title: Predicting cardiopulmonary involvement in patients with systemic sclerosis: complementary value of nailfold videocapillaroscopy patterns and disease-specific autoantibodies.
abstract: To evaluate the prevalence of anti-extractable nuclear antigen (anti-ENA) antibodies in Dutch SSc patients and the predictive power of the combination of specific anti-ENA antibodies and nailfold videocapillaroscopy (NVC) patterns to improve identification of patients with high risk for cardiopulmonary involvement.
  
  
  
  A total of 287 patients (79%) from the Leiden SSc-Cohort had data available on NVC-pattern (no SSc-specific, early, active, late) and anti-ENA antibodies. Associations between anti-ENA/NVC combinations with cardiopulmonary parameters were explored using logistic regression.
  
  
  
  Prevalence of ACA was 37%, anti-Scl-70 24%, anti-RNP 9%, anti-RNAPIII 5%, anti-fibrillarin 4%, anti-Pm/Scl 3%, anti-Th/To 0.3% and anti-Ku 1.4%. NVC showed a SSc-specific pattern in 88%: 10% early, 42% active and 36% late. The prevalence of different NVC patterns was equally distributed among specific anti-ENA antibodies, except for the absence of early pattern in anti-RNP positive patients. Fifty-one percent had interstitial lung disease (ILD), 59% had decreased diffusion capacity for carbon monoxide and 16% systolic pulmonary artery pressure >35 mmHg (sPAP↑). Regardless of ENA-subtype, NVC-pattern showed a stable association with presence of ILD or sPAP↑. For ILD, the odds ratios (ORs) were 1.3-1.4 ( P < 0.05 for analyses with anti-RNAPIII, anti-RNP). For diffusion capacity for carbon monoxide, the OR was 1.5 ( P < 0.05 for analyses with ACA, anti-Scl-70, anti-RNAPIII, anti-RNP). For sPAP↑, the ORs were 2.2-2.4 ( P < 0.05 for analyses with anti-RNAPIII, anti-RNP).
  
  
  
  In Dutch SSc patients, all SSc-specific auto-antibodies were found, with ACA and anti-Scl-70 being the most prevalent. Strikingly, the association between NVC-pattern and heart/lung involvement was independent of specific anti-ENA antibodies, which might indicate microangiopathy is an important cause of organ involvement.
authors.affiliation: Department of Rheumatology.
authors.forename: Iris M
authors.initials: IM
authors.lastname: Markusse
authors.affiliation: Department of Rheumatology.
authors.forename: Jessica
authors.initials: J
authors.lastname: Meijs
authors.affiliation: Department of Rheumatology.
authors.forename: Berber
authors.initials: B
authors.lastname: de Boer
authors.affiliation: Department of Clinical Chemistry and Laboratory Medicine.
authors.forename: Jaap A
authors.initials: JA
authors.lastname: Bakker
authors.affiliation: Department of Clinical Chemistry and Laboratory Medicine.
authors.forename: H Pascal C
authors.initials: HPC
authors.lastname: Schippers
authors.affiliation: Department of Rheumatology.
authors.forename: Anne A
authors.initials: AA
authors.lastname: Schouffoer
authors.affiliation: Department of Cardiology.
authors.forename: Nina
authors.initials: N
authors.lastname: Ajmone Marsan
authors.affiliation: Department of Radiology.
authors.forename: Lucia J M
authors.initials: LJM
authors.lastname: Kroft
authors.affiliation: Department of Pulmonology, Leiden University Medical Center, Leiden, the Netherlands.
authors.forename: Maarten K
authors.initials: MK
authors.lastname: Ninaber
authors.affiliation: Department of Rheumatology.
authors.forename: Tom W J
authors.initials: TWJ
authors.lastname: Huizinga
authors.affiliation: Department of Rheumatology.
authors.forename: Jeska K
authors.initials: JK
authors.lastname: de Vries-Bouwstra
mesh_terms: D000328:Adult; D017677:Age Distribution; D001323:Autoantibodies / Q000276:immunology*; D002318:Cardiovascular Diseases / Q000453:epidemiology* / Q000503:physiopathology; D015897:Comorbidity; D003430:Cross-Sectional Studies; D016208:Databases, Factual; D018450:Disease Progression; D005260:Female; D006801:Humans; D016015:Logistic Models; D008171:Lung Diseases / Q000453:epidemiology* / Q000503:physiopathology; D008297:Male; D020678:Microscopic Angioscopy / Q000379:methods; D008875:Middle Aged; D015999:Multivariate Analysis; D009262:Nails / Q000098:blood supply*; D009426:Netherlands; D011237:Predictive Value of Tests; D015995:Prevalence; D012189:Retrospective Studies; D018570:Risk Assessment; D012595:Scleroderma, Systemic / Q000175:diagnosis / Q000453:epidemiology* / Q000276:immunology*; D017678:Sex Distribution
chemical_list: D001323:Autoantibodies
keywords: anti-ENA; autoantibodies; cardiopulmonary involvement; nailfold videocapillaroscopy; screening; systemic sclerosis