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Original Research

Identification and management of interstitial lung disease associated with systemic sclerosis (SSc-ILD), rheumatoid arthritis (RA-ILD), and polymyositis/dermatomyositis (PM/DM-ILD): development of expert consensus-based clinical algorithms

ORCID Icon, , ORCID Icon, ORCID Icon, & ORCID Icon
Received 27 Mar 2024, Accepted 27 Jun 2024, Published online: 15 Jul 2024

Figures & data

Figure 1. Commonalities and differences in identification and management of SSc-ILD, RA-ILD, and PM/DM-ILD. ARS: aminoacyl tRNA synthetase; CRP: C-reactive protein; CT: computed tomography; CTD: connective tissue disease; DLco: diffusing capacity of the lungs for carbon monoxide; DM: dermatomyositis; ESR: erythrocyte sedimentation rate; FVC: forced vital capacity; HRCT: high-resolution computed tomography; ILD: interstitial lung disease; ILD-GAP: interstitial lung disease gender, age, physiology index score; KL-6: Krebs von den Lungen 6; MDA5: melanoma differentiation-associated gene 5; MRC: Medical Research Council dyspnea scale; mRSS: modified Rodnan total skin thickness score; PM/DM: polymyositis/dermatomyositis; QOL: quality of life; RA: rheumatoid arthritis; SADL: smoking, age, DLco; SGRQ: St George’s Respiratory Questionnaire; SPAR: SpO2 and arthritis; SP-D: surfactant protein D; SpO2: peripheral oxygen saturation; SSc: systemic sclerosis.

Figure 1. Commonalities and differences in identification and management of SSc-ILD, RA-ILD, and PM/DM-ILD. ARS: aminoacyl tRNA synthetase; CRP: C-reactive protein; CT: computed tomography; CTD: connective tissue disease; DLco: diffusing capacity of the lungs for carbon monoxide; DM: dermatomyositis; ESR: erythrocyte sedimentation rate; FVC: forced vital capacity; HRCT: high-resolution computed tomography; ILD: interstitial lung disease; ILD-GAP: interstitial lung disease gender, age, physiology index score; KL-6: Krebs von den Lungen 6; MDA5: melanoma differentiation-associated gene 5; MRC: Medical Research Council dyspnea scale; mRSS: modified Rodnan total skin thickness score; PM/DM: polymyositis/dermatomyositis; QOL: quality of life; RA: rheumatoid arthritis; SADL: smoking, age, DLco; SGRQ: St George’s Respiratory Questionnaire; SPAR: SpO2 and arthritis; SP-D: surfactant protein D; SpO2: peripheral oxygen saturation; SSc: systemic sclerosis.

Figure 2. Clinical algorithm for identification and management of SSc-ILD.

. CRP: C-reactive protein; CT: computed tomography; CTD: connective tissue disease; DLco: diffusing capacity of the lungs for carbon monoxide; ESR: erythrocyte sedimentation rate; FVC: forced vital capacity; HRCT: high-resolution computed tomography; ILD: interstitial lung disease; ILD-GAP: interstitial lung disease gender, age, physiology index score; KL-6: Krebs von den Lungen 6; MDA5: melanoma differentiation-associated gene 5; MRC: Medical Research Council dyspnea scale; mRSS: modified Rodnan total skin thickness score; QOL: quality of life; SADL: smoking, age, DLco; SGRQ: St George’s Respiratory Questionnaire; SPAR: SpO2 and arthritis; SP-D: surfactant protein D; SpO2: peripheral oxygen saturation; SSc: systemic sclerosis.

Figure 2. Clinical algorithm for identification and management of SSc-ILD. Display full size. CRP: C-reactive protein; CT: computed tomography; CTD: connective tissue disease; DLco: diffusing capacity of the lungs for carbon monoxide; ESR: erythrocyte sedimentation rate; FVC: forced vital capacity; HRCT: high-resolution computed tomography; ILD: interstitial lung disease; ILD-GAP: interstitial lung disease gender, age, physiology index score; KL-6: Krebs von den Lungen 6; MDA5: melanoma differentiation-associated gene 5; MRC: Medical Research Council dyspnea scale; mRSS: modified Rodnan total skin thickness score; QOL: quality of life; SADL: smoking, age, DLco; SGRQ: St George’s Respiratory Questionnaire; SPAR: SpO2 and arthritis; SP-D: surfactant protein D; SpO2: peripheral oxygen saturation; SSc: systemic sclerosis.

Figure 3. Clinical algorithm for identification and management of RA-ILD.

. CRP: C-reactive protein; CT: computed tomography; CTD: connective tissue disease; DLco: diffusing capacity of the lungs for carbon monoxide; ESR: erythrocyte sedimentation rate; FVC: forced vital capacity; HRCT: high-resolution computed tomography; ILD: interstitial lung disease; ILD-GAP: interstitial lung disease gender, age, physiology index score; KL-6: Krebs von den Lungen 6; MDA5: melanoma differentiation-associated gene 5; MRC: Medical Research Council dyspnea scale; mRSS: modified Rodnan total skin thickness score; QOL: quality of life; RA: rheumatoid arthritis; SGRQ: St George’s Respiratory Questionnaire; SP-D: surfactant protein D; SpO2: peripheral oxygen saturation.

Figure 3. Clinical algorithm for identification and management of RA-ILD. Display full size. CRP: C-reactive protein; CT: computed tomography; CTD: connective tissue disease; DLco: diffusing capacity of the lungs for carbon monoxide; ESR: erythrocyte sedimentation rate; FVC: forced vital capacity; HRCT: high-resolution computed tomography; ILD: interstitial lung disease; ILD-GAP: interstitial lung disease gender, age, physiology index score; KL-6: Krebs von den Lungen 6; MDA5: melanoma differentiation-associated gene 5; MRC: Medical Research Council dyspnea scale; mRSS: modified Rodnan total skin thickness score; QOL: quality of life; RA: rheumatoid arthritis; SGRQ: St George’s Respiratory Questionnaire; SP-D: surfactant protein D; SpO2: peripheral oxygen saturation.

Figure 4. Clinical algorithm for identification and management of PM/DM-ILD.

. ARS: aminoacyl tRNA synthetase; CRP: C-reactive protein; CT: computed tomography; CTD: connective tissue disease; DLco: diffusing capacity of the lungs for carbon monoxide; DM: dermatomyositis; ESR: erythrocyte sedimentation rate; FVC: forced vital capacity; HRCT: high-resolution computed tomography; ILD: interstitial lung disease; ILD-GAP: interstitial lung disease gender, age, physiology index score; KL-6: Krebs von den Lungen 6; MDA5: melanoma differentiation-associated gene 5; MRC: Medical Research Council dyspnea scale; mRSS: modified Rodnan total skin thickness score; PM/DM: polymyositis/dermatomyositis; QOL: quality of life; SGRQ: St George’s Respiratory Questionnaire; SP-D: surfactant protein D; SpO2: peripheral oxygen saturation.

Figure 4. Clinical algorithm for identification and management of PM/DM-ILD. Display full size. ARS: aminoacyl tRNA synthetase; CRP: C-reactive protein; CT: computed tomography; CTD: connective tissue disease; DLco: diffusing capacity of the lungs for carbon monoxide; DM: dermatomyositis; ESR: erythrocyte sedimentation rate; FVC: forced vital capacity; HRCT: high-resolution computed tomography; ILD: interstitial lung disease; ILD-GAP: interstitial lung disease gender, age, physiology index score; KL-6: Krebs von den Lungen 6; MDA5: melanoma differentiation-associated gene 5; MRC: Medical Research Council dyspnea scale; mRSS: modified Rodnan total skin thickness score; PM/DM: polymyositis/dermatomyositis; QOL: quality of life; SGRQ: St George’s Respiratory Questionnaire; SP-D: surfactant protein D; SpO2: peripheral oxygen saturation.
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Data availability statement

The data sets used and/or analyzed during the current study are available from the corresponding author on reasonable request.