title: Systemic sclerosis patients with and without pulmonary arterial hypertension: a nailfold capillaroscopy study.
abstract: OBJECTIVE
  Pulmonary arterial hypertension (PAH) is a complication of SSc due to increased vascular resistance, and abnormal vascularity is a well-known feature of the disease as shown by nailfold videocapillaroscopy (NVC). This study investigated for specific NVC changes in SSc patients with and without PAH to assess any useful difference.
  
  
  METHODS
  Twenty-four SSc patients, 12 with PAH and 12 without, entered the study. Evidence of PAH was defined as increased systolic pulmonary artery pressure (PAP) (≥35 mmHg), indirectly assessed by echocardiography and confirmed by right heart catheterization (mPAP > 25 mmHg). NVC was performed, and a semi-quantitative rating scale, a rating system for avascular areas and a specific NVC pattern evaluation, namely early, active and late, were used.
  
  
  RESULTS
  An NVC score >1 was more frequently found in patients with PAH than those without, 11 cases (92%) vs 5 cases (42%) (P = 0.03); an avascular areas grade >1 was present in 10 (83%) and 2 (17%) cases, respectively (P = 0.003); and a more severe NC pattern (active/late) was described in 11 (92%) and 5 (42%) patients, respectively (P = 0.03). When we compared the mPAP with NVC parameters, we found significant correlations between mPAP values and the NVC score (P < 0.005) and with the avascular areas score (P < 0.001).
  
  
  CONCLUSIONS
  Our results underline the relevance of early microvascular assessment in patients at risk of developing a severe complication such as PAH that can amplify the systemic microvascular impairment in SSc. More severe NVC abnormalities should lead to strict cardiopulmonary surveillance and a complete NVC study is indicated.
authors.affiliation: Department of Internal Medicine and Medical Specialities, Sapienza University of Rome, Policlinico Umberto I, Viale del Policlinico 155, 00161 Rome, Italy. valeria.riccieri@uniroma1.it
authors.forename: Valeria
authors.initials: V
authors.lastname: Riccieri
authors.forename: Massimiliano
authors.initials: M
authors.lastname: Vasile
authors.forename: Nicoletta
authors.initials: N
authors.lastname: Iannace
authors.forename: Katia
authors.initials: K
authors.lastname: Stefanantoni
authors.forename: Iliana
authors.initials: I
authors.lastname: Sciarra
authors.forename: Carmine D
authors.initials: CD
authors.lastname: Vizza
authors.forename: Roberto
authors.initials: R
authors.lastname: Badagliacca
authors.forename: Roberto
authors.initials: R
authors.lastname: Poscia
authors.forename: Silvia
authors.initials: S
authors.lastname: Papa
authors.forename: Mario
authors.initials: M
authors.lastname: Mezzapesa
authors.forename: Martina
authors.initials: M
authors.lastname: Nocioni
authors.forename: Guido
authors.initials: G
authors.lastname: Valesini
mesh_terms: D000368:Aged; D002196:Capillaries / Q000503:physiopathology; D002200:Capillary Resistance / Q000502:physiology*; D016022:Case-Control Studies; D018450:Disease Progression; D065627:Familial Primary Pulmonary Hypertension; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis* / Q000453:epidemiology; D008833:Microcirculation / Q000502:physiology; D020678:Microscopic Angioscopy / Q000379:methods; D008875:Middle Aged; D009262:Nails / Q000098:blood supply* / Q000503:physiopathology; D012016:Reference Values; D018570:Risk Assessment; D012595:Scleroderma, Systemic / Q000175:diagnosis* / Q000453:epidemiology; D012720:Severity of Illness Index; D018709:Statistics, Nonparametric
keywords: nailfold videocapillaroscopy; pulmonary arterial hypertension; systemic sclerosis