nach oben

Erschienen in:

07.02.2024 | Original Article

Clinical and immunological characteristics and prognosis of patients with autoantibody negative dermatomyositis: a case control study

verfasst von: Xiaoyan Xing, Yuzhou Gan, Wanxing Mo, Jian Zhang, Naidi Wang, Kai Zhang, Ke Ma, Lihua Zhang, Lin Ma, Dan Lu, Yuhui Li, Jing He

Erschienen in: Clinical Rheumatology | Ausgabe 3/2024

Einloggen, um Zugang zu erhalten

Abstract

Objectives

Myositis-specific antibodies (MSAs) and myositis-associated antibodies (MAAs) are associated with distinctive dermatomyositis (DM) clinical phenotypes. The aim of this study is to explicate the clinical and immunological features of MSAs-negative DM patients.

Methods

A total of 515 individuals diagnosed with DM was screened from 2013 to 2022 and 220 DM patients were enrolled in this retrospective cohort. Clinical and laboratory data of these patients were analyzed.

Results

MSAs-negative DM patients were categorized into two groups: MAAs-negative (MSAs (−)/MAAs (−)) group and MAAs-positive (MSAs (−)/MAAs (+)) group. The percentage of Raynaud’s phenomenon (P=0.026) was higher in the MSAs (−)/MAAs (+) DM patients than the MSAs-positive DM patients and MSAs (−)/MAAs (−) DM patients. The proportion of rapidly progressive interstitial lung disease (RP-ILD) in the MSAs-negative DM patients was lower than that in the MSAs-positive group. The MSAs (−)/MAAs (+) group had a higher proportion of organizing pneumonia and usual interstitial pneumonia (P=0.011), and elevated eosinophils in their bronchoalveolar lavage fluid (P=0.008). Counts of lymphocytes (P=0.001) and CD16⁺CD56⁺ natural killer (NK) cells (P=0.012) were higher in the MSAs-negative group. Additionally, the percentage of CD4⁺TNFα⁺ (P=0.040), CD4⁺IFNγ⁺ (P=0.037), and CD4⁺IL-2⁺ (P=0.018) cells among total CD4⁺ T cells were higher in the MSA-negative DM patients compared with the MSAs-positive DM patients. Besides, MSAs-negative patients demonstrated a more favorable prognosis than MSAs-positive patients. Multivariable regression analysis identified advanced onset age, higher level of carcinoembryonic antigen (CEA), and RP-ILD as risk factors for mortality in DM patients.

Conclusions

Compared with MSAs-positive group, MSAs-negative DM patients suffered less from organ involvement compared with MSAs-positive group and tend to have better prognosis.

Key Points

MSAs-negative DM patients exhibited distinct characteristics in comparison with MSAs-positive DM patients:

• The MSAs (−)/MAAs (+) DM patients demonstrated a higher prevalence of organizing pneumonia (OP) and usual interstitial pneumonia (UIP), and elevated eosinophil counts in bronchoalveolar lavage fluid.

• CEA levels were lower in MSAs-negative patients compared with MSAs-positive patients.

• Elevated counts of lymphocytes and CD16⁺CD56⁺ NK cells were identified in the MSAs-negative patients. Additionally, proportions of CD4⁺TNFα⁺, CD4⁺IFNγ⁺, and CD4⁺IL-2⁺ cells among total CD4⁺ T cells were higher in the MSAs-negative DM patients compared with DM MSAs-positive DM patients.

• MSAs-negative DM patients had a more favorable prognosis than MSAs-positive DM patients. A multivariable regression analysis revealed the advanced onset age, high CEA levels, and RP-ILD were risk factors for mortality in DM patients.

Lundberg IE, Fujimoto M, Vencovsky J, Aggarwal R, Holmqvist M, Christopher-Stine L, Mammen AL, Miller FW (2021) Idiopathic inflammatory myopathies. Nat Rev Dis Primers 7:86CrossRefPubMed

Mammen AL, Allenbach Y, Stenzel W, Benveniste O, Group EtWS (2020) 239th ENMC International Workshop: classification of dermatomyositis, Amsterdam, the Netherlands, 14-16 December 2018. Neuromuscul Disord 30:70–92CrossRefPubMed

Yang H, Peng Q, Yin L, Li S, Shi J, Zhang Y, Lu X, Shu X, Zhang S, Wang G (2017) Identification of multiple cancer-associated myositis-specific autoantibodies in idiopathic inflammatory myopathies: a large longitudinal cohort study. Arthritis Res Ther 19:259CrossRefPubMedPubMedCentral

Hamaguchi Y, Kuwana M, Hoshino K, Hasegawa M, Kaji K, Matsushita T, Komura K, Nakamura M, Kodera M, Suga N, Higashi A, Ogusu K, Tsutsui K, Furusaki A, Tanabe H, Sasaoka S, Muro Y, Yoshikawa M, Ishiguro N et al (2011) Clinical correlations with dermatomyositis-specific autoantibodies in adult Japanese patients with dermatomyositis: a multicenter cross-sectional study. Arch Dermatol 147:391–398CrossRefPubMed

Moghadam-Kia S, Oddis CV, Sato S, Kuwana M, Aggarwal R (2016) Anti-melanoma differentiation-associated gene 5 is associated with rapidly progressive lung disease and poor survival in US patients with amyopathic and myopathic dermatomyositis. Arthritis Care Res (Hoboken) 68:689–694CrossRefPubMed

Travis WD, Costabel U, Hansell DM, King TE Jr, Lynch DA, Nicholson AG, Ryerson CJ, Ryu JH, Selman M, Wells AU, Behr J, Bouros D, Brown KK, Colby TV, Collard HR, Cordeiro CR, Cottin V, Crestani B, Drent M et al (2013) An official American Thoracic Society/European Respiratory Society statement: update of the international multidisciplinary classification of the idiopathic interstitial pneumonias. Am J Respir Crit Care Med 188:733–748CrossRefPubMedPubMedCentral

American Thoracic Society (2000) Idiopathic pulmonary fibrosis: diagnosis and treatment. International consensus statement. American Thoracic Society (ATS), and the European Respiratory Society (ERS). Am J Respir Crit Care Med 161:646–664CrossRef

Badesch DB, Champion HC, Gomez Sanchez MA, Hoeper MM, Loyd JE, Manes A, McGoon M, Naeije R, Olschewski H, Oudiz RJ, Torbicki A (2009) Diagnosis and assessment of pulmonary arterial hypertension. J Am Coll Cardiol 54:S55–S66CrossRefPubMed

Lilleker JB, Vencovsky J, Wang G, Wedderburn LR, Diederichsen LP, Schmidt J, Oakley P, Benveniste O, Danieli MG, Danko K, Thuy NTP, Vazquez-Del Mercado M, Andersson H, De Paepe B, deBleecker JL, Maurer B, McCann LJ, Pipitone N, McHugh N, Betteridge ZE, New P, Cooper RG, Ollier WE, Lamb JA, Krogh NS, Lundberg IE, Chinoy H, all EuroMyositis c (2018) The EuroMyositis registry: an international collaborative tool to facilitate myositis research. Ann Rheum Dis 77:30-39

10.

Watanabe E, Gono T, Kuwana M, Terai C (2020) Predictive factors for sustained remission with stratification by myositis-specific autoantibodies in adult polymyositis/dermatomyositis. Rheumatology 59:586–593PubMed

11.

Betteridge Z, Tansley S, Shaddick G, Chinoy H, Cooper RG, New RP, Lilleker JB, Vencovsky J, Chazarain L, Danko K, Nagy-Vincze M, Bodoki L, Dastmalchi M, Ekholm L, Lundberg IE, McHugh N, Mann H, Krystufkova O, Klein M et al (2019) Frequency, mutual exclusivity and clinical associations of myositis autoantibodies in a combined European cohort of idiopathic inflammatory myopathy patients. J Autoimmun 101:48–55CrossRefPubMedPubMedCentral

12.

Chen F, Wang J, Zhang P, Zuo Y, Ye L, Wang G, Shu X (2022) Interstitial lung disease in dermatomyositis without myositis-specific and myositis-associated autoantibodies: study of a series of 72 patients from a single cohort. Front Immunol 13:879266CrossRefPubMedPubMedCentral

13.

Wang Q, Gao C, Zhang C, Yao M, Liang W, Sun W, Zheng Z (2022) Tumor markers are associated with rapidly progressive interstitial lung disease in adult-dermatomyositis. Clin Rheumatol 41:1731–1739CrossRefPubMed

14.

He L, Ge Y, Li S, Huang K, Liu X, Chen F, Li S, Yang H, Lu X, Wang G, Shu X (2021) Clinical role of bronchoalveolar lavage in dermatomyositis-associated interstitial lung disease. Rheumatology (Oxford) 61:345–354CrossRefPubMed

15.

Jin Q, Fu L, Yang H, Chen X, Lin S, Huang Z, Gao B, Tian X, Jiang W, Shu X, Lu X, Wang G, Peng Q (2023) Peripheral lymphocyte count defines the clinical phenotypes and prognosis in patients with anti-MDA5-positive dermatomyositis. J Intern Med 293:494–507CrossRefPubMed

16.

Zhao XH, Si SK (2023) Five genes as diagnostic biomarkers of dermatomyositis and their correlation with immune cell infiltration. Front Immunol 14:1053099

17.

Pawlitzki M, Nelke C, Rolfes L, Hasseli R, Tomaras S, Feist E, Schanzer A, Rauber S, Regner L, Preusse C, Allenbach Y, Benveniste O, Wiendl H, Stenzel W, Meuth SG, Ruck T (2021) NK cell patterns in idiopathic inflammatory myopathies with pulmonary affection. Cells 10(10):2551

18.

Yang Y, Day J, Souza-Fonseca Guimaraes F, Wicks IP, Louis C (2021) Natural killer cells in inflammatory autoimmune diseases. Clin Transl Immunology 10:e1250CrossRefPubMedPubMedCentral

19.

Feng M, Guo H, Zhang C, Wang Y, Liang Z, Zhao X, Qin Y, Wu Y, Liu G, Gao C, Luo J (2019) Absolute reduction of regulatory T cells and regulatory effect of short-term and low-dose IL-2 in polymyositis or dermatomyositis. Int Immunopharmacol 77:105912CrossRefPubMed

20.

De Bleecker JL, De Paepe B, Aronica E, de Visser M, Group EMMBS, Amato A, Aronica E, Benveniste O, De Bleecker J, de Boer O, De Paepe B, de Visser M, Dimachkie M, Gherardi R, Goebel HH, Hilton-Jones D, Holton J, Lundberg IE, Mammen A et al (2015) 205th ENMC International Workshop: pathology diagnosis of idiopathic inflammatory myopathies part II 28-30 March 2014, Naarden, The Netherlands. Neuromuscul Disord 25:268–272CrossRefPubMed

21.

Tanboon J, Inoue M, Saito Y, Tachimori H, Hayashi S, Noguchi S, Okiyama N, Fujimoto M, Nishino I (2022) Dermatomyositis: muscle pathology according to antibody subtypes. Neurology 98:e739–e749CrossRefPubMedPubMedCentral

22.

Li S, Gao S, Chen Q, Han J, Zhang L, Ge Y, Zuo Y, Duan J, Lu X, Wang G (2022) Clinical heterogeneities and prognoses of patients with myositis specific antibody negative dermatomyositis: a retrospective study in China. Clin Exp Rheumatol 40:284–291CrossRefPubMed

23.

Zhu D, Qiao J, Tang S, Pan Y, Li S, Yang C, Fang H (2021) Elevated carcinoembryonic antigen predicts rapidly progressive interstitial lung disease in clinically amyopathic dermatomyositis. Rheumatology (Oxford) 60:3896–3903CrossRefPubMed

Titel: Clinical and immunological characteristics and prognosis of patients with autoantibody negative dermatomyositis: a case control study
verfasst von: Xiaoyan Xing
Yuzhou Gan
Wanxing Mo
Jian Zhang
Naidi Wang
Kai Zhang
Ke Ma
Lihua Zhang
Lin Ma
Dan Lu
Yuhui Li
Jing He
Publikationsdatum: 07.02.2024
Verlag: Springer International Publishing
Erschienen in: Clinical Rheumatology / Ausgabe 3/2024
Print ISSN: 0770-3198
Elektronische ISSN: 1434-9949
DOI: https://doi.org/10.1007/s10067-024-06873-z

Leitlinien kompakt für die Innere Medizin

Mit medbee Pocketcards sicher entscheiden.

^{Seit 2022 gehört die medbee GmbH zum Springer Medizin Verlag}

Kostenlos registrieren

Update Innere Medizin

Bestellen Sie unseren Fach-Newsletter und bleiben Sie gut informiert.

Newsletter bestellen

Live-Webinar "Urologie und Sexualmedizin in der Praxis"

Springer Medizin

Clinical and immunological characteristics and prognosis of patients with autoantibody negative dermatomyositis: a case control study