Korean Circ J.  2013 Jul;43(7):504-507. 10.4070/kcj.2013.43.7.504.

Severe Pulmonary Hypertension in Primary Sjogren's Syndrome

• Hwang JA
• Yang TH
• Lee JY
• Koo DW
• Choi IS
• Cho SY
• Kim MS

• KMID: 2145509
• DOI: http://doi.org/10.4070/kcj.2013.43.7.504

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 Sjogren's syndrome. Her dyspnea symptom was much improved with endothelin receptor antagonist and azathioprine.

Keyword

Hypertension, pulmonary; Sjogren's syndrome

MeSH Terms

Antibodies
Azathioprine
Cardiac Catheterization
Cardiac Catheters
Dyspnea
Echocardiography
Female
Heart
Humans
Hydrogen-Ion Concentration
Hypertension, Pulmonary
Receptors, Endothelin
Salivary Glands
Sjogren's Syndrome
Submandibular Gland
Xerophthalmia
Xerostomia
Antibodies
Azathioprine
Receptors, Endothelin

Figure

• 

  Fig. 1 Initial transthoracic echocardiography demonstrates a dilated right ventricle (RV) and a slightly compressed left ventricle (LV) with a flattened ventricular septum (A and B). After 6 months of treatment, a follow-up exam shows that the RV cavity was decreased in size and the ventricular septum is deviated to the RV normally (C and D). RA: right atrium, LA: left atrium.

• 

  Fig. 2 Pulmonary artery pressure tracing right heart catheterization shows severe pulmonary arterial hypertension.

Reference

1. Kokosi M, Riemer EC, Highland KB. Pulmonary involvement in Sjögren syndrome. Clin Chest Med. 2010; 31:489–500.

2. Ungerer RG, Tashkin DP, Furst D, et al. Prevalence and clinical correlates of pulmonary arterial hypertension in progressive systemic sclerosis. Am J Med. 1983; 75:65–74.

3. Salerni R, Rodnan GP, Leon DF, Shaver JA. Pulmonary hypertension in the CREST syndrome variant of progressive systemic sclerosis (scleroderma). Ann Intern Med. 1977; 86:394–399.

4. Sanchez O, Humbert M, Sitbon O, Nunes H, Garcia G, Simonneau G. [Pulmonary hypertension associated with connective tissue diseases]. Rev Med Interne. 2002; 23:41–54.

5. Wiener-Kronish JP, Solinger AM, Warnock ML, Churg A, Ordonez N, Golden JA. Severe pulmonary involvement in mixed connective tissue disease. Am Rev Respir Dis. 1981; 124:499–503.

6. Kim KH, Jeong MH, Kim W, et al. A case of systemic lupus erythematosus with severe pulmonary hypertension and pericarditis. Korean Circ J. 2000; 30:605–610.

7. Quismorio FP Jr, Sharma O, Koss M, et al. Immunopathologic and clinical studies in pulmonary hypertension associated with systemic lupus erythematosus. Semin Arthritis Rheum. 1984; 13:349–359.

8. Nakagawa N, Osanai S, Ide H, et al. Severe pulmonary hypertension associated with primary Sjögren's syndrome. Intern Med. 2003; 42:1248–1252.

9. Sowa JM. The association of Sjögren's syndrome and primary pulmonary hypertension. Hum Pathol. 1993; 24:1035–1036.

10. Sato T, Matsubara O, Tanaka Y, Kasuga T. Association of Sjögren's syndrome with pulmonary hypertension: report of two cases and review of the literature. Hum Pathol. 1993; 24:199–205.

11. Launay D, Hachulla E, Hatron PY, Jais X, Simonneau G, Humbert M. Pulmonary arterial hypertension: a rare complication of primary Sjögren syndrome: report of 9 new cases and review of the literature. Medicine (Baltimore). 2007; 86:299–315.

12. Vitali C, Bombardieri S, Jonsson R, et al. Classification criteria for Sjögren's syndrome: a revised version of the European criteria proposed by the American-European Consensus Group. Ann Rheum Dis. 2002; 61:554–558.

13. Kassan SS, Moutsopoulos HM. Clinical manifestations and early diagnosis of Sjögren syndrome. Arch Intern Med. 2004; 164:1275–1284.

14. Distler O, Pignone A. Pulmonary arterial hypertension and rheumatic diseases--from diagnosis to treatment. Rheumatology (Oxford). 2006; 45:Suppl 4. iv22–iv25.

15. Fuster V, Steele PM, Edwards WD, Gersh BJ, McGoon MD, Frye RL. Primary pulmonary hypertension: natural history and the importance of thrombosis. Circulation. 1984; 70:580–587.

16. Sanchez O, Humbert M, Sitbon O, Simonneau G. Treatment of pulmonary hypertension secondary to connective tissue diseases. Thorax. 1999; 54:273–277.

17. Sanchez O, Sitbon O, Jaïs X, Simonneau G, Humbert M. Immunosuppressive therapy in connective tissue diseases-associated pulmonary arterial hypertension. Chest. 2006; 130:182–189.

18. Kamata Y, Nara H, Sato H, Masuyama JI, Minota S, Yoshio T. Effect of steroid pulse therapy on mixed connective tissue disease with pulmonary arterial hypertension. Ann Rheum Dis. 2005; 64:1236–1237.

19. Gonzalez-Lopez L, Cardona-Muñoz EG, Celis A, et al. Therapy with intermittent pulse cyclophosphamide for pulmonary hypertension associated with systemic lupus erythematosus. Lupus. 2004; 13:105–112.