PMID- 20144930
OWN - NLM
STAT- MEDLINE
DCOM- 20110311
LR  - 20220330
IS  - 1462-0332 (Electronic)
IS  - 1462-0324 (Linking)
VI  - 49
IP  - 5
DP  - 2010 May
TI  - Pulmonary arterial hypertension associated with systemic sclerosis in patients 
      with functional class II dyspnoea: mild symptoms but severe outcome.
PG  - 940-4
LID - 10.1093/rheumatology/kep449 [doi]
AB  - OBJECTIVE: To describe the history of SSc-associated pulmonary arterial 
      hypertension (SSc-PAH) in patients with New York Heart Association (NYHA) 
      functional class (FC) II dyspnoea at diagnosis. METHODS: Data at the time of 
      diagnosis were collected and analysed retrospectively for 77 consecutive patients 
      with SSc-PAH. RESULTS: Twelve patients (15.6%) presented with PAH and NYHA FC II 
      dyspnoea. After a mean follow-up of 44 months, only 4 out of the 12 PAH patients 
      remained stable in FC II, while 8 showed worsening to FC III or IV. Three 
      patients died during the observation period; two from PAH and one from rectal 
      cancer. Survival in patients in FC II at diagnosis was 100, 91 and 80% at 1, 2 
      and 3 years, respectively. CONCLUSIONS: A majority of patients with mildly 
      symptomatic SSc-PAH in NYHA FC II at diagnosis have a severe disease with poor 
      prognosis.
FAU - Hachulla, Eric
AU  - Hachulla E
AD  - Department of Internal Medicine, National Reference Center for Scleroderma, 
      Claude Huriez Hospital, University of Lille, France. ehachulla@chru-lille.fr
FAU - Launay, David
AU  - Launay D
FAU - Yaici, Azzedine
AU  - Yaici A
FAU - Berezne, Alice
AU  - Berezne A
FAU - de Groote, Pascal
AU  - de Groote P
FAU - Sitbon, Olivier
AU  - Sitbon O
FAU - Mouthon, Luc
AU  - Mouthon L
FAU - Guillevin, Loic
AU  - Guillevin L
FAU - Hatron, Pierre-Yves
AU  - Hatron PY
FAU - Simonneau, Gerald
AU  - Simonneau G
FAU - Clerson, Pierre
AU  - Clerson P
FAU - Humbert, Marc
AU  - Humbert M
CN  - French PAH-SSc Network
LA  - eng
PT  - Journal Article
PT  - Multicenter Study
PT  - Research Support, Non-U.S. Gov't
DEP - 20100208
PL  - England
TA  - Rheumatology (Oxford)
JT  - Rheumatology (Oxford, England)
JID - 100883501
SB  - IM
MH  - Adult
MH  - Aged
MH  - Analysis of Variance
MH  - Dyspnea/*complications/physiopathology
MH  - Female
MH  - Humans
MH  - Hypertension, Pulmonary/*complications/physiopathology
MH  - Male
MH  - Middle Aged
MH  - Prognosis
MH  - Retrospective Studies
MH  - Risk Factors
MH  - Scleroderma, Systemic/*complications/physiopathology
MH  - *Severity of Illness Index
MH  - Time Factors
FIR - Jais, Xavier
IR  - Jais X
FIR - Charlanne, Hilaire
IR  - Charlanne H
FIR - Lambert, Marc
IR  - Lambert M
FIR - Lamblin, Nicolas
IR  - Lamblin N
FIR - Maillard, Helene
IR  - Maillard H
FIR - Queyrel, Viviane
IR  - Queyrel V
FIR - Morell-Dubois, Sandrine
IR  - Morell-Dubois S
EDAT- 2010/02/11 06:00
MHDA- 2011/03/12 06:00
CRDT- 2010/02/11 06:00
PHST- 2010/02/11 06:00 [entrez]
PHST- 2010/02/11 06:00 [pubmed]
PHST- 2011/03/12 06:00 [medline]
AID - kep449 [pii]
AID - 10.1093/rheumatology/kep449 [doi]
PST - ppublish
SO  - Rheumatology (Oxford). 2010 May;49(5):940-4. doi: 10.1093/rheumatology/kep449. 
      Epub 2010 Feb 8.