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Review
. 2025 Jan 24;15(3):275.
doi: 10.3390/diagnostics15030275.

Recognition of Idiopathic Inflammatory Myopathies Underlying Interstitial Lung Diseases

Giulia Morina  1 , Domenico Sambataro  2 , Alessandro Libra  1 , Stefano Palmucci  3 , Michele Colaci  4 , Gaetano La Rocca  5 , Francesco Ferro  5 , Linda Carli  5 , Chiara Baldini  5 , Santa Valentina Liuzzo  1 , Carlo Vancheri  1 , Gianluca Sambataro  6
Affiliations
Review

Recognition of Idiopathic Inflammatory Myopathies Underlying Interstitial Lung Diseases

Giulia Morina et al. Diagnostics (Basel). .

Abstract

Interstitial Lung Disease (ILD) is one of the most common causes of mortality in idiopathic Inflammatory Myopathies (IIM). Despite these conditions being commonly associated with proximal weakness, skin rashes and arthritis, ILD can be the first or the sole clinical feature in up to 60% of patients, potentially leading to incorrect diagnosis. The early recognition of an underlying IIM in ILD patients can allow for prompt treatment, which could potentially stabilize or even improve the lung disease, also avoiding the development of other clinical features associated with the condition. The objective of this review is to describe the clinical, serological and radiological features associated with IIM-ILD, mainly focusing on dermatomyositis and antisynthetase syndrome.

Keywords: antisynthetase syndrome; autoantibodies; dermatomyositis; idiopathic inflammatory myopathies; interstitial lung disease; polymyositis.

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Conflict of interest statement

G.S. reports personal fees from Boehringer Ingelheim and Gentili outside the submitted work; S.P. reports personal fees from Boehringer Ingelheim, Delphi International srl, and F. Hoffmann-La Roche Ltd. outside the submitted work; C.V. is on the F. Hoffmann-La Roche Ltd. and Boehringer Ingelheim Scientific boards. He received consulting fees and/or speaker fees from AstraZeneca, Boehringer Ingelheim, C.F. Hoffmann-La Roche Ltd., and Menarini. G.M., D.S., S.V.L., A.L., G.L.R., F.F., C.B., M.C. declare no conflicts of interest.

Figures

Figure 1
Figure 1
Shawl Sign present in the last year in a 68-year-old woman with DM.
Figure 2
Figure 2
(A): Gottron Papules that first appeared a week before in a 43-year-old woman with DM; (B): Heliotropic Rash present in the last 6 months in a 64-year-old woman with DM; (C): Hiker’s Feet, undated, in a 53-year-old woman with ASyS.
Figure 3
Figure 3
NSIP + OP pattern in a 57-year-old male at the time of diagnosis of DM.
Figure 4
Figure 4
ANA: Antinuclear Antibody; ASA: Antisynthetase Antibodies; ASyS: Antisynthetase Syndrome; CADM: Clinically Amyopathic DM; CPK: Creatine Phosphokinase; DM: Dermatomyositis; DMA: DM Antibodies; ENA: Extractable Nuclear Antigen; IIM: Idiopathic Inflammatory Myopathy; IPAF: Interstitial Pneumonia with Autoimmune Features; HRCT: High-Resolution Computed Tomography; ILD: Interstitial Lung Disease; Ind: Indeterminate; MAA: Myositis Associated Antibodies; MSA: Myositis Associated Antibodies; NSIP: Nonspecific Interstitial Pneumonia; OM: Overlap Myositis; OP: Organizing Pneumonia; RP: Raynaud’s Phenomenon; UIP: Usual Interstitial Pneumonia. +: present; −: absent; ±: possible.
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