The utility of comprehensive autoantibody testing to differentiate connective tissue disease associated and idiopathic interstitial lung disease subgroup cases.
Interstitial lung disease (ILD) comprises many heterogeneous disease groups, the largest being CTD-associated and those labelled as idiopathic out of necessity. The mechanisms causing ILD are poorly understood, but most CTD- and idiopathic-ILD cases can respond to immunosuppression, clearly suggesting a pathological role for inflammation. By contrast, corticosteroid immunosuppression causes harm without benefit in the feared idiopathic pulmonary fibrosis, suggesting that inflammation plays little pathological role, and where ILD progresses rapidly to lethal outcome even with anti-fibrotic drug use. Given the treatment response differences apparent between ILD subgroups, and the dangers and costs of corticosteroid and anti-fibrotic drug use, respectively, it has become vital in every ILD patient to make an accurate subgroup diagnosis, to optimize treatment selections. This review discusses why differentiating CTD- and idiopathic-ILD subgroup cases remains so problematic, and why existing comprehensive CTD-specific serology would, if generally available, represent an ideal biomarker tool to enhance ILD subgroup diagnostic accuracy.
Department of Musculoskeletal Biology II, MRC/ARUK Institute of Ageing and Chronic Disease, University of Liverpool.|University Department of Rheumatology.
Caroline V
CV
Cotton
Department of Respiratory Medicine, Aintree Chest Centre, Aintree University Hospital, Liverpool, UK.
Lisa G
LG
Spencer
Department of Musculoskeletal Biology II, MRC/ARUK Institute of Ageing and Chronic Disease, University of Liverpool.
Robert P
RP
New
Department of Musculoskeletal Biology II, MRC/ARUK Institute of Ageing and Chronic Disease, University of Liverpool.|University Department of Rheumatology.
Robert G
RG
Cooper
D000305:Adrenal Cortex Hormones / Q000009:adverse effects; D000933:Antifibrinolytic Agents / Q000009:adverse effects; D001323:Autoantibodies / Q000097:blood*; D015415:Biomarkers / Q000097:blood; D003240:Connective Tissue Diseases / Q000175:diagnosis* / Q000276:immunology; D003937:Diagnosis, Differential; D003951:Diagnostic Errors / Q000009:adverse effects*; D006801:Humans; D007165:Immunosuppression Therapy / Q000009:adverse effects / Q000379:methods; D017563:Lung Diseases, Interstitial / Q000175:diagnosis* / Q000188:drug therapy / Q000276:immunology
D000305:Adrenal Cortex Hormones; D000933:Antifibrinolytic Agents; D001323:Autoantibodies; D015415:Biomarkers
anti-synthetase syndrome; antibodies; biomarkers; connective tissue disease; interstitial lung disease; myositis; serology