title: Severe Pulmonary Hypertension in Primary Sjögren's Syndrome.
abstract: A 65 year-old female with a history of xerostomia and xerophthalmia was presented with dyspnea on exertion (New York Heart Association class III). Echocardiography and cardiac catheterization demonstrated severe pulmonary hypertension (PH). Laboratory examinations showed positive anti-nuclear and anti-Ro/SS-A antibodies. Schirmer's test was positive and salivary gland scintigraphy revealed severely decreased tracer uptakes in both parotid and submandibular glands. By excluding other possible causes of PH during further examinations, she was diagnosed with severe PH associated with primary Sjögren's syndrome. Her dyspnea symptom was much improved with endothelin receptor antagonist and azathioprine.
authors: Division of Cardiology, Department of Internal Medicine, Inje University College of Medicine, Cardiovascular Research Institute, Busan Paik Hospital, Busan, Korea.
authors: Ji-An
authors: JA
authors: Hwang
authors: Tae-Hyun
authors: TH
authors: Yang
authors: Ji-Young
authors: JY
authors: Lee
authors: Dong-Wan
authors: DW
authors: Koo
authors: In Suk
authors: IS
authors: Choi
authors: Sun-Young
authors: SY
authors: Cho
authors: Min-Sung
authors: MS
authors: Kim
keywords: Hypertension, pulmonary; Sjögren's syndrome
references: Chest. 2006 Jul;130(1):182-9
references: 16840400
references: Am Rev Respir Dis. 1981 Oct;124(4):499-503
references: 7294510
references: Thorax. 1999 Mar;54(3):273-7
references: 10325906
references: Hum Pathol. 1993 Sep;24(9):1035-6
references: 8110261
references: Arch Intern Med. 2004 Jun 28;164(12):1275-84
references: 15226160
references: Circulation. 1984 Oct;70(4):580-7
references: 6148159
references: Semin Arthritis Rheum. 1984 May;13(4):349-59
references: 6374900
references: Intern Med. 2003 Dec;42(12):1248-52
references: 14714969
references: Rev Med Interne. 2002 Jan;23(1):41-54
references: 11859694
references: Am J Med. 1983 Jul;75(1):65-74
references: 6859087
references: Ann Rheum Dis. 2005 Aug;64(8):1236-7
references: 16014687
references: Medicine (Baltimore). 2007 Sep;86(5):299-315
references: 17873760
references: Rheumatology (Oxford). 2006 Oct;45 Suppl 4:iv22-5
references: 16980719
references: Ann Intern Med. 1977 Apr;86(4):394-9
references: 848800
references: Hum Pathol. 1993 Feb;24(2):199-205
references: 8432515
references: Clin Chest Med. 2010 Sep;31(3):489-500
references: 20692541
references: Ann Rheum Dis. 2002 Jun;61(6):554-8
references: 12006334
references: Lupus. 2004;13(2):105-12
references: 14995003