Abstract
Amyopathic dermatomyositis (ADM) is occasionally complicated by rapidly progressive interstitial pneumonia (RPIP), and in such cases, diffuse alveolar damage (DAD) is usually diagnosed at autopsy. Here, we present three patients with RPIP accompanied by ADM in whom lung disease was assessed at an early stage. High-resolution computed tomography (HRCT) carried out before the onset of dyspnoea revealed uniformly subpleural reticular opacity with faint ground-glass attenuation. At that stage, surgical lung biopsies from two patients showed histological patterns typical of cellular nonspecific interstitial pneumonia (NSIP). Despite pulse methylprednisolone and subsequent high-dose oral administration of prednisolone, lung disease progressed in all patients, with extensive areas of ground-glass opacity and consolidation observed in HRCT scans. DAD was confirmed histologically in one case. Additional administration of cyclosporine, pulse cyclophosphamide or high-dose intravenous administration of immunoglobulin rescued all patients. Our data suggest that ADM-associated interstitial pneumonia takes an aggressive course even when the radiological and histological features are consistent with NSIP. Aggressive combination therapy with high-dose steroids and immunosuppressive agents is required as early as possible for patients with this life-threatening disorder.
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References
Krain LS (1975) Dermatomyositis in six patients without initial muscle involvement. Arch Dermatol 111:241–245
Fudman EJ, Schnitzler TJ (1986) Dermatomyositis without creatine kinase elevation: a poor prognostic sign. Am J Med 80:329–332
Santiago MB, Chalhoub M, Pereira ST (1998) Amyopathic dermatomyositis complicated by interstitial pulmonary disease and pneumomediastinum. J Rheumatol 25:2042–2043
Sontheimer RD, Miyagawa S (2003) Potentially fatal interstitial lung disease can occur in clinically amyopathic dermatomyositis. J Am Acad Dermatol 48:797–798
Lee CS, Chen TL, Tzen CY, Lin FJ, Peng MJ, Wu CL, Chen PJ (2002) Idiopathic inflammatory myopathy with diffuse alveolar damage. Clin Rheumatol 21:391–396
Katzenstein A, Fiorelli RF (1994) Nonspecific interstitial pneumonia/fibrosis: histologic features and clinical significance. Am J Surg Pathol 18:136–147
Primack SL, Hartman TE, Ikezoe J, Akira M, Sakatani M, Muller NL (1993) Acute interstitial pneumonia: radiographic and CT findings in nine patients. Radiology 188:817–820
Johkoh T, Muller NL, Taniguchi H, Kondoh Y, Akira M, Ichikado K, Ando M, Honda O, Tomiyama N, Nakamura H (1999) Acute interstitial pneumonia: thin-section CT findings in 36 patients. Radiology 211:859–863
Akira M, Hara H, Sakatani M (1999) Interstitial lung disease in association with polymyositis–dermatomyositis: long-term follow-up CT evaluation in seven patients. Radiology 210:333–338
Ikezoe J, Johkoh T, Kohno N, Takeuchi N, Ichikado K, Nakamura H (1996) High-resolution CT findings of lung disease in patients with polymyositis and dermatomyositis. J Thorac Imaging 11:250–259
Arakawa H, Yamada H, Kurihara Y, Nakajima Y, Takada A, Fukushima Y, Fujioka M (2003) Nonspecific interstitial pneumonia associated with polymyositis and dermatomyositis. Chest 123:1096–1103
Tazelaar HD, Viggiano RW, Pickersgill J, Colby TV (1990) Interstitial lung disease in polymyositis and dermatomyositis. Clinical features and prognosis as correlated with histologic findings. Am Rev Respir Dis 141:727–733
Douglas WW, Tazelaar HD, Hartman TE, Hartman RP, Decker PA, Schroeder DR, Ryu JH (2001) Polymyositis–dermatomyositis-associated interstitial lung disease. Am J Respir Crit Care Med 164:1182–1185
Nagai S, Kitaichi M, Itoh H, Nishimura K, Izumi T, Colby TV (1998) Idiopathic nonspecific interstitial pneumonia/fibrosis: comparison with idiopathic pulmonary fibrosis and BOOP. Eur Respir J 12:1010–1019
Travis WD, Matsui K, Moss J, Ferrans VJ (2000) Idiopathic nonspecific interstitial pneumonia: prognostic significance of cellular and fibrosing patterns: survival comparison with usual interstitial pneumonia and desquamative interstitial pneumonia. Am J Surg Pathol 24:19–33
Kurasawa K, Nawata Y, Takabayashi K, Kumano K, Kita Y, Takiguchi Y, Kuriyama T, Sueishi M, Saito Y, Iwamoto I (2002) Activation of pulmonary T cells in corticosteroid-resistant and -sensitive interstitial pneumonitis in dermatomyositis/polymyositis. Clin Exp Immunol 129:541–548
Cottin V, Thivolet-Bejui F, Reynaud-Gaubert M, Cadranel J, Delaval P, Ternamian PJ, Cordier JF, Groupe d'Etudes et de Recherche sur les Maladies “Orphelines” Pulmonaires (2003) Interstitial lung disease in amyopathic dermatomyositis, dermatomyositis and polymyositis. Eur Respir J 22:245–250
Miyake S, Ohtani Y, Sawada M, Inase N, Miyazaki Y, Takano S, Miyasaka N, Yoshizawa Y (2002) Usefulness of cyclosporine A on rapidly progressive interstitial pneumonia in dermatomyositis. Sarcoidosis Vasc Diffuse Lung Dis 19:128–133
al-Janadi M, Smith CD, Karsh J (1989) Cyclophosphamide treatment of interstitial pulmonary fibrosis in polymyositis/dermatomyositis. J Rheumatol 16:1592–1596
Nagatomo Y, Okayama A, Murai K, Sasaki T, Kuroki M, Tanaka G, Iga M, Kai Y, Tsubouchi H (2000) Successful treatment using high-dose intravenous immunoglobulin in a patient with rapidly progressive interstitial pneumonia associated with dermatomyositis. Mod Rheumatol 10:165–168
Cherin P, Herson S, Wechsler B, Piette JC, Bletry O, Coutellier A, Ziza JM, Godeau P (1991) Efficacy of intravenous gamma-globulin therapy in chronic refractory polymyositis and dermatomyositis. Am J Med 91:162–168
Acknowledgements
The authors are grateful to Dr. Henry D. Tazelaar and Dr. Jeff Myers, Mayo Clinic, and Dr. Masanori Kitaichi, Kyoto University, for their comments on histopathology of the lung in three cases.
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Miyazaki, E., Ando, M., Muramatsu, T. et al. Early assessment of rapidly progressive interstitial pneumonia associated with amyopathic dermatomyositis. Clin Rheumatol 26, 436–439 (2007). https://doi.org/10.1007/s10067-005-0147-4
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DOI: https://doi.org/10.1007/s10067-005-0147-4