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- Title
HIPERTENSIUNEA ARTERIALă PULMONARă DIN SCLEROZA SISTEMICă, O PROVOCARE PERMANENTă ÎN PRACTICA CLINICă.
- Authors
Groşeanu, Laura; Gudu, Tania; Izzi, Khalid; Beghea, Florian; Bălănescu, Andra; Predeţeanu, Denisa; Bojincă, Violeta; Săulescu, Ioana; Constantinescu, Cosmin; Opriş, Daniela; Abobului, Mihai; Borangiu, Andreea; Negru, Maria-Magdalena; Vlad, Violeta; Ionescu, Ruxandra
- Abstract
Background. Pulmonary arterial hypertension is a frequent complication of systemic sclerosis (SSc) with high morbidity and mortality. Objectives. Evaluation of clinical features and follow-up of pulmonary hipertension in agroup of csleroderma patients. Matherial. 62 scleroderma patients were evaluated during January 208 - January 2013 in Internal Medicine and Rheumatology Department of Sf. Maria Hospital, Bucharest, Romania. We performed a complete evaluation of all patients following: MEDS evaluation sheets (cutaneous, musculoarticular, gastrointestinal, cardiac, pulmonary or renal involvement; inflammatory markers, autoantibodies, complement, capilaroscopy), disease activity was evaluated with the Disease Activity Score (DAS) according to the European Scleroderma Study Group guidelines, HAQ (Health assessment questionnaires) have been also completed. A follow-up of systolic pulmonary arterial pressure was done after 3 years and 5 years. Results. 28 patients (45.16%) were diffuse forms and 34 (54.74%) limited. Medium age at scleroderma diagnosis was 56,92 (12,33) years, medium disease duration was 12.22 (3,06) years. Prevalence of PAH at first evaluation was 22.5%, at 3 years 32.25%, and at 5 years was 33.87%. Medium disease duration until PAH diagnosis was 2.75 (4.9) years. Medium initial sPAP was 31.82 (10,86) mmHg, 46.63 (11,48 )mmHg at 3 years, 55.67 (16.59) mmHg at 5 years, having the most rapid progression between years 3-5. In most patients PAH developed after 5 years of scleroderma evolution (57.15%), prevalence at 5 years was 66.66% for diffuse forms and 55.55% for limited forms. PAH is independent of sex, age, disease duration, subset, autoantibodies, visceral involvement. Differences between SSc patients with PAH and those without PAH were dyspneea class (p = 0.002), dyastolic disfunction (p = 0.001), arythmias (p = 0.003), DLCO (p = 0.001) and capillaroscopy pattern (p = 0.001). Mortality for patients with PAH was higher than for others (14.28% versus 4.58%). Medium survival duration since the diagnosis of PAH was 47.53 month. Rodnan score was a predictor for PAH appearence and also for higher mortality. Conclusions. PAH is a frequent complication of systemic sclerosis no matter of sex, age, subtype, disease duration, visceral involvement, markers of activity and imunologic features. It is also an independent predictor of mortality. Annual screening for PAH should be implemented for all patients.
- Subjects
BUCHAREST (Romania); ROMANIA; SYSTEMIC scleroderma; PULMONARY hypertension; MORTALITY; FOLLOW-up studies (Medicine); TREATMENT duration; DISEASE duration
- Publication
Romanian Journal of Rheumatology / Revista Romana de Reumatologie, 2013, Vol 22, Issue 3, p150
- ISSN
1843-0791
- Publication type
Article