PMID- 17873760
OWN - NLM
STAT- MEDLINE
DCOM- 20080321
LR  - 20210108
IS  - 0025-7974 (Print)
IS  - 0025-7974 (Linking)
VI  - 86
IP  - 5
DP  - 2007 Sep
TI  - Pulmonary arterial hypertension: a rare complication of primary Sjogren syndrome: 
      report of 9 new cases and review of the literature.
PG  - 299-315
LID - 10.1097/MD.0b013e3181579781 [doi]
AB  - Primary Sjogren syndrome (pSS) is a fairly common autoimmune disease with 
      glandular and extraglandular manifestations. Pulmonary involvement mainly 
      corresponds to small airways and interstitial lung disease. Pulmonary arterial 
      hypertension (PAH) is rare: to our knowledge, only 32 cases have been reported in 
      pSS patients to date. PAH is a disease of the small pulmonary arteries 
      characterized by vascular proliferation and remodeling, resulting in a 
      progressive increase in pulmonary vascular resistance, and, ultimately, right 
      ventricular failure and death. We report 9 new cases of pSS-associated PAH with a 
      complete assessment including clinical characteristics (of both PAH and pSS), 
      hemodynamic parameters, medical management, and outcome. We also review the 19 
      fully documented PAH patients with pSS reported in the English-language 
      literature, therefore analyzing a total of 28 cases (27 women; mean age at PAH 
      diagnosis, 50 +/- 11 yr; range, 23-68 yr). Functional impairment at diagnosis was 
      severe, with a New York Heart Association (NYHA) functional class of III or IV in 
      most cases. Seven of 15 (47%) patients for whom data were available had history 
      or evidence of right heart failure at PAH diagnosis. Hemodynamic parameters were 
      moderate to severe with a mean pulmonary artery pressure of 44 +/- 11 mm Hg 
      (range, 24-60 mm Hg) and a cardiac index of 2.91 +/- 0.72 Lmin(-1)m(-2) (range, 
      1.36-3.88 Lmin(-1)m(-2)). Standard PAH therapy (endothelin receptor antagonists, 
      phosphodiesterase type 5 inhibitors, or prostanoids) was initially effective in 
      some patients but had short-term and long-term failures. Some patients were 
      treated with first-line immunosuppressants alone leading to improvement in some, 
      but second-line standard PAH therapy was added in all cases thereafter. The best 
      treatment strategy remains to be defined. Estimated survival rates were low (73% 
      and 66% at 1 and 3 years, respectively). Compared with pSS patients without PAH, 
      patients with pSS-associated PAH had Raynaud phenomenon, cutaneous vasculitis, 
      and interstitial lung disease significantly more frequently. They also more 
      frequently had antinuclear, anti-Ro/SSA, and anti-RNP autoantibodies, as well as 
      positive rheumatoid factor and hypergammaglobulinemia. These data suggest that 
      systemic vasculopathy, B-cell activation, and autoimmunity could play a role in 
      the pathophysiology of pSS-associated PAH. In conclusion, this report underlines 
      the rarity and severity of PAH in pSS patients. The best therapeutic regimen 
      remains to be defined but should include standard PAH therapy and/or 
      immunosuppressants.
FAU - Launay, David
AU  - Launay D
AD  - From Centre National de Reference de l'Hypertension Arterielle Pulmonaire, UPRES 
      EA2705, Service de Pneumologie et Reanimation Respiratoire, Hopital 
      Antoine-Beclere, Assistance Publique Hopitaux de Paris, Universite Paris-Sud 11, 
      Clamart (DL, XJ, GS, MH) and Service de Medecine Interne, Centre National de 
      Reference de la Sclerodermie, Hopital Claude-Huriez, CHRU Lille, Universite Lille 
      2, Lille (DL, EH, PYH), France.
FAU - Hachulla, Eric
AU  - Hachulla E
FAU - Hatron, Pierre-Yves
AU  - Hatron PY
FAU - Jais, Xavier
AU  - Jais X
FAU - Simonneau, Gerald
AU  - Simonneau G
FAU - Humbert, Marc
AU  - Humbert M
LA  - eng
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
PT  - Review
PL  - United States
TA  - Medicine (Baltimore)
JT  - Medicine
JID - 2985248R
RN  - 0 (Antibodies, Antinuclear)
RN  - 0 (Endothelin Receptor Antagonists)
RN  - 0 (Enzyme Inhibitors)
RN  - 0 (Immunosuppressive Agents)
RN  - 0 (Phosphodiesterase 5 Inhibitors)
RN  - 0 (Prostaglandins)
SB  - IM
MH  - Adult
MH  - Aged
MH  - Antibodies, Antinuclear/immunology
MH  - Case-Control Studies
MH  - Diagnosis, Differential
MH  - Endothelin Receptor Antagonists
MH  - Enzyme Inhibitors/therapeutic use
MH  - Female
MH  - Follow-Up Studies
MH  - France
MH  - Heart Failure/etiology
MH  - Humans
MH  - Hypertension, Pulmonary/*diagnosis/drug therapy/*etiology/physiopathology
MH  - Immunosuppressive Agents/therapeutic use
MH  - Male
MH  - Middle Aged
MH  - Phosphodiesterase 5 Inhibitors
MH  - Prostaglandins/therapeutic use
MH  - Pulmonary Wedge Pressure
MH  - Retrospective Studies
MH  - Sjogren's Syndrome/*complications/immunology/pathology
MH  - Survival Analysis
MH  - Treatment Outcome
RF  - 80
EDAT- 2007/09/18 09:00
MHDA- 2008/03/22 09:00
CRDT- 2007/09/18 09:00
PHST- 2007/09/18 09:00 [pubmed]
PHST- 2008/03/22 09:00 [medline]
PHST- 2007/09/18 09:00 [entrez]
AID - 00005792-200709000-00006 [pii]
AID - 10.1097/MD.0b013e3181579781 [doi]
PST - ppublish
SO  - Medicine (Baltimore). 2007 Sep;86(5):299-315. doi: 10.1097/MD.0b013e3181579781.