Pneumorrhachis and pneumomediastinum in connective tissue disease-related interstitial lung disease: case series from a tertiary care teaching hospital in South India

Springer Science and Business Media LLC - Tập 32 - Trang 1415-1419 - 2011
P. Sandhya1, Shyamkumar Nidugala Keshava2, Debashish Danda1, Prasanta Padhan1, John Mathew1, Sridhar Gibikote2
1Department of Clinical Immunology and Rheumatology, Christian Medical College and Hospital, Vellore, India
2Department of Radiology, Christian Medical College, Vellore, India

Tóm tắt

Pneumomediastinum has been described as a rare complication of connective tissue diseases. Here, we report four cases of pneumomediastinum: three of which are associated with dermatomyositis and one with mixed connective tissue disease. All our patients had interstitial lung disease. The first case of dermatomyositis described below was complicated by epidural emphysema (pneumorrhachis) in addition to pneumomediastinum. Pneumorrhachis is reported in many isolated case reports and series in the setting of asthma, pneumothorax, blunt chest trauma, etc. Less than 10% of pneumomediastinum cases develop this complication and vast majority of cases resolve spontaneously. The mechanism behind this has been postulated to be the passage of air through the intervertebral foramen. Others suggest entrapment of air which dissects between paraspinal soft tissues and along the vascular and nerve sheaths into the epidural space. This is the first ever reported case of epidural emphysema in connective tissue disease to the best of our knowledge.

Tài liệu tham khảo

Bousquet E, Rolland Y, Bon E, Cantagrel A, Tubery M, Murris M et al (1996) Pneumomediastinum, a rare complication of dermatomyositis. Report of a case. Rev Rheum Engle Ed 63(6):457–459 de Toro Santos FJ, Verea-Hernando H, Montero C, Blanco-Aparicio M, Torres Lanzas J, Pombo Felipe F (1995) Chronic pneumomediastinum and subcutaneous emphysema: association with dermatomyositis. Respiration 62:53–56 Santiago MB, Chalhoub M, Pereira ST (1998) Amyopathic dermatomyositis complicated by interstitial pulmonary disease and pneumomediastinum. J Rheumatol 25:2042–2043 Nagai Y, Ishikawa O, Miyachi Y (1997) Pneumomediastinum and subcutaneous emphysema associated with fatal interstitial pneumonia in dermatomyositis. J Dermatol 24:482–484 Matsuda Y, Tomii M, Kashiwazaki S (1993) Fatal pneumomediastinum in dermatomyositis without creatine kinase elevation. Intern Med 32:643–647 Cicuttini FM, Fraser KJ (1989) Recurrent pneumomediastinum in adult dermatomyositis. J Rheumatol 16:384–386 Le Goff B, Chérin P, Cantagrel A, Gayraud M, Hachulla E, Laborde F et al (2009) Pneumomediastinum in interstitial lung disease associated with dermatomyositis and polymyositis. Arthritis Rheum 61(1):108–118 Richards AJ, Talbot IC, Swinson DR, Hamilton EB (1975) Diffuse pulmonary fibrosis and bilateral pneumothoraces in systemic lupus erythematosus. Postgrad Med J 51:851–855 Masuda A, Tsushima T, Shizume K, Mochizuki T, Isono K, Demura H et al (1990) Recurrent pneumothoraces and mediastinal emphysema in systemic lupus erythematosus. J Rheumatol 17:544–548 Paira SO, Roverano S (1992) Bilateral pneumothorax and mediastinal emphysema in systemic lupus erythematosus. Clin Rheumatol 11:571–573 Koczka CP, Otterbeck P, Sharma V (2009) Pneumomediastinum in a 21-year-old female with lupus. South Med J 102(1):112–113 Patel A, Kesler B, Wise RA (2000) Persistent pneumomediastinum in interstitial fibrosis associated with rheumatoid arthritis: treatment with high-concentration oxygen. Chest 117:1809–1813 Mohammad A, Boon Low T, O’Dwyer D, McElvaney G, Kearns G (2007) Spontaneous pneumo-mediastinum in systemic sclerosis: a case report. Rheumatology (Oxford) 46:1376–1377 Teixeira Moreira Almeida Mdo S, Dias LT, Fernandes SJ, Almeida JV (2007) Spontaneous pneumomediastinum and subcutaneous emphysema in systemic sclerosis. Rheumatol Int 27:675–677 Kishi K, Kashiwabara K, Narushima K, Sarashina G, Kobayashi K, Yagyu H et al (2000) Mixed connective tissue disease-associated interstitial pneumonia complicated by pneumomediastinum during prednisolone therapy. Nihon Kokyuki Gakkai Zasshi 38(6):480–484 Japanese Koelliker PD, Brannam LA (1999) Epidural pneumatosis associated with spontaneous pneumomediastinum: case report and review of the literature. J Emerg Med 17(2):247–250 Aribas OK, Gormus N, Aydogdu Kiresi D (2001) Epidural emphysema associated with primary spontaneous pneumothorax. Eur J Cardiothorac Surg 20(3):645–646 Scialdone CJ, Wagle W (1990) Intraspinal air: an unusual manifestation of blunt chest trauma. Clin Imaging 14(1):59–60 Pifferi M, Marrazzini G, Baldini G et al (1997) Epidural emphysema in children with asthma. Pediatr Pulmonol 24:125–126 Hazouard E, Koninck JC, Attucci S, Fauchier-Rolland F, Brunereau L, Diot P (2001) Pneumorachis and pneumomediastinum caused by repeated Muller’s maneuvers: complications of marijuana smoking. Ann Emerg Med 38(6):694–697 Belotti EA, Rizzi M, Rodoni-Cassis P, Ragazzi M, Zanolari-Caledrerari M, Bianchetti MG (2010) Air within the spinal canal in spontaneous pneumomediastinum. Chest 137(5):1197–1200 Oertel MF, Korinth MC, Reinges MH, Krings T, Terbeck S, Gilsbach JM (2006) Pathogenesis, diagnosis and management of pneumorrhachis. Eur Spine J 15(Suppl 5):636–643 Jansen TL, Barrera P, van Engelen BG, Cox N, Laan RF, van de Putte LB (1998) Dermatomyositis with subclinical myositis and spontaneous pneumomediastinum with pneumothorax: case report and review of the literature. Clin Exp Rheumatol 16:733–735 Kono H, Inokuma S, Nakayama H, Suzuki M (2000) Pneumomediastinum in dermatomyositis: association with cutaneous vasculopathy Ann Rheum Dis 59:372–376 Powell C, Kendall B, Wernick R, Heffner JE (2007) A 34-year-old man with amyopathic dermatomyositis and rapidly progressive dyspnea with facial swelling. Diagnosis: pneumomediastinum and subcutaneous emphysema secondary to amyopathic dermatomyositis-associated interstitial lung disease. Chest 132(5):1710–1713 Yamanishi Y, Maeda H, Konishi F, Hiyama K, Yamana S, Ishioka S et al (1999) Dermatomyositis associated with rapidly progressive fatal interstitial pneumonitis and pneumomediastinum. Scand J Rheumatol 28:58–61