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Kim, Kim, Kim, Sohn, Jung, Choi, Sung, Jun, Uhm, Kim, Bae, and Yoo: Clinicial Significance of Spontaneous Pneumomediastinum in Dermatomyositis/Polymyositis
Original Article

J Korean Rheum Assoc 2010;17(2):143-152.

Published online: 23 June 2010

DOI: https://doi.org/10.4078/jkra.2010.17.2.143

Clinicial Significance of Spontaneous Pneumomediastinum in Dermatomyositis/Polymyositis

Jin Ju Kim1, Dam Kim1, Eun Kyoung Kim1, Il Woong Sohn1, Kyong Hee Jung2, Chan-Bum Choi1, 2, Yoon-Kyoung Sung1, 2, Jae-Bum Jun1, 2, Wan-sik Uhm1, 2, Tae Hwan Kim1, 2, Sang Cheol Bae1, 2, Dae Hyun Yoo1, 2

1Department of Internal Medicine, College of Medicine, Hanyang University, Seoul, Korea

2Department of Internal Medicine, The Hospital for Rheumatic Diseases, College of Medicine, Hanyang University, Seoul, Korea

Received 27 April 201013 May 2010       Accepted 13 May 2010

Copyright © 2010 The Korean Rheumatism Association

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Objective

Pneumomediastinum (PnM), a rare complication of dermatomyositis and polymyositis (DM/PM), is sporadic and has an unclear pathogenesis. PnM is almost always associated with interstitial lung disease (ILD), and is a poor prognostic factor in inflammatory myositis patients. We studied the prevalence of PnM in Korean DM/PM and its clinical significance.

Methods

We retrospectively studied the medical records of 161 patients diagnosed with DM/PM meeting Bohan-Peter's criteria at Hanyang University Hospital for Rheumatic Diseases from 1995 to 2010. We collected following findings; demographic data, diagnosis, lung involvement, cause of death, and duration from diagnosis to death.

Results

One hundred nineteen patients (73.9%) were DM and 42 patients (26.1%) were PM. Eighty three patients (51.6%) developed ILD at diagnosis or during follow up. Eighteen patients (11.2%) died because of ILD aggravation, infection, or malignancy. The mean duration from diagnosis to death was 11.5 months, with 10 patients (6.2%) dying from from ILD aggravation but none with spontaneous PnM. 6 patients (3.7%) presented with PnM, and it was associated with ILD worsening in all cases. PnM resolved with O2 inhalation, corticosteroids, and/or immunosuppressive agents after 11 weeks (mean) of therapy

Conclusion

PnM is rare but associates with DM and aggravation of ILD. PnM does not usually cause fatalities and can be cured by appropriate therapy.

Keywords: Pneumomediastinum, Dermatomyositis/polymyositis, Interstitial lung disease, Prognosis

REFERENCES

1). Dickey BF., Myers AR. Pulmonary disease in polymyositis/dermatomyositis. Semin Arthritis Rheum. 1984. 14:60–76.
crossref
2). Marie I., Hachulla E., Cherin P., Dominique S., Hatron PY., Hellot MF, et al. Interstitial lung disease in polymyositis and dermatomyositis. Arthritis Rheum. 2002. 47:614–22.
crossref
3). Kang EH., Lee EB., Shin KC., Im CH., Chung DH., Han SK, et al. Interstitial lung disease in patients with polymyositis, dermatomyositis and amyopathic dermatomyositis. Rheumatology (Oxford). 2005. 44:1282–6.
crossref
4). Kim DH., Park JH., Chei CS., Hwang SW., Kim HY., Yoo BH. Spontaneous Pneumomediastinum: clinical investigation. Korean J Thorac Cardiovasc Surg. 2006. 39:220–5.
5). Lee SY., Jo YM., Kim MS., Kim HI., Lee SW., Chung WT. A Case of Pneumomediastinum in the Course of Treatment in Lupus Myocarditis. J Korean Rheum Assoc. 2008. 15:322–7.
crossref
6). Patel A., Kesler B., Wise RA. Persistent pneumomediastinum in interstitial fibrosis associated with rheumatoid arthritis: treatment with high-concentration oxygen. Chest. 2000. 117:1809–13.
7). Kishi K., Kashiwabara K., Narushima K., Sarashina G., Kobayashi K., Yagyu H, et al. Mixed connective tissue disease-associated interstitial pneumonia complicated by pneumomediastinum during prednisolone therapy. Nihon Kokyuki Gakkai Zasshi. 2000. 38:480–4.
8). Matsuda Y., Tomii M., Kashiwazaki S. Fatal pneumomediastinum in dermatomyositis without creatine kinase elevation. Intern Med. 1993. 32:643–7.
crossref
9). Yamanishi Y., Maeda H., Konishi F., Hiyama K., Yamana S., Ishioka S, et al. Dermatomyositis associated with rapidly progressive fatal interstitial pneumonitis and pneumomediastinum. Scand J Rheumatol. 1999. 28:58–61.
10). Kuroda T., Morikawa H., Satou T., Tanabe Y., Murakami S., Ito S, et al. A case of dermatomyositis complicated with pneumomediastinum successfully treated with cyclosporin A. Clin Rheumatol. 2003. 22:45–8.
crossref
11). Rhee K., Jung C., Bae J., So M., Oh J., Kim S, et al. A case of dermatomyositis complicated with Pneumomediastinum successfully treated with Cyclosporin A. J Korean Rheum Assoc. 2006. 13:160–5.
12). Yoshida K., Kurosaka D., Kingetsu I., Hirai K., Yamada A. Pneumomediastinum in dermatomyositis itself is not a poor prognostic factor: report of a case and review of the literature. Rheumatol Int. 2008. 28:913–7.
crossref
13). Bohan A., Peter JB. Polymyositis and dermatomyositis (first of two parts) N Engl J Med. 1975. 292:344–7.
14). Lynch D., Travis W., Muller N., Galvin J., Hansell D., Grenier P, et al. Idiopathic Interstitial Pneumonias: CT Features. Radiology. 2005. 236:10.
crossref
15). Kim HI., Back HK., Jung JK., Jo YM., Lee SY., Lee SW, et al. Prognostic factors affecting survival rate in inflammatory myositis. J Korean Rheum Assoc. 2009. 6:108–14.
crossref
16). Schwarz MI., Matthay RA., Sahn SA., Stanford RE., Marmorstein BL., Scheinhorn DJ. Interstitial lung disease in polymyositis and dermatomyositis: analysis of six cases and review of the literature. Medicine (Baltimore). 1976. 55:89–104.
crossref
17). Kono H., Inokuma S., Nakayama H., Suzuki M. Pneumomediastinum in dermatomyositis: association with cutaneous vasculopathy. Ann Rheum Dis. 2000. 59:372–6.
crossref
18). Barvaux V., Van Mullem X., Pieters T., Houssiau F. Persistent pneumomediastinum and dermatomyositis: a case report and review of the literature. Clin Rheumatol. 2001. 20:359–61.
crossref
19). Terao M., Ozawa K., Inui S., Murota H., Yokomi A., Itami S. A case of dermatomyositis complicated with pneumomediastinum. Mod Rheumatol. 2007. 17:156–9.
crossref
20). de Souza Neves F., Shinjo S., Carvalho J., Levy-Neto M., Borges C. Spontaneous pneumomediastinum and dermatomyositis may be a not so rare association: report of a case and review of the literature. Clin Rheumatol. 2007. 26:105–7.
crossref
21). Jansen T., Barrera P., Van Engelen B., Cox N., Laan R., Van de Putte L. Dermatomyositis with subclinical myositis and spontaneous pneumomediastinum with pneumothorax: case report and review of the literature. Clin Exp Rheumatol. 1998. 16:733–5.
22). Cicuttini FM., Fraser KJ. Recurrent pneumomediastinum in adult dermatomyositis. J Rheumatol. 1989. 16:384–6.
23). Tong S., Shi X., Su J., Zhao Y., Zhang F. Clinical analysis of pneumomediastinum complicated in polymyositis and dermatomyositis. Zhonghua Yi Xue Za Zhi. 2006. 86:624.
24). Le Goff B., Cherin P., Cantagrel A., Gayraud M., Hachulla E., Laborde F, et al. Pneumomediastinum in interstitial lung disease associated with dermatomyositis and polymyositis. Arthritis Care Res. 2008. 61:108–18.
crossref
25). Park SH., Kum YS., Kim KC., Choe JY., Kim SK. Pneumomediastinum and subcutaneous emphysema secondary to amyopathic dermatomyositis with cryptogenic organizing pneumonia in invasive breast cancer: a case report and review of literature. Rheumatol Int. 2009. 29:1231–5.
crossref
26). Kim HJ., Hong YK., Yoo WH. Dermatomyositis, complicated with pneumomediastinum, successfully treated with cyclosporine A: a case report and review of literature. Rheumatol Int. 2009. 29:1101–4.
crossref
27). Oh JH., Ko JJ., Lee CK., Ko YH., Yun KS., Lim YS, et al. A case of spontaneous pneumomediastinum and subcutaneous emphysema in dermatomyositis: Including analysis of cases with dermatomyositis and pneumomediastinum in the literature. Koreran J Med. 1998. 55:131–6.
28). Park JH., Park SH., Kim PS., Lee JW., Yoo WH., Joo YS, et al. Fatal pneumomediastinum and subcutaneous emphysema in a patient with dermatomyositis. J Korean Rheum Assoc. 1999. 6:287.
29). Song JS., Park YB., Lee JG., Kwon KH., Lee WK., Suh CH, et al. Two cases of spontaneous pneumomediastinum in dermatomyositis. J Korean Rheum Assoc. 1998. 5:152–7.
30). Lee HY., Moon HI., Kang KY., Ju JH., Park SH., Kim HY. Two cases of Spontaneous pneumomediastinum in amyopathic dermatomyositis. Korean J Med. 2009. 77:479–83.
31). Hochberg M., Feldman D., Stevens M., Arnett F., Reichlin M. Antibody to Jo-1 in polymyositis/dermatomyositis: association with interstitial pulmonary disease. J Rheumatol. 1984. 11:663.
32). Fudman E., Schnitzer T. Dermatomyositis without creatine kinase elevation. A poor prognostic sign. Am J Med. 1986. 80:329–32.
crossref

Classification and patterns of Interstitial lung disease
Morphologic pattern Clinical diagnosis Histologic features Imaging features
Usual interstitial pneumonia Idiopathic pulmonary fibrosis Spatial and temporal heterogeneity, dense fibrosis, fibroblastic foci, honeycombing Basal, peripheral predominance, often patchy, reticular abnormality, honeycombing
Nonspecific interstitial pneumonia Nonspecific interstitial pneumonia Spatially and temporally homogeneous lung fibrosis or inflammation Basal predominance, ground-glass abnormality, reticular abnormality
Desquamative interstitial pneumonia Desquamative interstitial pneumonia Diffuse macrophage accumulation in alveoli Basal, peripheral predominance ground-glass attenuation; sometimes cysts
Respiratory bronchiolitis Respiratory bronchiolitis-associated interstitial lung disease Peribronchiolar macrophage accumulation, bronchiolar fibrosis; macrophages have dusty, brown cytoplasm Centrilobular nodules, ground-glass attenuation
Organizing pneumonia COP, BOOP Patchy distribution of intraluminal organizing fibrosis in distal airspaces; preservation of lung architecture; uniform temporal appearance; mild interstitial chronic inflammation Ground-glass attenuation; consolidation basal, peripheral predominance
Diffuse alveolar damage Acute interstitial pneumonia Diffuse distribution, uniform temporal appearance, alveolar septal thickening due to organizing fibrosis, airspace organization, hyaline membranes Diffuse, ground-glass attenuation, consolidation
Lymphoid interstitial pneumonia Lymphoid interstitial pneumonia Diffuse lymphoplasmacytic infiltration of alveolar septa Ground-glass attenuation, cysts

COP: cryptogenic organizing pneumonia, BOOP: bronchiectasis obliterans with organizing pneumonia

Characteristics of dermatomyositis/polymyositis patients
Dermatomyositis Polymyositis
Numbers of patients 119 (73.9%) 42 (26.1%)
Mean age at diagnosis (years) 43.6 (6~75) 38.7 (16~75)
Mean period of follow up (months) 26.6 (1~155) 32.6 (1~121)
Female/Male 94/25 36/6
Interstitial lung disease 69 (42.9%) 14 (33.3%)
Malignancy 11 (9.2%) 2 (4.8%)
Death causes 16 (13.4%) 2 (4.8%)
Interstitial lung disease 9 1
Malignancy 4 1
Sepsis 3
Radiologic classification of interstitial lung disease
IPF BOOP NSIP AIP Mixed Unknown
Numbers 14 30 25 1 8 5
Result
Alive (PnM) 14 26 (4) 23 (1) 0 4 (1) 5
Expire 0 4 2 1 4 0

IPF: idiopathic pulmonary fibrosis, BOOP: bronchiectasis obliterans with organizing pneumonia, NSIP: non-specific interstitial pneumonitis, AIP: acute interstitial pneumonitis, PnM: pneumomediastinum

Clinical course of interstitial lung disease among dermatomyositis/polymyositis patients
No interval change Slow progression Rapid progression Unknown
Numbers 51 17 10 5
Result
Alive (PnM) 51 16 (6) 0 5
Expire 1 10 0

PnM: pneumomediastinum

Characteristics of 6 cases of spontaneous pneumomediastinum at dermatomyositis/polymyositis patients
Age/sex CK LDH Aldo. ANA Anti Jo-1 CV ILD agg. Treatment before PnM PM/DM, time to PnM Treatment after PnM Recovery
59/F 24 142 18.4 (+)∗ (–) (–) (+) PDS DM, initial. Steroid pulse, CsA 5 month
51/F 32 305 5.4 (–) (– (–) (+) PDS, CsA, HCQ DM, 1 month Steroid pulse, CsA, O2 2 month
42/F 166 267 37.6 (+)† (– (–) (+) PDS, CsA, HCQ DM, 2 years Steroid pulse, CsA, O2 2 weeks
43/F 125 448 12.9 (–) (–) (–) (+) PDS, CsA DM, 6 month IVIG, PDS, CsA, HCQ, O2, 5 month
44/F 49 251 3.7 (+)‡ (–) (–) (+) PDS, AZA PM, initial. PDS, CsA, O2, Collar 2 month
recur 290 346 7.1 (+) PDS, CsA DM, 1 week incision Unaltered, O2 1 month
54/F 26 122 7.1 (–) (–) (–) (+) PDS, CsA DM, 1 year IVIG, O2, PDS, Tacrolimus 2 weeks

Aldo.: aldolase, CV: cutaneous vasculopathy, agg.: aggravation, PDS: prednisolone, CsA: cyclosporine A, HCQ: hydroxycloroquinolone, AZA: azathioprine, IVIG: intravascular immunoglobulin. ∗Speckled pattern 1 : 640, †Cytoplasmic pattern 1 : 320, ‡Cytoplasmic patthern 1 : 160

Comparison of clinical features between Interstitial lung disease patients with and without pneumomediastinum
Pneumomediastinum (+) Pneumomediastium (–)
Numbers of patients 6 (7.1%) 77 (92.9%)
Age 48.8 (42~59) 45.4 (27~75)
Male/Female 0/6 14/63
DM/PM 6/0 63/14
Follow up duration (months) 22 (2~57) 30.3 (1~155)
ANA (+) 3 (50%) 47 (61.0%)
CK/LDH (mean) 101.7/268.7 596.4/358.3
Review of reported spontaneous pneumomediastinum cases with dermatomyositis/polymyositis in Korean
Ref. Age/Sex CK Aldolase Anti Jo-1 CV ILD agg. Treatment before PnM PM/DM, time to PnM Treatment after PnM Recovery
11 40/F 1,521 22.6 (–) (+) (+) PDS, IVIG DM, 3 weeks Steroid IV, CsA, O2 3 weeks
25 45/F NL ? (–) (+) (+) PDS, AZA, CPM DM, 2 months Unaltered 3 month
26 38/F 131 13.2 (–) (+) (+) PDS, HCQ DM, 4 months Unaltered, O2 1 month
27 18/M 4.6 13.8 (–) (–) (+) PDS, HCQ, IVIG DM, 1 months PDS, CPM, O2, thoracostomy Expire (2 m.)
28 56/F 1,405 ? (–) (–) (+) PDS DM, 2 weeks PDS, O2 Expire (1 m.)
29 53/F 20 ? (–) (–) (+) PDS, AZA DM, ? Unaltered, O2 ?
29 41/F 38 ? (–) (–) (+) PDS DM, 1 month PDS, AZA, O2 2 weeks
30 36/F 73 ? (–) (+) (+) PDS, MTX, HCQ DM, 4 months PDS, HCQ, MTX, IVIG, O2 Expire (1 m.)
30 45/F 156 ? (–) (+) (+) PDS, HCQ DM, 11 months Steroid IV, CsA IV, O2 Expire (1 m.)

Ref.: reference, CV: cutaneous vasculopathy, agg.: aggravation, NL: normal, ?: unknown, PDS: prednisolone, CsA: cyclosporine A, HCQ: hydroxycloroquinolone, AZA: azathioprine, IVIG: intravascular immunoglobulin, M: month

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