Optimal management of interstitial lung disease associated with dermatomyositis/polymyositis: lessons from the Japanese experience

Figures & data

Figure 1 Cyclosporine therapy improved prognosis of glucocorticoid-resistant ILD associated with DM/PM.

Notes: Data from Chiba University Hospital (1990–2004). Data include cases in studies by Nawata et al¹⁴ and Kurasawa et al.⁶⁸ Patients were initially treated with high-dose GC. If respiratory symptoms, arterial oxygen tension levels, or abnormalities on high-resolution computed tomography scan images worsened within 2 weeks after starting GC therapy or were not improved within 4 weeks, ILD was judged as GC-resistant ILD and additional CsA was administrated.
Abbreviations: CsA, cyclosporine; DM, dermatomyositis; GC, glucocorticoid; ILD, interstitial lung disease; PM, polymyositis.

Figure 2 Prognostic factors (short-term) in ILD associated with DM/PM.

Note: The figure shows short-term prognostic factors, but not long-term ones.
Abbreviations: a-ARS Ab, anti-aminoacyl-tRNA-synthetases antibody; a-MDA5 Ab, anti-melanoma differentiation-associated gene 5 antibody; ADM, amyopathic dermatomyositis; CK, creatine kinase; DAD, diffuse alveolar damage; DM, dermatomyositis; GGO, ground-glass opacities; ILD, interstitial lung disease; NSIP, nonspecific interstitial pneumonia; OP, organizing pneumonia; PM, polymyositis; UIP, usual interstitial pneumonia.

Table 1 Subtypes of ILD associated with DM/PM

Onset/progression	Type of myositis	Histopathology	Radiographic findings	Other findings	ILD prognosis	Therapy
Acute/subacute
1. a-MDA5 (+)	ADM/DM	DAD NSIP?	GGO + lower/random consolidation	Ferritin elevation Fever Skin ulcer Palmar papules	Poor	GC + CsA + CY (IVIG, PMX-DHP)
2. a−MDA5 (−)	DM (ADM)	NSIP (DAD)	GGO Reticular (consolidation)		Fair (GC-resistance*)	GC + CsA (TAC)/CY
3. a−MDA5 (−)	PM	NSIP	GGO Reticular		Good	GC (CsA/CY/ TAC/MMF)
Chronic
1. a−ARS (+)	PM (DM)	NSIP	Reticular (GGO)	Raynaud’s phenomenon Arthritis Mechanics’ hand	Good (Frequent recurrence)	GC (CsA/CY/ TAC/AZA/MMF)
2. a−ARS (−)	DM/PM	NSIP	Reticular (GGO)	Some patients positive for a-MDA5 Ab	Good	GC (CsA/CY/ TAC/AZA/MMF)

Notes: *GC-resistance; GC-resistance is judged when respiratory symptoms, arterial oxygen tension levels, or abnormalities on CT scan images worsened within 2 weeks after starting GC therapy or were not improved within 4 weeks. (+) means positive for the antibody. (−) means negative for the antibody. ? denotes the controversial and questionable existence of NSIP.

Abbreviations: a-ARS, anti-aminoacyl-tRNA-synthetases; a-MDA5, anti-melanoma differentiation-associated gene 5; Ab, antibody; ADM, amyopathic dermatomyositis; AZA, azathioprine; CsA, cyclosporine; CT, computed tomography; CY, cyclophosphamide; DAD, diffuse alveolar damage; DM, dermatomyositis; GC, glucocorticoid; GGO, ground-glass opacities; ILD, interstitial lung disease; IVIG, intravenous immunoglobulin; MMF, mycophenolate mofetil; NSIP, nonspecific interstitial pneumonia; PM, polymyositis; PMX-DHP, polymyxin-B direct hemoperfusion; TAC, tacrolimus.

Figure 3 Algorithm for management of DM/PM-ILD.

Abbreviations: a-MDA5 Ab, anti-melanoma differentiation-associated gene 5 antibody; ADM, amyopathic dermatomyositis; CsA, cyclosporine; DAD, diffuse alveolar damage; DM, dermatomyositis; GC, glucocorticoid; GGO, ground-glass opacities; HRCT, high-resolution computed tomography; ILD, interstitial lung disease; IVCY, intravenous-pulse cyclophosphamide; IVIG, intravenous immunoglobulin; PaO₂, arterial oxygen tension; PM, polymyositis; PMX-DHP, polymyxin-B direct hemoperfusion; VC, vital capacity.

Nawata Y, Kurasawa K, Takabayashi K, et al. Corticosteroid resistant interstitial pneumonitis in dermatomyositis/polymyositis: prediction and treatment with cyclosporine. J Rheumatol. 1999;26(7):1527–1533.

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Kurasawa K, Nawata Y, Takabayashi K, et al. Activation of pulmonary T cells in corticosteroid-resistant and -sensitive interstitial pneumonitis in dermatomyositis/polymyositis. Clin Exp Immunol. 2002;129(3):541–548.

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