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Table 1 Risk factors for Chronic Pulmonary Disease in CTDs

From: Contribution of pulmonary function tests (PFTs) to the diagnosis and follow up of connective tissue diseases

DISEASE ILD OLD PAH
RA Older age; male sex, smoking history; ACPA positivity, LDH, longstanding or persistently active disease; rheumatoid nodules, articular erosions, genetic predisposition (HLA linked) Smoking, older age  
SSDs Scl70, Th/To positivity, dSSc, smoking, older age, rapidly progressive disease.   ACA, LAC, and anti RNA polymerase I, II, III, AECA positivity, older age, RP, NVC positivity; telangiectasias, seric NT-pro-BNP and urate, RAD, RAA, TV, PE
IIMs Cutaneous manifestations; telangiectasias, RP, ATSA positivity (rapidly progressive for non-Jo1 positivity); older age, acute/subacute onset, CADM, Hamann Rich presentation correlated with worse prognosis   Cutaneous manifestations, peripheral microangiopathy, positivity for SSA/Ro, severe ILD, polyarthralgia, longstanding disease
SjS Hypergammaglobulinemia, lymphopenia, RF, SSA/Ro, SSB/La positivity Sicca syndrome, smoking RP, PE, hepatic injury, RF positivity
SLE    APLA, cardiac or vascular dysfunction, ILD, SLS
  1. ACA anticentromeric antibodies, ACPA Anti Citrullined Peptides Antibody, AECA Anti Endothelial Cell Antibody, ATSA Anti tRNA Synthetase Antibodies, CTDs Connective Tissue Diseases, dSSC diffuse Systemic Sclerosis, HLA Human Lymphocitic Antigen, IIMs Idiopathic Inflammatory Myopathies, ILD interstitial Lung Disease, LAC Lupus Anticoagulant, LDH Lactic Dehydrogenase, OLD Obstructive Lung Disease, PAH Pulmonary Artery Hypertension, PE pericardial Effusion, RA Rheumatoid Arthritis, RAA Right Atrium Area in echocardiography, RAD Right Axis deviation in electrocardiogram, RF Rheumatoid Factor, RP Raynaud Phenomenon, SLE Systemic Lupus Erythematosus, SjS Sjӧgren Syndrome, SSDs Scleroderma Spectrum Disorders, TV tricuspid velocity in echocardiography, SLD Shrinking Lung Syndrome