Systemic Lupus Erythematosus: Primary Care Approach to Diagnosis and Management

 


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Am Fam Physician. 2016 Aug 15;94(4):284-294.

  Patient information: See related handout on lupus, written by the authors of this article.

Related Close-Up: Joint Pain and Fatigue: It Could Be Anything.

Author disclosure: No relevant financial affiliations.

Systemic lupus erythematosus is an autoimmune disease that affects many systems, including the skin, musculoskeletal, renal, neuropsychiatric, hematologic, cardiovascular, pulmonary, and reproductive systems. Family physicians should be familiar with the manifestations of lupus to aid in early diagnosis, monitoring patients with mild disease, recognizing warning signs that require referral to a rheumatologist, and helping to monitor disease activity and treatment in patients with moderate to severe disease. The American College of Rheumatology has 11 classification criteria for lupus. If a patient meets at least four criteria, lupus can be diagnosed with 95% specificity and 85% sensitivity. All patients with lupus should receive education, counseling, and support. Hydroxychloroquine is the cornerstone of treatment because it reduces disease flares and other constitutional symptoms. Low-dose glucocorticoids can be used to treat most manifestations of lupus. The use of immunosuppressive and cytotoxic agents depends on the body systems affected. Patients with mild disease that does not involve major organ systems can be monitored by their family physician. Patients with increased disease activity, complications, or adverse effects from treatment should be referred to a rheumatologist. To optimize treatment, it is important that a rheumatologist coordinate closely with the patient's family physician to improve chronic care as well as preventive health services.

Systemic lupus erythematosus (SLE) is an autoimmune disease that affects the skin and musculoskeletal, renal, neuropsychiatric, hematologic, cardiovascular, pulmonary, and reproductive systems. Its course is typically recurrent, with periods of relative remission followed by flares. SLE can be fatal and significantly increases the risk of cardiovascular disease.

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BEST PRACTICES IN RHEUMATOLOGY: RECOMMENDATIONS FROM THE CHOOSING WISELY CAMPAIGN

RecommendationSponsoring organization

Do not test antinuclear antibodies (ANA) subserologies without a positive ANA and clinical suspicion of immune-mediated disease.

American College of Rheumatology


Source: For more information on the Choosing Wisely Campaign, see http://www.choosingwisely.org. For supporting citations and to search Choosing Wisely recommendations relevant to primary care, see http://www.aafp.org/afp/recommendations/search.htm.

BEST PRACTICES IN RHEUMATOLOGY: RECOMMENDATIONS FROM THE CHOOSING WISELY CAMPAIGN

RecommendationSponsoring organization

Do not test antinuclear antibodies (ANA) subserologies without a positive ANA and clinical suspicion of immune-mediated disease.

American College of Rheumatology


Source: For more information on the Choosing Wisely Campaign, see http://www.choosingwisely.org. For supporting citations and to search Choosing Wisely recommendations relevant to primary care, see http://www.aafp.org/afp/recommendations/search.htm.

View/Print Table

SORT: KEY RECOMMENDATIONS FOR PRACTICE

Clinical recommendationEvidence ratingReferences

The initial evaluation for suspected SLE should include an antinuclear antibody test.

C

15

Patients diagnosed with SLE must meet at least four of the 11 American College of Rheumatology diagnostic criteria.

C

8

Treatment of mild SLE includes patient education, expectations of treatment, and counseling to avoid extensive ultraviolet light exposure and overexertion.

C

8

Hydroxychloroquine (Plaquenil) has been shown to reduce arthritis pain associated with SLE.

A

32

A combination of glucocorticoid plus immunosuppressant is more effective than glucocorticoids alone in preserving renal function in patients with SLE.

A

32

A combination of glucocorticoid and mycophenolate (Cellcept) or cyclophosphamide is effective in achieving remission in patients with SLE nephritis.

A

28


SLE = systemic lupus erythematosus.

A = consistent, good-quality patient-oriented evidence; B = inconsistent or limited-quality patient-oriented evidence; C = consensus, disease-oriented evidence, usual practice, expert opinion, or case series. For information about the SORT evidence rating system, go to http://www.aafp.org/afpsort.

SORT: KEY RECOMMENDATIONS FOR PRACTICE

Clinical recommendationEvidence ratingReferences

The initial evaluation for suspected SLE should include an antinuclear antibody test.

C

15

Patients diagnosed with SLE must meet at least four of the 11 American College of Rheumatology diagnostic criteria.

C

8

Treatment of mild SLE includes patient education, expectations of treatment, and counseling to avoid extensive ultraviolet light exposure and overexertion.

C

8

Hydroxychloroquine (Plaquenil) has been shown to reduce arthritis pain associated with SLE.

A

32

A combination of glucocorticoid plus immunosuppressant is more effective than glucocorticoids alone in preserving renal function in patients with SLE.

A

32

A combination of glucocorticoid and mycophenolate (Cellcept) or cyclophosphamide is effective in achieving remission in patients with SLE nephritis.

A

28


SLE = systemic lupus erythematosus.

A = consistent,

The Authors

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NGUYET-CAM VU LAM, MD, is associate program director at St. Luke's Family Medicine Residency, St. Luke's University Hospital, Bethlehem, Pa....

MARIA V. GHETU, MD, is clinical faculty and staff geriatrician at St. Luke's Family Medicine Residency, St. Luke's University Hospital, Bethlehem, Pa.

MARZENA L. BIENIEK, MD, is a rheumatologist in Whitehall, Pa., who is affiliated with St. Luke's University Hospital.

Address correspondence to Nguyet-Cam Vu Lam, MD, St. Luke's Family Medicine Residency, 2830 Easton Ave., Bethlehem, PA 18017 (e-mail: nguyet-cam.lam@sluhn.org). Reprints are not available from the authors.

Author disclosure: No relevant financial affiliations.

REFERENCES

show all references

1. Helmick CG, Felson DT, Lawrence RC, et al.; National Arthritis Data Workgroup. Estimates of the prevalence of arthritis and other rheumatic conditions in the United States. Part I. Arthritis Rheum. 2008;58(1):15–25....

2. Somers EC, Marder W, Cagnoli P, et al. Population-based incidence and prevalence of systemic lupus erythematosus: the Michigan Lupus Epidemiology and Surveillance program. Arthritis Rheumatol. 2014;66(2):369–378.

3. Bernatsky S, Boivin JF, Joseph L, et al. Mortality in systemic lupus erythematosus. Arthritis Rheum. 2006;54(8):2550–2557.

4. Jakes RW, Bae SC, Louthrenoo W, Mok CC, Navarra SV, Kwon N. Systematic review of the epidemiology of systemic lupus erythematosus in the Asia-Pacific region: prevalence, incidence, clinical features, and mortality. Arthritis Care Res (Hoboken). 2012;64(2):159–168.

5. Burgos PI, Alarcón GS. Preventive health services for systemic lupus erythematosus patients: whose job is it? Arthritis Res Ther. 2010;12(3):124.

6. Hale ED, Treharne GJ, Lyons AC, et al. “Joining the dots” for patients with systemic lupus erythematosus: personal perspectives of health care from a qualitative study. Ann Rheum Dis. 2006;65(5):585–589.

7. Yazdany J, Tonner C, Trupin L, et al. Provision of preventive health care in systemic lupus erythematosus: data from a large observational cohort study. Arthritis Res Ther. 2010;12(3):R84.

8. American College of Rheumatology Ad Hoc Committee on Systemic Lupus Erythematosus Guidelines. Guidelines for referral and management of systemic lupus erythematosus in adults. Arthritis Rheum. 1999;42(9):1785–1796.

9. Zonana-Nacach A, Roseman JM, McGwin G Jr, et al. Systemic lupus erythematosus in three ethnic groups. VI: Factors associated with fatigue within 5 years of criteria diagnosis. LUMINA Study Group. LUpus in MInority populations: NAture vs Nurture. Lupus. 2000;9(2):101–109.

10. Zoma A. Musculoskeletal involvement on systemic lupus erythematosus. Lupus. 2004;13(11):851–853.

11. Cervera R, Khamashta MA, Font J, et al.; European Working Party on Systemic Lupus Erythematosus. Morbidity and mortality in systemic lupus erythematosus during a 10-year period: a comparison of early and late manifestations in a cohort of 1,000 patients. Medicine (Baltimore). 2003;82(5):299–308.

12. Longo DL, Fauci AS, Kasper DL, Hauser SL, Jameson JL, Loscalzo J, eds. Harrison's Principles of Internal Medicine. 18th ed. New York, NY: McGraw-Hill; 2012.

13. Petri M, Orbai AM, Alarcón GS, et al. Derivation and validation of the Systemic Lupus International Collaborating Clinics classification criteria for systemic lupus erythematosus. Arthritis Rheum. 2012;64(8):2677–2686.

14. Narain S, Richards HB, Satoh M, et al. Diagnostic accuracy for lupus and other systemic autoimmune diseases in the community setting. Arch Intern Med. 2004;164(22):2435–2441.

15. Hietarinta M, Lassila O. Clinical significance of antinuclear antibodies in systemic rheumatic diseases. Ann Med. 1996;28(4):283–291.

16. Tan EM, Cohen AS, Fries JF, et al. The 1982 revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum. 1982;25(11):1271–1277.

17. Hochberg MC. Updating the American College of Rheumatology revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum. 1997;40(9):1725.

18. Cojocaru M, Cojocaru IM, Silosi I, Vrabie CD. Manifestations of systemic lupus erythematosus. Maedica (Buchar). 2011;6(4):330–336.

19. Sinicato NA, da Silva Cardoso PA, Appenzeller S. Risk factors in cardiovascular disease in systemic lupus erythematosus. Curr Cardiol Rev. 2013;9(1):15–19.

20. Bertsias G, Fanouriakis A, Boumpas DT. Treatment of systemic lupus erythematosus. In: Firestein GS, Budd RC, Gabriel SE, McInnes IB, O'Dell JR, eds. Kelly's Textbook of Rheumatology. 9th ed. Philadelphia, Pa.: Saunders Elsevier; 2013:1304–1330.

21. Del Pino-Sedeño T, Trujillo-Martín MM, Ruiz-Irastorza G, Cuellar-Pompa L, de Pascual-Medina AM, Serrano-Aguilar P; Spanish Systemic Lupus Erythematosus CPG Development Group. Effectiveness of nonpharmacologic interventions for decreasing fatigue in adults with systemic lupus erythematosus: a systemic review. Arthritis Care Res (Hoboken). 2016;68(1):141–148.

22. Drakoulogkona O, Barbulescu AL, Rica I, Musetescu AE, Ciurea PL. The outcome of patients with lupus nephritis and the impact of cardiovascular risk factors. Curr Health Sci J. 2011;37(2):70–74.

23. Petri M, Purvey S, Fang H, Magder LS. Predictors of organ damage in systemic lupus erythematosus: the Hopkins Lupus Cohort. Arthritis Rheum. 2012;64(12):4021–4028.

24. Ruiz-Irastorza G, Ramos-Casals M, Brito-Zeron P, Khamashta MA. Clinical efficacy and side effects of antimalarials in systemic lupus erythematosus: a systematic review. Ann Rheum Dis. 2010;69(1):20–28.

25. Wallace DJ, Gudsoorkar VS, Weisman MH, Venuturupalli SR. New insights into mechanisms of therapeutic effects of antimalarial agents in SLE. Nat Rev Rheumatol. 2012;8(9):522–533.

26. Muangchan C, van Vollenhoven RF, Bernatsky SR, et al. Treatment algorithms in systemic lupus erythematosus. Arthritis Care Res (Hoboken). 2015;67(9):1237–1245.

27. Hahn BH, McMahon MA, Wilkinson A, et al. American College of Rheumatology guidelines for screening, treatment, and management of lupus nephritis. Arthritis Care Res (Hoboken). 2012;64(6):797–808.

28. Henderson L, Masson P, Craig JC, et al. Treatment for lupus nephritis. Cochrane Database Syst Rev. 2012;(12):CD002922.

29. Bertsias GK, Tektonidou M, Amoura Z, et al. Joint European League Against Rheumatism and European Renal Association-European Dialysis and Transplant Association (EULAR/ERA-EDTA) recommendations for the management of adult and paediatric lupus nephritis. Ann Rheum Dis. 2012;71(11):1771–1782.

30. Fernandes Moça Trevisani V, Castro AA, Ferreira Neves Neto J, Atallah AN. Cyclophosphamide versus methylprednisolone for treating neuropsychiatric involvement in systemic lupus erythematosus. Cochrane Database Syst Rev. 2013;(2):CD002265.

31. Bertsias GK, Ioannidis JP, Aringer M, et al. EULAR recommendations for the management of systemic lupus erythematosus with neuropsychiatric manifestations: report of a task force of the EULAR standing committee for clinical affairs. Ann Rheum Dis. 2010;69(12):2074–2082.

32. Madhok R, Wu O. Systemic lupus erythematosus. BMJ Clin Evid. 2009;2009:1123.

33. Dall'era M, Wofsy D. Clinical features of systemic lupus erythematosus. In: Firestein GS, Budd RC, Gabriel SE, McInnes IB, O'Dell JR, eds. Kelly's Textbook of Rheumatology. 9th ed. Philadelphia, Pa.: Saunders Elsevier; 2013:1283–1303.

34. Appel GB, Contreras G, Dooley MA, et al.; Aspreva Lupus Management Study Group. Mycophenolate mofetil versus cyclophosphamide for induction treatment of lupus nephritis. J Am Soc Nephrol. 2009;20(5):1103–1112.

35. Muscal E, Brey RL. Neurologic manifestations of systemic lupus erythematosus in children and adults. Neurol Clin. 2010;28(1):61–73.

36. Sivaraj RR, Durrani OM, Denniston AK, Murray PI, Gordon C. Ocular manifestations of systemic lupus erythematosus. Rheumatology (Oxford). 2007;46(12):1757–1762.

37. Merrill JT, Burgos-Vargas R, Westhovens R, et al. The efficacy and safety of abatacept in patients with non-life-threatening manifestations of systemic lupus erythematosus: results of a twelve-month, multicenter, exploratory, phase IIb, randomized, double-blind, placebo-controlled trial. Arthritis Rheum. 2010;62(10):3077–3087.

38. Hartkamp A, Geenen R, Godaert GL, Bijl M, Bijlsma JW, Derksen RH. Effects of dehydroepiandrosterone on fatigue and well-being in women with quiescent systemic lupus erythematosus: a randomised controlled trial. Ann Rheum Dis. 2010;69(6):1144–1147.

39. Neill J, Belan I, Ried K. Effectiveness of non-pharmacological interventions for fatigue in adults with multiple sclerosis, rheumatoid arthritis, or systemic lupus erythematosus: a systematic review [published correction appears in J Adv Nurs. 2007;57(2):225]. J Adv Nurs. 2006;56(6):617–635.

40. Tench CM, McCarthy J, McCurdie I, White PD, D'Cruz DP. Fatigue in systemic lupus erythematosus: a randomized controlled trial of exercise. Rheumatology (Oxford). 2003;42(9):1050–1054.

41. Balsamo S, Santos-Neto LD. Fatigue in systemic lupus erythematosus: an association with reduced physical fitness. Autoimmun Rev. 2011;10(9):514–518.

42. Manzi S, Meilahn EN, Rairie JE, et al. Age-specific incidence rates of myocardial infarction and angina in women with systemic lupus erythematosus: comparison with the Framingham Study. Am J Epidemiol. 1997;145(5):408–415.

43. Zuily S, Regnault V, Selton-Suty C, et al. Increased risk for heart valve disease associated with antiphospholipid antibodies in patients with systemic lupus erythematosus: meta-analysis of echocardiographic studies. Circulation. 2011;124(2):215–224.

44. Mosca L, Benjamin EJ, Berra K, et al. Effectiveness-based guidelines for the prevention of cardiovascular disease in women—2011 update: a guideline from the American Heart Association [published corrections appear in Circulation. 2011;123(22):e624 and Circulation. 2011;124(16):e427]. Circulation. 2011;123(11):1243–1262.

45. James PA, Oparil S, Carter BL, et al. 2014 evidence-based guideline for the management of high blood pressure in adults: report from the panel members appointed to the Eighth Joint National Committee (JNC 8) [published correction appears in JAMA. 2014;311(17):1809]. JAMA. 2014;311(5):507–520.

46. Stone NJ, Robinson JG, Lichtenstein AH, et al. 2013 ACC/AHA guideline on the treatment of blood cholesterol to reduce atherosclerotic cardiovascular risk in adults: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines [published correction appears in J Am Coll Cardiol. 2014;63(25 pt B):3024–3025]. J Am Coll Cardiol. 2014;63(25 pt B):2889–2934.

47. Esdaile JM, Abrahamowicz M, Grodzicky T, et al. Traditional Framingham risk factors fail to fully account for accelerated atherosclerosis in systemic lupus erythematosus. Arthritis Rheum. 2001;44(10):2331–2337.

48. Bruce IN, Urowitz MB, Gladman DD, Hallet DC. The natural history of hypercholesterolaemia in systemic lupus erythematosus. J Rheumatol. 1999;26(10):2137–2143.

49. McMahon M, Hahn BH, Skaggs BJ. Systemic lupus erythematosus and cardiovascular disease: prediction and potential for therapeutic intervention. Expert Rev Clin Immunol. 2011;7(2):227–241.

50. Keane MP, Lynch JP III. Pleuropulmonary manifestations of systemic lupus erythematosus. Thorax. 2000;55(2):159–166.

51. Todd NW, Wise RA. Respiratory complications in the collagen vascular diseases. Clin Pulm Med. 1996;3(2):101–112.

52. Carmier D, Marchand-Adam S, Diot P, Diot E. Respiratory involvement in systemic lupus erythematosus. Rev Mal Respir. 2010;27(8):e66–e78.

53. Culwell KR, Curtis KM, del Carmen Cravioto M. Safety of contraceptive method use among women with systemic lupus erythematosus: a systematic review. Obstet Gynecol. 2009;114(2 pt 1):341–353.

54. Centers for Disease Control and Prevention (CDC). Use of 13-valent pneumococcal conjugate vaccine and 23-valent pneumococcal polysaccharide vaccine for adults with immunocompromising conditions: recommendations of the Advisory Committee on Immunization Practices (ACIP). MMWR Morb Mortal Wkly Rep. 2012;61(40):816–819.

55. Goldberg A, Katzap E. Belimumab for the treatment of systemic lupus erythematosus. Int J Clin Rheum. 2010;5(4):407–413.

56. U.S. Food and Drug Administration. FDA news release. FDA approves Benlysta to treat lupus. http://www.fda.gov/NewsEvents/Newsroom/PressAnnouncements/ucm246489.htm. Accessed April 1, 2015.

57. Gordon C. Long-term complications of systemic lupus erythematosus. Rheumatology (Oxford). 2002;41(10):1095–1100.

58. Bernatsky S, Boivin JF, Joseph L, et al. An international cohort study of cancer in systemic lupus erythematosus. Arthritis Rheum. 2005;52(5):1481–1490.

59. Gill JM, Quisel AM, Rocca PV, Walters DT. Diagnosis of systemic lupus erythematosis. Am Fam Physician. 2003;68(11):2179–2187.



 

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