Severe pulmonary hypertension as the initial manifestation of systemic lupus erythematosus: A case report and review of the literature

Clinical and Experimental Rheumatology

Volumn 32, Issue 2, 2014, Pages 267-274

  Prete, Marcella ^a   Fatone, Maria Celeste ^a   Vacca, Angelo ^a   Racanelli, Vito ^a   Perosa, Federico ^a  

^a UNIVERSITY OF BARI MEDICAL SCHOOL   (Italy)

Author keywords

Cyclosporine; Mycophenolate mofetil; Pulmonary hypertension; Systemic lupus erythematosus; Therapy

Indexed keywords

AMINO TERMINAL PRO BRAIN NATRIURETIC PEPTIDE; AMLODIPINE; ANTINUCLEAR ANTIBODY; BOSENTAN; CYCLOSPORIN; D DIMER; DOUBLE STRANDED DNA ANTIBODY; ENOXAPARIN; ILOPROST; LOSARTAN; MYCOPHENOLIC ACID 2 MORPHOLINOETHYL ESTER; PREDNISOLONE; RIBONUCLEOPROTEIN ANTIBODY; RO ANTIBODY; SILDENAFIL; SM ANTIBODY; ALBUMIN; C REACTIVE PROTEIN; CYCLOPHOSPHAMIDE; HEMOGLOBIN; MYCOPHENOLATE MOFETIL;

ADULT; ARTHRALGIA; CARDIOMEGALY; CASE REPORT; COMPUTED TOMOGRAPHIC ANGIOGRAPHY; DRUG DOSE INCREASE; DYSPNEA; FEMALE; FEVER; FORCED EXPIRATORY VOLUME; FORCED VITAL CAPACITY; HEART CATHETERIZATION; HEART PALPITATION; HEART STRESS; HEPATOMEGALY; HIGH RESOLUTION COMPUTER TOMOGRAPHY; HUMAN; HYPERGAMMAGLOBULINEMIA; LUNG ANGIOGRAPHY; LUNG CONGESTION; LUNG DIFFUSION CAPACITY; LUNG EMBOLISM; LYMPHADENOPATHY; PERICARDIAL EFFUSION; PLEURA EFFUSION; PRIORITY JOURNAL; PROTEINURIA; PULMONARY HYPERTENSION; REVIEW; SPLENOMEGALY; SYSTEMIC LUPUS ERYTHEMATOSUS; SYSTOLIC HEART MURMUR; THORAX PAIN; TRANSTHORACIC ECHOCARDIOGRAPHY; WEAKNESS; COMPUTER ASSISTED TOMOGRAPHY; EARLY DIAGNOSIS; ERYTHROCYTE SEDIMENTATION RATE; HEART MURMUR; IMMUNOSUPPRESSIVE TREATMENT; INTERSTITIAL LUNG DISEASE; LUNG ARTERY PRESSURE; LUNG CAPACITY; MEDIASTINUM LYMPHADENOPATHY; NEW YORK HEART ASSOCIATION CLASS; THORAX RADIOGRAPHY; THROMBOCYTE COUNT; TREATMENT DURATION; TREATMENT REFUSAL; TRICUSPID VALVE REGURGITATION; VEIN DILATATION;

ADULT; ANTIBODIES, ANTIPHOSPHOLIPID; AUTOIMMUNITY; BIOLOGICAL MARKERS; CLINICAL TRIALS AS TOPIC; CYCLOSPORINE; DELAYED DIAGNOSIS; DISEASE MANAGEMENT; ENDOTHELIN-1; FEMALE; HUMANS; HYPERTENSION, PULMONARY; IMMUNOSUPPRESSIVE AGENTS; LUPUS ERYTHEMATOSUS, SYSTEMIC; MYCOPHENOLIC ACID; PREDICTIVE VALUE OF TESTS; RHEUMATOID FACTOR; SEVERITY OF ILLNESS INDEX; TREATMENT OUTCOME;

EID: 84904747335     PISSN: 0392856X     EISSN: 1593098X     Source Type: Journal    
DOI: None     Document Type: Review

References (57)

1. POPE J: An update in pulmonary hypertension in systemic lupus erythematosus-do we need to know about it. Lupus 2008; 17: 274-7.
2. AGMON-LEVIN N, MOSCA M, PETRI M, SHOENFELD Y: Systemic lupus erythematosus one disease or many. Autoimmun Rev 2012; 11: 593-5.
3. McLAUGHLIN VV, ARCHER SL, BADESCH DB et al.: ACCF/AHA 2009 expert consensus document on pulmonary hypertension a report of the American College of Cardiology Foundation Task Force on Expert Consensus Documents and the American Heart Association developed in collaboration with the American College of Chest Physicians; American Thoracic Society, Inc.; and the Pulmonary Hypertension Association. .
4. 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: 349-59.
5. BERNATSKY S, BOIVIN JF, JOSEPH L et al.:Mortality in systemic lupus erythematosus. Arthritis Rheum 2006; 54: 2550-7.
6. KAMEL SR, OMAR GM, DARWISH AF, ASKLANY HT, ELLABBAN AS: Asymptomatic pulmonary hypertension in systemic lupus erythematosus. Clin Med Insights Arthritis Musculoskelet Disord 2011; 4: 77-86.
7. LIAN F, CHEN D, WANG Y et al.: Clinical features and independent predictors of pulmonary arterial hypertension in systemic lupus erythematosus. Rheumatol Int 2012; 32: 1727-31.
8. XIA YK, TU SH, HU YH et al.: Pulmonary hypertension in systemic lupus erythematosus: a systematic review and analysis of 642 cases in Chinese population. Rheumatol Int 2012; 33: 1211-7.
9. JOHNSON SR, GRANTON JT: Pulmonary hypertension in systemic sclerosis and systemic lupus erythematosus. Eur Respir Rev 2011; 20: 277-86.
10. McGOON MD, MILLER DP: REVEAL: a contemporary US pulmonary arterial hypertension registry. Eur Respir Rev 2012; 21: 8-18.
11. VEGH J, SZODORAY P, KAPPELMAYER J et al.: Clinical and immunoserological characteristics of mixed connective tissue disease associated with pulmonary arterial hypertension. Scand J Immunol 2006; 64: 69-76.
12. MUKERJEE D, ST GD, COLEIRO B et al.: Prevalence and outcome in systemic sclerosis associated pulmonary arterial hypertension: application of a registry approach. Ann Rheum Dis 2003; 62: 1088-93.
13. IUDICI M, CODULLO V, GIUGGIOLI D et al.: Pulmonary hypertension in systemic sclerosis: prevalence, incidence and predictive factors in a large multicentric Italian cohort. Clin Exp Rheumatol 2013; 31 (Suppl. 76): 31-6.
14. LAUNAY D, HACHULLA E, HATRON PY, JAIS X, SIMONNEAU G, HUMBERT M: Pulmonary arterial hypertension: a rare complication of primary Sjogren syndrome: report of 9 new cases and review of the literature. Medicine (Baltimore) 2007; 86: 299-315.
15. DAWSON JK, GOODSON NG, GRAHAM DR, LYNCH MP: Raised pulmonary artery pressures measured with Doppler echocardiography in rheumatoid arthritis patients. Rheumatology (Oxford) 2000; 39: 1320-5.
16. KONIARI I, SIMINELAKIS SN, BAIKOUSSIS NG, PAPADOPOULOS G, GOUDEVENOS J, APOSTOLAKIS E: Antiphospholipid syndrome; its implication in cardiovascular diseases: a review. J Cardiothorac Surg 2010; 5: 101.
17. MINAI OA: Pulmonary hypertension in polymyositis-dermatomyositis: clinical and hemodynamic characteristics and response to vasoactive therapy. Lupus 2009; 18 :1006-10.
18. FUNAUCHI M, SHIMADZU H, TAMAKI C et al.: Survival study by organ disorders in 306 Japanese patients with systemic lupus erythematosus: results from a single center. Rheumatol Int 2007; 27: 243-9.
19. HAAS C: [Pulmonary hypertension associated with systemic lupus erythematosus]. Bull Acad Natl Med 2004; 188: 985-97.
20. RUBIN LJ, BADESCH DB, BARST RJ et al.: Bosentan therapy for pulmonary arterial hypertension. N Engl J Med 2002; 346: 896-903.
21. RUIZ-IRASTORZA G, GARMENDIA M, VILLAR I, EGURBIDE MV, AGUIRRE C: Pulmonary hypertension in systemic lupus erythematosus: prevalence, predictors and diagnostic strategy. Autoimmun Rev 2013; 12: 410-5.
22. CHUNG SM, LEE CK, LEE EY, YOO B, LEE SD, MOON HB: Clinical aspects of pulmonary hypertension in patients with systemic lupus erythematosus and in patients with idiopathic pulmonary arterial hypertension. Clin Rheumatol 2006; 25: 866-72.
23. PRABU A, PATEL K, YEE CS et al.: Prevalence and risk factors for pulmonary arterial hypertension in patients with lupus. Rheumatology (Oxford) 2009; 48: 1506-11.
24. CHUNG L, LIU J, PARSONS L et al.: Characterization of connective tissue disease-associated pulmonary arterial hypertension from REVEAL: identifying systemic sclerosis as a unique phenotype. Chest 2010; 138: 1383-94.
25. KAWAMURA N, TSUTSUI H, FUKUYAMA K et al.: Severe pulmonary hypertension in a patient with systemic lupus erythematosus and minimal lupus activity. Intern Med 2002; 41: 109-12.
26. LUO YF, ROBBINS IM, KARATAS M, BRIXEY AG, RICE TW, LIGHT RW: Frequency of pleural effusions in patients with pulmonary arterial hypertension associated with connective tissue diseases. Chest 2011; 140: 42-7.
27. DOMICIANO DS, CARVALHO JF, SHOENFELD Y: Pathogenic role of anti-endothelial cell antibodies in autoimmune rheumatic diseases. Lupus 2009; 18: 1233-8.
28. KANAKIS MA, KAPSIMALI V, VAIOPOULOS AG, VAIOPOULOS GA, SAMARKOS M: The lung in the spectrum of antiphospholipid syndrome. Clin Exp Rheumatol 2013; 31: 452-7.
29. CEFLE A, INANC M, SAYARLIOGLU M et al.: Pulmonary hypertension in systemic lupus erythematosus: relationship with antiphospholipid antibodies and severe disease outcome. Rheumatol Int 2011; 31: 183-9.
30. ALLEN KL, FONSECA FV, BETAPUDI V, WILLARD B, ZHANG J, McCRAE KR: A novel pathway for human endothelial cell activation by antiphospholipid/anti-beta2 glycoprotein I antibodies. Blood 2012; 119: 884-93.
31. SATTA N, KRUITHOF EK, FICKENTSCHER C et al.: Toll-like receptor 2 mediates the activation of human monocytes and endothelial cells by antiphospholipid antibodies. Blood 2011; 117: 5523-31.
32. XU G, WEN H, ZHOU H et al.: Involvement of IRAKs and TRAFs in anti-beta(2)GPI/ beta(2)GPI-induced tissue factor expression in THP-1 cells. Thromb Haemost 2011; 106: 1158-69.
33. KIROU KA, GKROUZMAN E: Anti-interferon alpha treatment in SLE. Clin Immunol 2013; 148: 303-12.
34. SHEN JY, CHEN SL, WU YX et al.: Pulmonary hypertension in systemic lupus erythematosus. Rheumatol Int 1999; 18: 147-51.
35. SASAKI N, KAMATAKI A, SAWAI T: A histopathological study of pulmonary hypertension in connective tissue disease. Allergol Int 2011; 60: 411-7.
36. DHALA A: Pulmonary arterial hypertension in systemic lupus erythematosus: current status and future direction. Clin Dev Immunol 2012; 2012: 854-941.
37. CONDLIFFE R, KIELY DG, PEACOCK AJ et al.: Connective tissue disease-associated pulmonary arterial hypertension in the modern treatment era. Am J Respir Crit Care Med 2009; 179: 151-7.
38. TANASEANU C, TUDOR S, TAMSULEA I, MARTA D, MANEA G, MOLDOVEANU E: Vascular endothelial growth factor, lipoporotein-associated phospholipase A2, sP-selectin and antiphospholipid antibodies, biological markers with prognostic value in pulmonary hypertension associated with chronic obstructive pulmonary disease and systemic lupus erithematosus. Eur J Med Res 2007; 12: 145-51.
39. KASUKAWA R, TOJI T, MIYAWAKI S: Preliminary diagnostic criteria for classification of mixed connective tissue disease. In: KASUKAWA R, SHARP G (Eds.): Mixed connective tissue disease and antinuclear antibodies. Amsterdam, Elsevier, 1987: 41-7.
40. ALARCON-SEGOVIA D, CARDIEL MH: Comparison between 3 diagnostic criteria for mixed connective tissue disease. Study of 593 patients. J Rheumatol 1989; 16: 328-34.
41. ORTEGA-HERNANDEZ OD, SHOENFELD Y: Mixed connective tissue disease: an overview of clinical manifestations, diagnosis and treatment. Best Pract Res Clin Rheumatol 2012; 26: 61-72.
42. ZHENG Y, LI M, ZHANG Y, SHI X, LI L, JIN M: The effects and mechanisms of mycophenolate mofetil on pulmonary arterial hypertension in rats. Rheumatol Int 2010; 30: 341-8.
43. SUZUKI C, TAKAHASHI M, MORIMOTO H et al.: Mycophenolate mofetil attenuates pulmonary arterial hypertension in rats. Biochem Biophys Res Commun 2006; 349: 781-8.
44. KOULMANN N, NOVEL-CHATE V, PEINNEQUIN A et al.: Cyclosporin A inhibits hypoxia-induced pulmonary hypertension and right ventricle hypertrophy. Am J Respir Crit Care Med 2006; 174: 699-705.
45. LI M, LIU Y, JIN F et al.: Endothelin-1 induces hypoxia inducible factor 1alpha expression in pulmonary artery smooth muscle cells. FEBS Lett 2012; 586: 3888-93.
46. BONNET S, ROCHEFORT G, SUTENDRA G et al.: The nuclear factor of activated T cells in pulmonary arterial hypertension can be therapeutically targeted. Proc Natl Acad Sci USA 2007; 104: 11418-23.
47. GONZALEZ-LOPEZ L, CARDONA-MUNOZ EG, CELIS A et al.: Therapy with intermittent pulse cyclophosphamide for pulmonary hypertension associated with systemic lupus erythematosus. Lupus 2004; 13: 105-12.
48. OUDIZ RJ, SCHILZ RJ, BARST RJ et al.: Treprostinil, a prostacyclin analogue, in pulmonary arterial hypertension associated with connective tissue disease. Chest 2004; 126: 420-7.
49. DENTON CP, HUMBERT M, RUBIN L, BLACK CM: Bosentan treatment for pulmonary arterial hypertension related to connective tissue disease: a subgroup analysis of the pivotal clinical trials and their open-label extensions. Ann Rheum Dis 2006; 65: 1336-40.
50. SANCHEZ O, SITBON O, JAIS X, SIMONNEAU G, HUMBERT M: Immunosuppressive therapy in connective tissue diseases-associated pulmonary arterial hypertension. Chest 2006; 130: 182-9.
51. BADESCH DB, HILL NS, BURGESS G et al.: Sildenafil for pulmonary arterial hypertension associated with connective tissue disease. J Rheumatol 2007; 34: 2417-22.
52. DENTON CP, POPE JE, PETER HH et al.: Long-term effects of bosentan on quality of life, survival, safety and tolerability in pulmonary arterial hypertension related to connective tissue diseases. Ann Rheum Dis 2008; 67: 1222-8.
53. JAIS X, LAUNAY D, YAICI A et al.: Immunosuppressive therapy in lupus-and mixed connective tissue disease-associated pulmonary arterial hypertension: a retrospective analysis of twenty-three cases. Arthritis Rheum 2008; 58: 521-31.
54. MIYAMICHI-YAMAMOTO S, FUKUMOTO Y, SUGIMURA K et al.: Intensive immunosuppressive therapy improves pulmonary hemodynamics and long-term prognosis in patients with pulmonary arterial hypertension associated with connective tissue disease. Circ J 2011; 75: 2668-74.
55. GALIE N, HOEPER MM, HUMBERT M et al.: Guidelines for the diagnosis and treatment of pulmonary hypertension: the Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS), endorsed by the International Society of Heart and Lung Transplantation (ISHLT). Eur Heart J 2009; 30: 2493-537.
56. MOSCA M, BOUMPAS DT, BRUCE IN et al.: Treat-to-target in systemic lupus erythematosus: where are we today. Clin Exp Rheumatol 2012; 30 (Suppl. 73): S112-S115.
57. IACCARINO L, RAMPUDDA M, CANOVA M, DELLA LIBERA S, SARZI-PUTTINI P, DORIA A: Mycophenolate mofetil: what is its place in the treatment of autoimmune rheumatic diseases. Autoimmun Rev 2007; 6: 190-5.