History

Eosinophilic granulomatosis with polyangiitis (EGPA) has three phases, as follows:

Allergic rhinitis and asthma
Eosinophilic infiltrative disease (eg, eosinophilic pneumonia or gastroenteritis)
Systemic medium- and small-vessel vasculitis with granulomatous inflammation

The vasculitic phase usually develops within 3 years after the onset of asthma, although it may be delayed for several decades. The most prominent symptoms and signs are those related to pulmonary, cardiac, dermatologic, renal, and peripheral nerve involvement. Mononeuritis multiplex is a major clinical finding.

The following symptoms and signs of the disease were reported by Guillevin et al in their case series^[3]:

Constitutional symptoms - Malaise, fatigue, flulike symptoms, weight loss (70%), fever (57%), myalgias (52%)
Asthma symptoms - Asthma is a central feature of EGPA, occurring in 97% of patients. Asthma may precede vasculitis by up to 10 years or, less frequently, may coincide with the appearance of vasculitis. Asthma symptoms are usually persistent; therefore, patients are usually treated with steroids. This, in turn, might mask other features of the syndrome.^[27]
Paranasal sinusitis (61%) - Usually responds to oral steroids
Allergic rhinitis - This is a common symptom. Additionally, recurrent sinusitis and polyposis are seen, but unlike in granulomatosis with polyangiitis [Wegener granulomatosis], necrotizing lesions of the upper airway are unusual.
Pulmonary symptoms (37%), including cough and hemoptysis
Arthralgias (40%)
Skin manifestations (49%)
Purpura - Skin nodules, urticarial rash, necrotic bulla, digital ischemia
Cardiac manifestations - Symptoms related to heart failure, myocarditis, pericarditis, constrictive pericarditis, and myocardial infarction
Gastrointestinal (GI) symptoms (31%) - Symptoms related to GI vasculitis, eosinophilic gastritis, or colitis; these include abdominal pain (59%), diarrhea (33%), and GI bleeding (18%).
Peripheral neuropathy - Mononeuritis multiplex (most frequent form, occurring in as many as 77% of patients)
Less frequent symptoms - Symptoms related to stroke, ophthalmologic involvement, and other rare symptoms

Physical Examination

Except for fever, the physical findings in EGPA are specific to organ-system involvement. Pulmonary involvement is the most prominent. In fact, a pneumonitis plus eosinophilia warrants consideration of this syndrome and a search for evidence of systemic vasculitis elsewhere.^[3]In addition to asthma and eosinophilia, a dermato-pulmonary-renal syndrome is the feature of this disease. Mononeuritis multiplex is common.

Skin involvement (60%) may include the following:

Leukocytoclastic angiitis with palpable purpura
Livedo reticularis, skin necrosis and gangrene, digital ischemia, urticaria, and subcutaneous nodules (See the image below.)
The skin rashes of Churg-Strauss syndrome. The biopsy of this rash showed eosinophilic leukocytoclastic angiitis with poorly formed granulomas.
View Media Gallery

Upper respiratory involvement may include the following:

Allergic rhinitis
Paranasal sinusitis
Nasal polyposis

Lower respiratory system physical findings are related to the following:

Asthma (ie, wheeze), expiratory rhonchi
Pneumonitis
Hemoptysis secondary to pulmonary alveolar hemorrhage (alveolar capillaritis)

Cardiovascular findings may include the following:

Myocarditis and signs related to heart failure
Myocardial infarction secondary to coronary vasculitis

Renal findings may include the following:

Hypertension
Signs of uremia and advanced kidney failure

Gastrointestinal findings may include the following:

GI bleeding
Bowel ischemia and perforation
Gastroenteritis
Appendicitis
Pancreatitis

Nervous system findings may include the following:

Peripheral neuropathy (includes mononeuritis multiplex [77%])
Central nervous system (includes stroke [5%])

In a study of 15 ANCA-positive and 35 ANCA-negative patients with EGPA, ANCA-positive patients had a significantly higher incidence of renal involvement, skin involvement, and peripheral neuropathy in the form of mononeuritis multiplex at the time of diagnosis. Over the entire follow-up period, ANCA-negative patients had significantly more frequent cardiac manifestations. ANCA-positive patients required significantly higher steroid doses to maintain remission.^[28]

Differential Diagnoses

Nguyen Y, Guillevin L. Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss). Semin Respir Crit Care Med. 2018 Aug. 39 (4):471-481. [QxMD MEDLINE Link].
[Guideline] Groh M, Pagnoux C, Baldini C, Bel E, Bottero P, Cottin V, et al. Eosinophilic granulomatosis with polyangiitis (Churg-Strauss) (EGPA) Consensus Task Force recommendations for evaluation and management. Eur J Intern Med. 2015 Sep. 26 (7):545-53. [QxMD MEDLINE Link]. [Full Text].
Guillevin L, Cohen P, Gayraud M, et al. Churg-Strauss syndrome. Clinical study and long-term follow-up of 96 patients. Medicine (Baltimore). 1999 Jan. 78(1):26-37. [QxMD MEDLINE Link].
Churg J, Strauss L. Allergic granulomatosis, allergic angiitis, and periarteritis nodosa. Am J Pathol. 1951 Mar-Apr. 27(2):277-301. [QxMD MEDLINE Link].
Churg A. Pulmonary angiitis and granulomatosis revisited. Hum Pathol. 1983 Oct. 14(10):868-83. [QxMD MEDLINE Link].
Keogh KA, Specks U. Churg-Strauss syndrome: clinical presentation, antineutrophil cytoplasmic antibodies, and leukotriene receptor antagonists. Am J Med. 2003 Sep. 115(4):284-90. [QxMD MEDLINE Link].
Masi AT, Hunder GG, Lie JT, et al. The American College of Rheumatology 1990 criteria for the classification of Churg-Strauss syndrome (allergic granulomatosis and angiitis). Arthritis Rheum. 1990 Aug. 33(8):1094-100. [QxMD MEDLINE Link].
Jennette JC, Falk RJ, Andrassy K, et al. Nomenclature of systemic vasculitides. Proposal of an international consensus conference. Arthritis Rheum. 1994 Feb. 37(2):187-92. [QxMD MEDLINE Link].
[Guideline] Grayson PC, Ponte C, Suppiah R, Robson JC, Craven A, Judge A, et al. 2022 American College of Rheumatology/European Alliance of Associations for Rheumatology Classification Criteria for Eosinophilic Granulomatosis with Polyangiitis. Ann Rheum Dis. 2022 Mar. 81 (3):309-314. [QxMD MEDLINE Link].
Cottin V, et al; Groupe d’Etudes et de Recherche sur les Maladies Orphelines Pulmonaires (GERM“O”P). Revisiting the systemic vasculitis in eosinophilic granulomatosis with polyangiitis (Churg-Strauss): A study of 157 patients by the Groupe d'Etudes et de Recherche sur les Maladies Orphelines Pulmonaires and the European Respiratory Society Taskforce on eosinophilic granulomatosis with polyangiitis (Churg-Strauss). Autoimmun Rev. 2017 Jan. 16 (1):1-9. [QxMD MEDLINE Link].
Hellmich B, Ehlers S, Csernok E, Gross WL. Update on the pathogenesis of Churg-Strauss syndrome. Clin Exp Rheumatol. 2003 Nov-Dec. 21(6 Suppl 32):S69-77. [QxMD MEDLINE Link].
Lyons PA, Peters JE, Alberici F, et al. Genome-wide association study of eosinophilic granulomatosis with polyangiitis reveals genomic loci stratified by ANCA status. Nat Commun. 2019 Nov 12. 10 (1):5120. [QxMD MEDLINE Link]. [Full Text].
Franco J, Artes MJ. Pulmonary eosinophilia associated with montelukast. Thorax. 1999 Jun. 54(6):558-60. [QxMD MEDLINE Link].
Wechsler ME, Finn D, Gunawardena D, et al. Churg-Strauss syndrome in patients receiving montelukast as treatment for asthma. Chest. 2000 Mar. 117(3):708-13. [QxMD MEDLINE Link].
Hauser T, Mahr A, Metzler C, Coste J, Sommerstein R, Gross WL, et al. The leucotriene receptor antagonist montelukast and the risk of Churg-Strauss syndrome: a case-crossover study. Thorax. 2008 Aug. 63(8):677-82. [QxMD MEDLINE Link].
Wechsler ME, Garpestad E, Flier SR, et al. Pulmonary infiltrates, eosinophilia, and cardiomyopathy following corticosteroid withdrawal in patients with asthma receiving zafirlukast. JAMA. 1998 Feb 11. 279(6):455-7. [QxMD MEDLINE Link].
Tuggey JM, Hosker HS. Churg-Strauss syndrome associated with montelukast therapy. Thorax. 2000 Sep. 55(9):805-6. [QxMD MEDLINE Link].
Vaglio A, Martorana D, Maggiore U, Grasselli C, Zanetti A, Pesci A, et al. HLA-DRB4 as a genetic risk factor for Churg-Strauss syndrome. Arthritis Rheum. 2007 Sep. 56(9):3159-66. [QxMD MEDLINE Link].
Chang HC, Chou PC, Lai CY, Tsai HH. Antineutrophil Cytoplasmic Antibodies and Organ-Specific Manifestations in Eosinophilic Granulomatosis with Polyangiitis: A Systematic Review and Meta-Analysis. J Allergy Clin Immunol Pract. 2021 Jan. 9 (1):445-452.e6. [QxMD MEDLINE Link].
Sinico RA, Sabadini E, Maresca AM. Mesalazine-induced Churg-Strauss syndrome in a patient with Crohn's disease and sclerosing cholangitis. Clin Exp Rheumatol. 2006 Mar-Apr. 24(2 Suppl 41):S104. [QxMD MEDLINE Link].
Orriols R, Munoz X, Ferrer J, Huget P, Morell F. Cocaine-induced Churg-Strauss vasculitis. Eur Respir J. 1996 Jan. 9(1):175-7. [QxMD MEDLINE Link].
Nappi E, De Santis M, Paoletti G, Pelaia C, Terenghi F, Pini D, et al. New Onset of Eosinophilic Granulomatosis with Polyangiitis Following mRNA-Based COVID-19 Vaccine. Vaccines (Basel). 2022 May 3. 10 (5):[QxMD MEDLINE Link]. [Full Text].
Eustace JA, Nadasdy T, Choi M. Disease of the month. The Churg Strauss Syndrome. J Am Soc Nephrol. 1999 Sep. 10(9):2048-55. [QxMD MEDLINE Link].
Boyer D, Vargas SO, Slattery D, Rivera-Sanchez YM, Colin AA. Churg-Strauss syndrome in children: a clinical and pathologic review. Pediatrics. 2006 Sep. 118(3):e914-20. [QxMD MEDLINE Link].
Keogh KA, Specks U. Churg-Strauss syndrome: update on clinical, laboratory and therapeutic aspects. Sarcoidosis Vasc Diffuse Lung Dis. 2006 Mar. 23(1):3-12. [QxMD MEDLINE Link].
Hasley PB, Follansbee WP, Coulehan JL. Cardiac manifestations of Churg-Strauss syndrome: report of a case and review of the literature. Am Heart J. 1990 Oct. 120(4):996-9. [QxMD MEDLINE Link].
Wechsler ME, Finn D, Gunawardena D, Westlake R, Barker A, Haranath SP, et al. Churg-Strauss syndrome in patients receiving montelukast as treatment for asthma. Chest. 2000 Mar. 117(3):708-13. [QxMD MEDLINE Link].
Sokolowska BM, Szczeklik WK, Wludarczyk AA, Kuczia PP, Jakiela BA, Gasior JA, et al. ANCA-positive and ANCA-negative phenotypes of eosinophilic granulomatosis with polyangiitis (EGPA): outcome and long-term follow-up of 50 patients from a single Polish center. Clin Exp Rheumatol. 2014 May-Jun. 32(3 Suppl 82):S41-7. [QxMD MEDLINE Link].
Sablé-Fourtassou R, Cohen P, Mahr A, Pagnoux C, Mouthon L, Jayne D, et al. Antineutrophil cytoplasmic antibodies and the Churg-Strauss syndrome. Ann Intern Med. 2005 Nov 1. 143(9):632-8. [QxMD MEDLINE Link].
Choi YH, Im JG, Han BK, Kim JH, Lee KY, Myoung NH. Thoracic manifestation of Churg-Strauss syndrome: radiologic and clinical findings. Chest. 2000 Jan. 117(1):117-24. [QxMD MEDLINE Link].
Kim Y, Lee KS, Choi DC, et al. The spectrum of eosinophilic lung disease: radiologic findings. J Comput Assist Tomogr. 1997 Nov-Dec. 21(6):920-30. [QxMD MEDLINE Link].
Buschman DL, Waldron JA Jr, King TE Jr. Churg-Strauss pulmonary vasculitis. High-resolution computed tomography scanning and pathologic findings. Am Rev Respir Dis. 1990 Aug. 142(2):458-61. [QxMD MEDLINE Link].
Worthy SA, Müller NL, Hansell DM, Flower CD. Churg-Strauss syndrome: the spectrum of pulmonary CT findings in 17 patients. AJR Am J Roentgenol. 1998 Feb. 170(2):297-300. [QxMD MEDLINE Link].
Mavrogeni S, Karabela G, Gialafos E, Stavropoulos E, Spiliotis G, Katsifis G, et al. Cardiac involvement in ANCA (+) and ANCA (-) Churg-Strauss syndrome evaluated by cardiovascular magnetic resonance. Inflamm Allergy Drug Targets. 2013 Oct. 12(5):322-7. [QxMD MEDLINE Link].
Lie JT. Histopathologic specificity of systemic vasculitis. Rheum Dis Clin North Am. 1995 Nov. 21(4):883-909. [QxMD MEDLINE Link].
Katzenstein AL. Diagnostic features and differential diagnosis of Churg-Strauss syndrome in the lung. A review. Am J Clin Pathol. 2000 Nov. 114(5):767-72. [QxMD MEDLINE Link].
Chemmalakuzhy AJ, Zhou XJ, Hedayati SS. ANCA-negative glomerulonephritis associated with nonasthmatic Churg-Strauss syndrome. Nat Clin Pract Nephrol. 2008 Oct. 4(10):568-74. [QxMD MEDLINE Link].
Lhote F, Cohen P, Guilpain P, Guillevin L. [Churg-Strauss syndrome]. Rev Prat. 2008 Jun 15. 58(11):1165-74. [QxMD MEDLINE Link].
Hervier B, Durant C, Masseau A, Ponge T, Hamidou M, Mussini JM. Use of muscle biopsies for diagnosis of systemic vasculitides. J Rheumatol. 2011 Mar. 38(3):470-4. [QxMD MEDLINE Link].
Ford JA, Aleatany Y, Gewurz-Singer O. Therapeutic advances in eosinophilic granulomatosis with polyangiitis. Curr Opin Rheumatol. 2022 May 1. 34 (3):158-164. [QxMD MEDLINE Link].
Mukhtyar C, Flossmann O, Hellmich B, Bacon P, Cid M, Cohen-Tervaert JW, et al. Outcomes from studies of antineutrophil cytoplasm antibody associated vasculitis: a systematic review by the European League Against Rheumatism systemic vasculitis task force. Ann Rheum Dis. 2008 Jul. 67(7):1004-10. [QxMD MEDLINE Link].
Mohammad AJ, Hot A, Arndt F, Moosig F, Guerry MJ, Amudala N, et al. Rituximab for the treatment of eosinophilic granulomatosis with polyangiitis (Churg-Strauss). Ann Rheum Dis. 2016 Feb. 75 (2):396-401. [QxMD MEDLINE Link].
Kitching AR, Anders HJ, Basu N, Brouwer E, Gordon J, Jayne DR, et al. ANCA-associated vasculitis. Nat Rev Dis Primers. 2020 Aug 27. 6 (1):71. [QxMD MEDLINE Link]. [Full Text].
Jachiet M, Samson M, Cottin V, Kahn JE, Le Guenno G, et al. Anti-IgE monoclonal antibody (omalizumab) in refractory and relapsing eosinophilic granulomatosis with polyangiitis (Churg-Strauss): Data from 17 patients. Arthritis Rheumatol. 2016 Mar 4. [QxMD MEDLINE Link].
Tiliakos A 4th, Shaia S, Hostoffer R, Kent L. The use of infliximab in a patient with steroid-dependent churg-strauss syndrome. J Clin Rheumatol. 2004 Apr. 10(2):96-7. [QxMD MEDLINE Link].
Casian A, Jayne D. Plasma exchange in the treatment of Wegener's granulomatosis, microscopic polyangiitis, Churg-Strauss syndrome and renal limited vasculitis. Curr Opin Rheumatol. 2011 Jan. 23(1):12-7. [QxMD MEDLINE Link].
Guillevin L, Cevallos R, Durand-Gasselin B, et al. Treatment of glomerulonephritis in microscopic polyangiitis and Churg-Strauss syndrome. Indications of plasma exchanges, Meta-analysis of 2 randomized studies on 140 patients, 32 with glomerulonephritis. Ann Med Interne (Paris). 1997. 148(3):198-204. [QxMD MEDLINE Link].
Bonaci-Nikolic B, Nikolic MM, Andrejevic S, Zoric S, Bukilica M. Antineutrophil cytoplasmic antibody (ANCA)-associated autoimmune diseases induced by antithyroid drugs: comparison with idiopathic ANCA vasculitides. Arthritis Res Ther. 2005. 7(5):R1072-81. [QxMD MEDLINE Link].
Wechsler ME, Akuthota P, Jayne D, Khoury P, Klion A, Langford CA, et al. Mepolizumab or Placebo for Eosinophilic Granulomatosis with Polyangiitis. N Engl J Med. 2017 May 18. 376 (20):1921-1932. [QxMD MEDLINE Link].
Koga Y, Aoki-Saito H, Kamide Y, Sato M, Tsurumaki H, Yatomi M, et al. Perspectives on the Efficacy of Benralizumab for Treatment of Eosinophilic Granulomatosis With Polyangiitis. Front Pharmacol. 2022. 13:865318. [QxMD MEDLINE Link]. [Full Text].
Espinoza LR. Combination therapy to treat churg-strauss syndrome: corticosteroids with short- or long-term cyclophosphamide pulses. Curr Rheumatol Rep. 2008 Dec. 10(6):427-9. [QxMD MEDLINE Link].
Yamasaki A, Tomita K, Fujii Y, Hasegawa Y, Watanabe M, Sano H, et al. Repressed ileal artery aneurysms in Churg-Strauss syndrome following combination treatment with glucocorticoid and cyclophosphamide. Rheumatol Int. 2008 Aug 8. [QxMD MEDLINE Link].
Ribi C, Cohen P, Pagnoux C, Mahr A, Arène JP, Lauque D, et al. Treatment of Churg-Strauss syndrome without poor-prognosis factors: a multicenter, prospective, randomized, open-label study of seventy-two patients. Arthritis Rheum. 2008 Feb. 58(2):586-94. [QxMD MEDLINE Link].
Tatsis E, Schnabel A, Gross WL. Interferon-alpha treatment of four patients with the Churg-Strauss syndrome. Ann Intern Med. 1998 Sep 1. 129(5):370-4. [QxMD MEDLINE Link].
Metzler C, Schnabel A, Gross WL, Hellmich B. A phase II study of interferon-alpha for the treatment of refractory Churg-Strauss syndrome. Clin Exp Rheumatol. 2008 May-Jun. 26(3 Suppl 49):S35-40. [QxMD MEDLINE Link].
Cohen P, Guillevin L, Baril L, et al. Persistence of antineutrophil cytoplasmic antibodies (ANCA) in asymptomatic patients with systemic polyarteritis nodosa or Churg-Strauss syndrome: follow-up of 53 patients. Clin Exp Rheumatol. 1995 Mar-Apr. 13(2):193-8. [QxMD MEDLINE Link].
[Guideline] Chung SA, Langford CA, Maz M, et al. 2021 American College of Rheumatology/Vasculitis Foundation Guideline for the Management of Antineutrophil Cytoplasmic Antibody-Associated Vasculitis. Arthritis Care Res (Hoboken). 2021 Aug. 73 (8):1088-1105. [QxMD MEDLINE Link]. [Full Text].
Beasley R, Bibby S, Weatherall M. Leukotriene receptor antagonist therapy and Churg-Strauss syndrome: culprit or innocent bystander?. Thorax. 2008 Oct. 63(10):847-9. [QxMD MEDLINE Link].
Giavina-Bianchi P, Giavina-Bianchi M, Agondi R, Kalil J. Three months' administration of anti-IgE to a patient with Churg-Strauss syndrome. J Allergy Clin Immunol. 2007 May. 119(5):1279; author reply 1279-80. [QxMD MEDLINE Link].
Green RL, Vayonis AG. Churg-Strauss syndrome after zafirlukast in two patients not receiving systemic steroid treatment. Lancet. 1999 Feb 27. 353(9154):725-6. [QxMD MEDLINE Link].
Guillevin L, Lhote F, Gayraud M, et al. Prognostic factors in polyarteritis nodosa and Churg-Strauss syndrome. A prospective study in 342 patients. Medicine (Baltimore). 1996 Jan. 75(1):17-28. [QxMD MEDLINE Link].
Kaushik VV, Reddy HV, Bucknall RC. Successful use of rituximab in a patient with recalcitrant Churg-Strauss syndrome. Ann Rheum Dis. 2006 Aug. 65(8):1116-7. [QxMD MEDLINE Link].
Kümpers P, Erdbrügger U, Grossheim M, Meyer GP, Hiss M, Gwinner W, et al. Endothelial microparticles as a diagnostic aid in Churg-Strauss vasculitis-induced cardiomyopathy. Clin Exp Rheumatol. 2008 May-Jun. 26(3 Suppl 49):S86-9. [QxMD MEDLINE Link].
Lie JT. Illustrated histopathologic classification criteria for selected vasculitis syndromes. American College of Rheumatology Subcommittee on Classification of Vasculitis. Arthritis Rheum. 1990 Aug. 33(8):1074-87. [QxMD MEDLINE Link].
Pabst S, Tiyerili V, Grohé C. Apparent response to anti-IgE therapy in two patients with refractory "forme fruste" of Churg-Strauss syndrome. Thorax. 2008 Aug. 63(8):747-8. [QxMD MEDLINE Link].
Roccatello D, Baldovino S, Alpa M, Rossi D, Napoli F, Naretto C, et al. Effects of anti-CD20 monoclonal antibody as a rescue treatment for ANCA-associated idiopathic systemic vasculitis with or without overt renal involvement. Clin Exp Rheumatol. 2008 May-Jun. 26(3 Suppl 49):S67-71. [QxMD MEDLINE Link].
Todd DC, Cockcroft DW. Prolonged survival in Churg-Strauss syndrome. Ann Allergy Asthma Immunol. 2004 Jan. 92(1):92-3. [QxMD MEDLINE Link].
Greco A, Rizzo MI, De Virgilio A, Gallo A, Fusconi M, Ruoppolo G, et al. Churg-Strauss syndrome. Autoimmun Rev. 2015 Apr. 14 (4):341-8. [QxMD MEDLINE Link].
Wechsler ME, Akuthota P, Jayne D, Khoury P, Klion A, Langford CA, et al. Mepolizumab or Placebo for Eosinophilic Granulomatosis with Polyangiitis. N Engl J Med. 2017 May 18. 376 (20):1921-1932. [QxMD MEDLINE Link].
Wechsler ME, Akuthota P, Jayne D, Khoury P, Klion A, Langford CA, et al. Mepolizumab or Placebo for Eosinophilic Granulomatosis with Polyangiitis. N Engl J Med. 2017 May 18. 376 (20):1921-1932. [QxMD MEDLINE Link]. [Full Text].
Canzian A, et al; French Vasculitis Study Group and the European EGPA Study Group. Use of Biologics to Treat Relapsing and/or Refractory Eosinophilic Granulomatosis With Polyangiitis: Data From a European Collaborative Study. Arthritis Rheumatol. 2021 Mar. 73 (3):498-503. [QxMD MEDLINE Link].

Media Gallery

Transient pulmonary infiltrates in a patient with Churg-Strauss syndrome (CSS).
Eosinophilic granuloma in a patient with Churg-Strauss syndrome (CSS).
The skin rashes of Churg-Strauss syndrome. The biopsy of this rash showed eosinophilic leukocytoclastic angiitis with poorly formed granulomas.

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Author

Spencer T Lowe, MD Rheumatologist, Private Practice, Peninsula Medical Group, Burlingame, CA

Spencer T Lowe, MD is a member of the following medical societies: American College of Rheumatology, California Medical Association, International Society for Clinical Densitometry

Disclosure: Nothing to disclose.

Coauthor(s)

Christopher L Tracy, MD Associate Program Director, Rheumatology Fellowship Program, Walter Reed National Military Medical Center

Christopher L Tracy, MD is a member of the following medical societies: American College of Physicians, American College of Rheumatology, American Medical Association

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Lawrence H Brent, MD Associate Professor of Medicine, Sidney Kimmel Medical College of Thomas Jefferson University; Chair, Program Director, Department of Medicine, Division of Rheumatology, Albert Einstein Medical Center

Lawrence H Brent, MD is a member of the following medical societies: American Association for the Advancement of Science, American Association of Immunologists, American College of Physicians, American College of Rheumatology

Disclosure: Stock ownership for: Johnson & Johnson.

Chief Editor

Herbert S Diamond, MD Visiting Professor of Medicine, Division of Rheumatology, State University of New York Downstate Medical Center; Chairman Emeritus, Department of Internal Medicine, Western Pennsylvania Hospital

Herbert S Diamond, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians, American College of Rheumatology, American Medical Association, Phi Beta Kappa

Disclosure: Nothing to disclose.

Additional Contributors

Mehran Farid-Moayer, MD Adjunct Clinical Faculty, Department of Psychiatry, Sleep Disorders Clinic, Stanford Medical Center

Mehran Farid-Moayer, MD is a member of the following medical societies: American Academy of Sleep Medicine, American Medical Association

Disclosure: Nothing to disclose.

Acknowledgements

The authors and editors of Medscape Reference gratefully acknowledge the contributions of previous coauthor, Sandra L Sessoms, MD, to the development and writing of this article.