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SAT0482 Clinical and Serological Features of Patients with Dermatomyositis Complicated by Spontaneous Pneumomediastinum
  1. Z. Chen1,
  2. Y. Wang1,
  3. M. Kuwana2,
  4. L. Sun1
  1. 1Department Of Rheumatology And Immunology, The Affiliated Drum Tower Hospital, Nanjing University Medical School, Nanjing, China., Nanjing, China
  2. 2Department of Allergy and Rheumatology, Nippon Medical School Graduate School of Medicine, Tokyo, Japan

Abstract

Background Interstitial lung disease (ILD) is a common complication and is associated with the prognosis of dermatomyositis (DM). The respiratory function deteriorate quickly and leads to poor prognoses in some individuals, a condition known as rapidly progressive ILD (RP-ILD). Some patients with DM associated RP-ILD present characteristic cutaneous rash of DM but have not clinically significant muscle weakness, a subgroup of DM designated as clinically amyopathic dermatomyositis (CADM) [1]. Recent studies have demonstrated that most patients with DM and RP-ILD are positive for anti-melanoma differentiation-associated gene 5 (MDA5). Spontaneous pneumomediastinum (PNM) is a rare but devastating illness in DM-ILD. Albeit that previous studies have revealed that some patients with DM and PNM have mild muscle involvement and are anti-MDA5 positive [2], the clinical and serological features of this severe entity have not been fully elucidated.

Objectives To explore the clinical and serological features of patients with DM and PNM.

Methods A total of 93 (68 classic DM and 25 CADM) patients were recruited. Anti-MDA5 antibodies were detected using ELISA [3]. Variables were compared between patients with and without PNM. Multivariate analysis was performed using a multivariate logistic regression model. The following variables were assessed as potential factors associated with spontaneous PNM in DM: presence of RP-ILD, DM subgroup (classic DM or CADM), presence of skin ulcers and presence of anti-MDA5 antibodies.

Results A total of 11 patients (7 man and 4 women) developed PNM. Seven patients were diagnosed with CADM while 4 patients were diagnosed with classic DM. Despite being treated with the combination of glucocorticoids and immunosuppressants, during the follow-up period, 6 patients died of respiratory failure, with a varied interval after the occurrence of PNM ranged from 3 to 48 weeks. Compared with DM patients without PNM, DM patients with PNM had significantly higher frequencies of RP-ILD (63.6% vs 24.4%, P =0.01), anti-MDA5 antibodies (90.9% vs 52.4%, P =0.02), CADM diagnoses (63.6% vs 22.0%, P =0.007) and cutaneous ulcers (36.4% vs 10.2%, P =0.04), but significantly lower creatine kinase (CK) levels (58.5 vs 284 ng/ml, P =0.04). The logistic multivariate analysis indicated that cutaneous ulcer was the only independent risk factor for the occurrence of PNM in DM (OR =5.98, 95% CI 1.12–31.98, P=0.037).

Conclusions Spontaneous PNM is a refractory complication and tends to occur in DM patients with RP-ILD, anti-MDA5 antibody, CADM diagnosis and low CK level, especially in patients with cutaneous ulcers.

References

  1. Sato S, Kuwana M (2010) Clinically amyopathic dermatomyositis. Curr Opin Rheumatol 22:639-643. doi:10.1097/BOR.0b013e32833f1987

  2. Le Goff B, Cherin P, Cantagrel A, Gayraud M, Hachulla E, Laborde F, Papo T, Sibilia J, Zabraniecki L, Ravaud P, Puechal X (2009) Pneumomediastinum in interstitial lung disease associated with dermatomyositis and polymyositis. Arthritis Rheum 61 (1):108-118. doi:10.1002/art.24372

  3. Sato S, Hoshino K, Satoh T, Fujita T, Kawakami Y, Fujita T, Kuwana M (2009) RNA helicase encoded by melanoma differentiation-associated gene 5 is a major autoantigen in patients with clinically amyopathic dermatomyositis: Association with rapidly progressive ILD. Arthritis Rheum 60:2193-2200. doi:10.1002/art.24621

Disclosure of Interest None declared

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