comparable between the groups.

Conclusions. Anti-PM/Scl positivity was observed in 17.2% of IIMs’ patients without SSc. Patients with this autoantibody present clinical manifestations resembling antisynthetase syndrome with increased disease relapse and disease activity.

Anti-PM/Scl

Autoantibodies

Phenotypic

Systemic autoimmune myopathies

Inflammatory myopathies

Myositis

Idiopathic inflammatory myopathies (IIMs) or systemic autoimmune myopathies are a group of rare rheumatic diseases with inflammatory characteristics that mainly affect skeletal musculature [1,2]. Based on demographics, evolutionary and histological characteristics, such as systemic manifestations, IIMs can be classified as dermatomyositis (DM), polymyositis (PM), clinically amyopathic dermatomyositis (CADM), immune-mediated necrotizing myopathy (IMNM), anti-synthetase syndrome (ASyS), inclusion body myositis (IBM), among others [1–3].

Various autoantibodies are associated with IIMs. These, in turn, can be classified as myositis-specific or myositis-associated autoantibodies [1]. Among the myositis-associated autoantibodies, anti-PM/Scl has been described in patients with an overlap between autoimmune myopathy (for example, PM) and systemic sclerosis [4–11].

However, few studies are available in the literature, mainly case reports and series including patients affected by IIMs with anti-PM/Scl autoantibodies without systemic sclerosis. These patients apparently exhibit joint and/or pulmonary involvement [4,12–24], “hiker’s feet” [20,24–26], esophageal involvement [15,18,23,27] and/or Raynaud’s phenomenon [15,18,24]. Furthermore, as limitations, these studies defined patients with IIMs according to the Bohan and Peter criteria [13,15,17,21,23,28,29], did not simultaneously analyze the presence of others myositis-specific or myositis-associated autoantibodies [13,15–18,21] and/or did not exclude patients with others systemic autoimmune diseases, such as Sjögren’s syndrome [20,23,28] and systemic sclerosis [14,16,20,23,28,29].

Therefore, the present study aimed to evaluate the isolated importance of anti-PM/Scl autoantibodies in a representative sample of patients with IIMs (DM or PM) without systemic sclerosis or other systemic autoimmune diseases as well as other myositis-specific and myositis-associated autoantibodies. Second, patients were assessed for demographic, clinical, laboratory, therapeutic, evolutionary, and prognostic parameters.

This was a retrospective cohort single-center study conducted from 2004 to 2024 that included adult patients with IIMs (DM or PM) from the inflammatory myopathy outpatient clinic of a tertiary center.

Inclusion criteria. Adult patients with IIMs according to the European League Against Rheumatism/American College of Rheumatology (EULAR/ACR) 2017 criteria classification [30]; availability of blood samples for myositis-specific and myositis-associated autoantibody tests including anti-PM/Scl.

Exclusion criteria. Patients with IIMs (DM or PM) associated with other systemic autoimmune diseases (rheumatoid arthritis, systemic sclerosis, systemic lupus erythematosus, or Sjögren’s disease); patients with IMNM, CADM, IBM, and myositis associated with neoplasms; except for anti-PM/Scl autoantibodies, which were positive for any other myositis-specific and myositis-associated autoantibodies.

Control group. Adult patients with IIMs according to the European League Against Rheumatism/American College of Rheumatology (EULAR/ACR) 2017 criteria classification [30] and negative for myositis-specific and myositis-associated autoantibodies, including anti-PM/Scl.

The following data from eligible patients (anti-PM/Scl cases and control group) were collected from electronic medical records with pre-standardized and parameterized information:

Demographics: Current age, age at disease diagnosis, sex, and ethnicity;

Clinical manifestations: Symptoms onset, following time, cutaneous involvement (heliotrope, facial rash, Gottron’s sign/papule, “V-neck” sign, “shawl” sign, “mechanic’s hand,” “hiker’s feet,” periungual hyperemia, vasculopathy, ulcers and calcinosis), Raynaud’s phenomenon, systemic manifestations (dysphagia, arthritis, dyspnea, weight loss and fever), muscle weakness of upper and lower limbs graded according the Medical Research Council [31]; medications prescribed at the first and last appointments and their respective doses;

Laboratory: Serum levels of muscle enzymes [creatine phosphokinase (CPK), aspartate aminotransferase (AST), alanine aminotransferase (ALT), and lactic dehydrogenase (LDH)], and antinuclear antibodies (ANA) in blood samples;

Complementary examinations: Changes in high-resolution computer tomography images of the lung, finding “ground glass” (opacity), pulmonary fibrosis, and/or incipient interstitial lung disease (ILD).

An analysis of myositis-specific and myositis-associated autoantibodies was performed in patients’ serum samples stored at − 20ºC. These biological samples were collected at the time of the initial patient follow-up and after obtaining informed and voluntary consent from the patients.

Anti-PM/Scl, -OJ, -EJ, -PL-7, -PL-12, -SRP, -Jo-1, -SAE-1, -NXP-2, -MDA-5, -TIF-1γ, -Mi-2, -Ro-52, and -Ku autoantibodies were performed using a commercial kit (Euroimmun, Lübeck, Germany), according to the manufacturer’s protocol. Evaluation of the results was based on the method established in a previous study [32].

Statistical analysis. The Kolmogorov-Smirnov test was used to evaluate the distribution of continuous variables. Results are presented as median (interquartile 75th -25th ) or frequency (%). Data evaluation of patients with and without anti-PM/Scl was performed using the Student’s t-test or Mann-Whitney U test for continuous variables and the chi-square test or Fisher’s exact test for categorical variables. Statistical significance was set at p < 0.05. All analyses were performed using SPSS Statistics, version 15.0 (Chicago, IL, USA).

Of the 589 adult patients with IIMs (DM or PM), were excluded 257 patients, because they presented with IMNM (n = 68), CADM (n = 61), IBM (n = 32), cancer-associated myositis (n = 60), and/or myositis associated with other types of rheumatic diseases (n = 67). Some patients had overlap of more than one type of rheumatic diseases and/or neoplasm. Furthermore, were excluded 74 cases, because they did not present the availability of blood samples for autoantibodies analysis and 165 cases because they presented myositis-specific (44 with anti-Jo-1, 32 with anti-MDA-5, 18 with anti-Mi-2, 14 with anti-PL-7, 12 with anti-PL-12, 10 with anti-TIF-1γ, seven with anti-NXP-2, seven with anti-SAE, six with anti-EJ, and/or four with anti-SRP) and/or myositis-associated autoantibodies (74 with anti-Ro-52 or 12 with anti-Ku). Some patients tested positive for more than one autoantibody (Fig. 1).

Of the remaining 93 patients, 16 (17.2%) presented with only anti-PM/Scl(+) and 77 (82.8%) with anti-PM/Scl(-) (control group), of which 66 and 27 patients were initially diagnosed with DM and PM, respectively.

Age, sex, and ethnicity distributions were comparable between the anti-PM/Scl(+) and anti-PM/Scl(-) groups (Table 1).

Table 1

General characteristics of adult patients with idiopathic inflammatory myopathies, according to presence or absence of anti-PM/Scl autoantibodies
Characteristics	Anti-PM/Scl(+) (n = 16)	Anti-PM/Scl(-) (n = 77)	p value
Age at disease diagnosis (years)	42 (29–59)	41 (31–53)	0.607
Female sex	13 (81.3)	60 (77.9)	> 0.999
White ethnicity	14 (87.5)	57 (74.0)	0.342
Initial disease diagnosis
Dermatomyositis	11 (68.8)	55 (71.4)	> 0.999
Polymyositis	5 (31.2)	22 (28.6)	> 0.999
Fever	3 (18.8)	17 (22.1)	> 0.999
Weight loss	9 (56.2)	33 (42.9)	0.411
Gottron’s sign / papules	10 (62.4)	48 (62.3)	> 0.999
Heliotrope rash	7 (43.8)	46 (59.7)	0.276
Facial rash	2 (12.5)	33 (42.9)	0.025
“V”-neck sign	1 (6.3)	33 (42.9)	0.005
“Shawl” sign	0	21 (27.3)	-
Periungual hyperemia	8 (50.0)	30 (39.0)	0.420
Raynaud’s phenomenon	11 (68.8)	29 (37.7)	0.028
Vasculopathy	0	14 (18.2)	-
“Mechanics’ hands”	11 (68.8)	17 (22.1)	0.001
“Hiker’s feet”	8 (50.0)	5 (6.5)	< 0.001
Skin ulcers	2 (12.5)	9 (11.7)	> 0.999
Calcinosis	0	4 (5.2)	-
Muscle strength (MRC)
Upper limbs
V degree	3 (18.8)	16 (20.8)	> 0.999
IV degree	10 (62.4)	44 (57.1)
III degree	3 (18.8)	14 (18.2)
II degree	0	3 (3.9)
Lower limbs
V degree	0	16 (20.8)	> 0.999
IV degree	14 (87.5)	42 (54.5)
III degree	2 (12.5)	18 (23.4)
II degree	0	1 (1.3)
Dysphagia	7 (43.8)	30 (39.0)	0.784
Arthritis	10 (62.4)	24 (31.2)	0.024
Lung involvement
Dyspnea	10 (62.4)	27 (35.1)	0.005
Interstitial pneumopathy	14 (87.5)	25 (32.5)	< 0.001
Ground glass opacities	11 (68.8)	21 (27.3)	0.003
Fibrosis	8 (50.0)	11 (14.3)	0.003
Maximum levels of muscle enzymes
Creatine phosphokinase (U/L)	544 (202–3819)	277 (67-1748)	0.228
Aspartate aminotransferase (U/L)	45 (28–155)	47 (27–131)	0.891
Alanine aminotransferase (U/L)	40 (28–121)	40 (24–133)	0.935
Lactic dehydrogenase (U/L)	545 (350–867)	592 (372–833)	0.695
Antinuclear antibodies	16 (100)	55 (71.4)	0.034
Data are expressed as median (IQR 25th − 75th ) or percentage (%).
MRC: Medical Research Council.

Clinical manifestations were also similar between the groups, except by a higher frequency of lung and joint involvements, “mechanics’ hand,” “hiker’s feet,” and Raynaud’s phenomenon; and a lower frequency of facial rash and “V”-neck sign in patients with anti-PM/Scl(+), when they are compared to the anti-PM/Scl(-).

Serum levels of muscle enzymes were comparable between the groups; however, there was a higher frequency of antinuclear antibodies in patients with anti-PM/Scl(+) than in those with anti-PM/Scl(-).

The follow-up durations were similar between the groups (Table 2). During follow-up, there was no difference in the frequency of infections and deaths between the groups, except for disease relapse, number of individuals with active disease, and disease remission (at the last medical evaluation) among the patients with anti-PM/Scl(+).

Table 2

Treatment characteristics and follow-up of 93 patients with idiopathic inflammatory myopathies
Characteristics	Anti-PM/Scl(+) (n = 16)	Anti-PM/Scl(-) (n = 77)	p value
Current follow-up
Duration of follow-up (months)	94 (20–140)	64 (65–93)	0.244
Infections	2 (12.5)	21 (27.3)	0.341
Relapses	11 (68.8)	26 (33.8)	0.023
Death	1 (6.3)	1 (1.3)	0.402
Disease status at the last medical evaluation
Active disease (clinical relapse)	8 (50.0)	19 (24.7)	0.025
Complete clinical response	6 (37.5)	21 (27.3)
Disease remission	2 (12.5)	37(48.0)
Treatment
Previous IVMP pulse therapy	5 (31.2)	12 (15.6)	0.235
Previous IVIg	0	5 (6.5)	-
Previous IVIg + IVMP pulse therapy	3 (18.8)	21 (27.3)	0.478
No IVMP or IVIg	8 (50.0)	39 (50.6)	0.962
Current treatment
Glucocorticoid
Current use	7 (43.8)	21 (27.3)	0.134
Current dose (mg/day)	10 (5–20)	10 (5–20)	0.534
Methotrexate	6 (37.5)	10 (13.0)	0.027
Azathioprine	5 (31.2)	8 (10.4)	0.029
Mycophenolate mofetil	4 (25.0)	13 (16.9)	0.696
Leflunomide	0	4 (5.2)	-
Cyclosporine	5 (31.2)	22 (28.6)	0.823
Cyclophosphamide	3 (18.8)	7 (9.1)	0.256
Rituximab	4 (25.0)	8 (10.4)	0.113
Data are expressed as median (IQR 25th − 75th ) or percentage (%).
IVIg: intravenous immunoglobulin; IVMP: intravenous methylprednisolone.

Previous treatments were comparable between the analyzed groups, anti-PM/Scl(+) and anti-PM/Scl(-), as well as the medical treatment established at the last medical evaluation, except for the higher use of methotrexate and azathioprine in patients with anti-PM/Scl(+).

In the present study, the isolated presence of anti-PM/Scl autoantibodies in patients with IIMs (DM or PM) and without systemic sclerosis was 17.2%. These patients, when they are compared to patients with anti-PM/Scl(-), presented higher frequencies of lung and joint involvements, “mechanics’ hands,” “hiker’s feet” and Raynaud’s phenomenon, and a high relapse rates and a low rate of disease remission. Furthermore, the patients presented with a higher frequency of disease activity and immunosuppressant use during the last medical evaluation.

Unlike the studies available in the literature [13–18,20–21,23,28,29], the present study evaluated the presence of anti-PM/Scl in a homogeneous and representative sample of patients, without associated systemic autoimmune diseases or the presence of any other myositis-specific and myositis-associated autoantibodies. In contrast, the studies available in the literature conduct the anti-PM/Scl analysis based in cases reports or series [13,17,19–20,23–27], in addition to not having excluded the possible presence of others myositis-specific and myositis-associated autoantibodies [13,15–18,21,29] or others systemic autoimmune diseases [14,16,23,28,29]. These parameters can be confounders factors to a probable joint or lung manifestations, besides the presence of Raynaud’s phenomenon in these studies [13–14,16–18,21,23,28–29].

Furthermore, in the present study, we used the European League Against Rheumatism/American College of Rheumatology (EULAR/ACR) 2017 criteria classification for the definition of IIMs, whereas the studies available in the literature [13,15,17,21,23,28–29] used the old Bohan and Peter criteria [33] that present a low specificity, especially for the PM definition. Moreover, we excluded cases of CADM, cancer-associated myositis, and IMNM, which resulted in only DM or PM.

When compared with our patients with anti-PM/Scl(-), those with anti-PM/Scl(+) presented clinical manifestations similar to ASyS, with Raynaud’s phenomenon, “mechanics’ hands,” “hiker’s feet,” joints, and lung involvement. Similarly to the literature, more than 60% of the patients with anti-PM/Scl(+) presented Raynaud phenomenon [4,12–13,18,21,24], and more than 75% of patients manifested interstitial lung disease [4,12,14,16–17,20,24]. Other few studies [3,20,22] reported the presence of “Hiker’s feet” in more than 80% of the analyzed cases.

Li et al. [34] evaluated 97 patients with DM and without myositis-specific autoantibodies (anti-Mi-2, -TIF-1γ, -MDA-5, -NXP-2, -SAE-1, -SRP, -Jo-1, -PL-7, -PL-12, -EJ, -OJ, and -HMGCR). Subsequently, these patients were divided into two clusters, one of which presented a higher frequency of lung involvement and, consequently, of “mechanic’s hands,” Gottron’s sign and papules, and arthritis; therefore, they resembled the phenotypic manifestations of ASyS. The other cluster presented with less lung involvement and a significantly higher heliotrope rash rate. However, these authors [34] did not evaluate myositis-associated antibodies, including anti-PM/Scl; thus, it is possible that in the cluster where the patients’ manifestations are similar to those with ASyS, there is, for instance, a higher prevalence of anti-PM/Scl.

Regarding follow-up, the infection rates were similar between patients in the anti-PM/Scl(+) and anti-PM/Scl(-) groups. Among the studies available in the literature, only two evaluated the infection rate in patients with anti-PM/Scl(+) [12,16], mainly related to hard lung infections associated with ILD [16] and a case of meningitis that evolved to death [12]. However, in the latest study [12], there was no specification if the patient had anti-PM/Scl(+). In the present study, the prevalence of infections was 12.5%, characterized mainly by viral and bacterial infections; however, unlike the literature, it did not present any connection with lung involvement.

Some studies [4,9,12,15–18,20,28] have analyzed mortality rates, which varied between 0% and 10%. In the present study, the mortality prevalence is in agreement with that presented in the literature, since its value is 6.3%, with the referred cause of death being acute myocardial infarction, that is, cardiac involvement. And, despite the variety of causes of death presented in the literature (lung, infectious, cardiac, and neoplastic involvement), the cause of the death in the present study coincides with that of 2.3% of the patients in Váncsa et al. [12] and of 5% of those in Marie et al. [15].

In addition, in the last patient evaluation, our patients with anti-PM/Scl(+) presented a higher frequency of relapses and, therefore, active disease, with a higher use of immunosuppressants. Supporting our data, Marie et al. [15] showed that 12 of 20 anti-PM/Scl(+) patients, without anti-Jo-1, -PL-7, -PL-12, -Mi-2, -Ku, and -Ro-52, presented relapses during the follow-up. Lega et al. [17], for their part, demonstrated that 44% of the patients, with anti-PM/Scl(+) and ILD, experienced disease worsening or death, in the last medical evaluation, wherein all patients were taking corticosteroids and only 44% of them were following a drug treatment associated to immunosuppressants. Espinosa-Ortega et al. [35] realized an analysis of the damage index suffered by the patients with IIM, they included patients with and without myositis-specific and myositis-associated autoantibodies. It was noted that patients with anti-PM/Scl(+) presented a higher rate of increase in the Myositis Damage Index (MDI) extent score per unit of time compared to other patients, indicating greater damage accrual among these patients, showing a faster progression of lung fibrosis.

One limitation of the present study is that it was a retrospective cohort study. However, the data in this study were collected from electronic medical records with pre-standardized and parameterized information, allowing the reliability of the collected data.

Anti-PM/Scl autoantibodies were observed in 17.2% of patients with DM/PM, without SSc, and without other myositis-specific and myositis-associated autoantibodies. Patients with anti-PM/Scl presented a phenotypic pattern similar to that of ASyS, in addition to higher rates of relapse and disease activity.

ACR American College of Rheumatology

ALT Alanine aminotransferase

ANA Antinuclear antibodies

ASyS Antisynthetase syndrome

AST Aspartate aminotransferase

CADM Clinically amyopathic dermatomyositis

CPK Creatine phosphokinase

DM Dermatomyositis

EULAR European League Against Rheumatism

IBM Inclusion body myositis

IIMs Idiopathic inflammatory myopathies

ILD Interstitial lung disease

IMNM Immune-mediated necrotizing myopathy

IVIg Intravenous immunoglobulin

IVMP Intravenous methylprednisolone

LDH Lactic dehydrogenase

MDI Myositis Damage Index

MRC Medical Research Council

PM Polymyositis

SSc Systemic sclerosis

ACKNOLEDGMENTS

Maria Aurora Gomes da Silva for assistance and support in all laboratory processing.

AUTHORS’ CONTRIBUTIONS

All authors contributed equally to the writing and reviewing of the manuscript.

FUNDING

Conselho Nacional de Desenvolvimento Científico e Tecnológico (CNPq): #139748/2024-5 and #301500/2022-3.

AVAILABILITY OF DATA AND MATERIALS

Not applicable.

ETHICS APPROVAL AND CONSENT TO PARTICIPATE

The study was approved by the local ethics committee (CAAE 34954620.5.0000.0068).

CONSENT FOR PUBLICATION

Not applicable.

COMPETING INTERESTS

All authors declare that they have no conflicts of interest.

Lundberg IE, Fujimoto M, Vencovsky J, Aggarwal R, Holmqvist M, Christopher-Stine L, et al. Idiopathic inflammatory myopathies. Nat Rev Dis Primer. 2021;7:86.
Dalakas MC. Autoimmune inflammatory myopathies. Handb Clin Neurol. 2023;195:425-460.
Shinjo SK. Miopatias autoimunes sistêmicas. Rev Paul Reumatol. 2017;16:6-11.
Vandergheynst F, Ocmant A, Sordet C, Humbel RL, Goetz J, Roufosse F, et al. Anti-PM/Scl antibodies in connective tissue disease: Clinical and biological assessment of 14 patients. Clin Exp Rheumatol. 2006;24:129-133.
Raijmakers R, Renz M, Wiemann C, Egberts WV, Seelig HP, van Venrooij WJ, et al. PM-Scl-75 is the main autoantigen in patients with the polymyositis/scleroderma overlap syndrome. Arthritis Rheum. 2004;50:565-569.
Selva-O’Callaghan A, Simeon-Aznar CP. The scleromyositis phenotype. Lessons from a multicentre international cohort of anti-PM/Scl-positive patients. Rheumatolology. 2021;60:4956-4957.
Reichlin M, Maddison PJ, Targoff I, Bunch T, Arnett F, Sharp G, et al. Antibodies to a nuclear/nucleolar antigen in patients with polymyositis overlap syndromes. J Clin Immunol. 1984;4:40-44.
Mahler M, Raijmakers R. Novel aspects of autoantibodies to the PM/Scl complex: clinical, genetic and diagnostic insights. Autoimmun Rev. 2007;6:432-437.
Oddis CV, Okano Y, Rudert WA, Trucco M, Duquesnoy RJ, Medsger TA. Serum autoantibody to the nucleolar antigen PM-Scl. Clinical and immunogenetic associations. Arthritis Rheum. 1992;35:1211-1217.
Júnior JG, Mugii N, Inaoka PT, Sampaio-Barros PD, Shinjo SK. Inflammatory myopathies overlapping with systemic sclerosis: a systematic review. Clin Rheumatol. 2022;41:1951-1963.
Wielosz E, Dryglewska M, Majdan M. The prevalence and significance of anti-PM/Scl antibodies in systemic sclerosis. Ann Agric Environ Med AAEM. 2021;28:189-192.
Váncsa A, Gergely L, Ponyi A, Lakos G, Németh J, Szodoray P, et al. Myositis-specific and myositis-associated antibodies in overlap myositis in comparison to primary dermatopolymyositis: Relevance for clinical classification: retrospective study of 169 patients. Joint Bone Spine. 2010;77:125-130.
Alves SP, Silva MG, Borges IBP, Shinjo SK. Patients with pure dermatomyositis/polymyositis and anti-PM/Scl autoantibody resembling anti-synthetase syndrome. MedicalExpress. 2018;5:1-7.
Ussavarungsi K, Nugent K, Gerke AK, Krasowski MD, Tuetken RS, Lenert PS. Interstitial lung disease associated with anti-PM-Scl antibody: A single center experience. Autoimmun Rev. 2019;18:102355.
Marie I, Lahaxe L, Benveniste O, Delavigne K, Adoue D, Mouthon L, et al. Long-term outcome of patients with polymyositis/ dermatomyositis and anti-PM-Scl antibody. Br J Dermatol. 2010;162:337-344.
Ge Y, Shu XM, He L, Li C, Lu X, Wang G. Interstitial lung disease is a major characteristic of patients who test positive for anti-PM/Scl antibody. Front Med. 2022;8:778211.
Lega JC, Cottin V, Fabien N, Thivolet-Béjui F, Cordier JF. Interstitial lung disease associated with anti-PM/Scl or anti-aminoacyl-tRNA synthetase autoantibodies: a similar condition? J Rheumatol. 2010;37:1000-1009.
De Lorenzo R, Pinal-Fernandez I, Huang W, Albayda J, Tiniakou E, Johnson C, et al. Muscular and extramuscular clinical features of patients with anti-PM/Scl autoantibodies. Neurology. 2018;90:2068-2076.
Kowalski EH, Kenney HM, Richardson CT. Wong-type dermatomyositis with interstitial lung disease and anti-SRP and -PM/Scl antibodies treated with intravenous immunoglobulin. JAAD Case Rep. 2023;41:26-29.
Kohara A, Yanaba K, Muro Y, Ito H, Nakagawa H, Noda K, et al. Anti-PM/Scl antibody-positive dermatomyositis in a Japanese patient: a case report and review of the literature. Int J Rheum Dis. 2017;20:2186-2189.
Lega JC, Reynaud Q, Belot A, Fabien N, Durieu I, Cottin V. Idiopathic inflammatory myopathies and the lung. Eur Respir Soc. 2015;24:216-238.
Muro Y, Hosono Y, Sugiura K, Ogawa Y, Mimori T, Akiyama M. Anti-PM/Scl antibodies are found in Japanese patients with various systemic autoimmune conditions besides myositis and scleroderma. Arthritis Res Ther. 2015;17:57.
Marie I, Lahaxe L, Tiev K, Duval-Modeste AB, Vittecoq O, Levesque H, et al. [Idiopathic inflammatory myopathies with anti-PM-Scl antibodies: case series and literature review]. Rev Med Interne. 2010;31:540-544.
Verma I, Dube AH, Kumbhalkar S, Nagpure K, Sawatkar G, Chuadhari SR, et al. A case of palmoplantar keratoderma in the constellation of connective tissue diseases. Cureus. 2024;16:56531.
Sanz-Correa J, Esteban-Vazquez A, Cobo-Ibáñez T, Caelles IP, Sanz-Sanchez T, Cortes MDML, et al. “Hiker’s feet” in a patient with anti-PM-Scl autoantibodies-associated idiopathic inflammatory myopathy. J Dtsch Dermatol Ges J Ger Soc Dermatol JDDG. 2023;21:534-536.
Taki T, Muro Y, Ogawa Y, Akiyama M. Image Gallery: Palmoplantar hyperkeratosis in dermatomyositis with anti-PM/Scl antibodies. Br J Dermatol. 2017;176:e94.
Srikantharajah D, Lloyd ME, Kiely PDW. Rituximab and intravenous immunoglobulin treatment in PM/Scl antibody-associated disease: case-based review. Rheumatol Int. 2022;42:359-364.
Marguerie C, Bunn CC, Copier J, Bernstein RM, Gilroy JM, Black CM, et al. The clinical and immunogenetic features of patients with autoantibodies to the nucleolar antigen PM-Scl. Medicine (Baltimore). 1992;71:327-336.
Lega JC, Fabien N, Reynaud Q, Durieu I, Durupt S, Dutertre M, et al. The clinical phenotype associated with myositis-specific and associated autoantibodies: a meta-analysis revisiting the so-called antisynthetase syndrome. Autoimmun Rev. 2014;13:883-891.
Lundberg IE, Tjärnlund A, Bottai M, Werth VP, Pilkington C, de Visser M, Alfredsson L, et al. 2017 European League Against Rheumatism/American College of Rheumatology classification criteria for adult and juvenile idiopathic inflammatory myopathies and their major subgroups. Ann Rheum Dis. 2017;76:1955-1964.
Compston A. Aids to the investigation of peripheral nerve injuries. Medical Research Council: Nerve Injuries Research Committee. His Majesty's Stationery Office: 1942; pp. 48 (iii) and 74 figures and 7 diagrams; with aids to the examination of the peripheral nervous system. By Michael O'Brien for the Guarantors of Brain. Saunders Elsevier: 2010; pp. [8] 64 and 94 Figures. Brain. 2010;133:2838-2844.
Cruellas MGP, Viana V dos ST, Levy-Neto M, Souza FHC de, Shinjo SK. Myositis-specific and myositis-associated autoantibody profiles and their clinical associations in a large series of patients with polymyositis and dermatomyositis. Clinics. 2013;68:909-914.
Bohan A, Peter JB. Polymyositis and dermatomyositis (first of two parts). N Engl J Med. 1975;292:344-347.
Li S, Gao S, Chen Q, et al. Clinical heterogeneities and prognoses of patients with myositis specific antibody negative dermatomyositis: a retrospective study in China. Clin Exp Rheumatol. 2022;40(2):284-291.
Espinosa-Ortega F, Lodin K, Dastmalchi M, et al. Autoantibodies and damage in patients with idiopathic inflammatory myopathies: A longitudinal multicenter study from the MYONET international network. Semin Arthritis Rheum. 2024;68:152529.

No competing interests reported.

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Patients with anti-PM/Scl-positive and idiopathic inflammatory myopathy resemble antisynthetase syndrome

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