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The mainstay of therapy for systemic lupus erythematosus (SLE) is to control disease activity and prevent organ damage. The treatment choice for systemic lupus erythematosus (SLE) varies based on the severity of the disease and symptoms. Generally, all the patients with any type of SLE manifestation should be treated with [[hydroxychloroquine]] regardless of the level of their disease. Other [[Pharmacology|pharmacologic]] medical therapies for SLE include [[glucocorticoids]] like oral [[prednisone]] or [[Intravenous therapy|intravenous]] [[methylprednisolone]], [[Non-steroidal anti-inflammatory drug|NSAIDs]] like [[celecoxib]], and [[immunosuppressive therapy]] with [[mycophenolate]], [[cyclophosphamide]], or [[rituximab]], particularly in severe cases. Cutaneous lupus erythematosus (CLE), if presented separately without any other system involvement, can be treated with [[Topical steroid|topical corticosteroids]]. Other organ-related complications of SLE should be treated separately.
The mainstay of therapy for systemic lupus erythematosus (SLE) is to control disease activity and prevent organ damage. The treatment choice for systemic lupus erythematosus (SLE) varies based on the severity of the disease and symptoms. Generally, all the patients with any type of SLE manifestation should be treated with [[hydroxychloroquine]] regardless of the level of their disease. Other [[Pharmacology|pharmacologic]] medical therapies for SLE include [[glucocorticoids]] like oral [[prednisone]] or [[Intravenous therapy|intravenous]] [[methylprednisolone]], [[Non-steroidal anti-inflammatory drug|NSAIDs]] like [[celecoxib]], and [[immunosuppressive therapy]] with [[mycophenolate]], [[cyclophosphamide]], or [[rituximab]], particularly in severe cases. Cutaneous lupus erythematosus (CLE), if presented separately without any other system involvement, can be treated with [[Topical steroid|topical corticosteroids]]. Other organ-related complications of SLE should be treated separately.
==Medical Therapy==
==Medical Therapy==
Treatment goals in systemic lupus erythematosus (SLE):
Treatment goals in systemic lupus erythematosus (SLE) include:
* Ensure long-term survival
* Ensure long-term survival
* Achieve the lowest possible disease activity
* Achieve the lowest possible disease activity
Line 15: Line 15:


===== General treatment =====
===== General treatment =====
* [[Hydroxychloroquine]] : 200 to 400 mg daily as a single daily dose or in 2 divided doses
* [[Hydroxychloroquine]]: 200 to 400 mg daily as a single daily dose or in 2 divided doses
** Generally all the patients with any type of SLE manifestation should be treated with [[hydroxychloroquine]] despite the level of their disease.
** Generally, all the patients with any type of SLE manifestation should be treated with [[hydroxychloroquine]] regardless of the level of their disease
The treatment choice for systemic lupus erythematosus (SLE) is varied based on the severity of the disease and symptoms:
The treatment choice for systemic lupus erythematosus (SLE) is varied based on the severity of the disease and symptoms:
* Severe cases are defined as presentation of the disease with life threatening complications and multiple (more than 2) organ involvements.
* Severe cases are defined as presentation of the disease with life threatening complications and multiple (more than 2) organ involvements.
Line 52: Line 52:
==== Raynaud phenomenon treatment<ref name="pmid3691593">{{cite journal |vauthors=Challenor VF, Waller DG, Francis DA, Francis JL, Mani R, Roath S |title=Nisoldipine in primary Raynaud's phenomenon |journal=Eur. J. Clin. Pharmacol. |volume=33 |issue=1 |pages=27–30 |year=1987 |pmid=3691593 |doi= |url=}}</ref> ====
==== Raynaud phenomenon treatment<ref name="pmid3691593">{{cite journal |vauthors=Challenor VF, Waller DG, Francis DA, Francis JL, Mani R, Roath S |title=Nisoldipine in primary Raynaud's phenomenon |journal=Eur. J. Clin. Pharmacol. |volume=33 |issue=1 |pages=27–30 |year=1987 |pmid=3691593 |doi= |url=}}</ref> ====
* Preferred regimen (1): [[Calcium channel blocker]] ([[nifedipine]]) 10 to 30 mg 3 times daily  
* Preferred regimen (1): [[Calcium channel blocker]] ([[nifedipine]]) 10 to 30 mg 3 times daily  
* Preferred regimen (2): Antiplatelet therapy with low-dose [[aspirin]] (75 or 81 mg/day) in all patients with secondary [[raynaud phenomenon]]
* Preferred regimen (2): Antiplatelet therapy with low-dose [[aspirin]] (75 or 81 mg/day) in all patients with secondary [[Raynaud phenomenon]]
* Alternative regimen (1): [[Phosphodiesterase inhibitors|Phosphodiesterase (PDE) inhibitor]] ([[sildenafil]]) 20 mg once or twice daily
* Alternative regimen (1): [[Phosphodiesterase inhibitors|Phosphodiesterase (PDE) inhibitor]] ([[sildenafil]]) 20 mg once or twice daily
** Inadequate response to a [[CCB]]
** Inadequate response to a [[CCB]]

Revision as of 18:43, 2 August 2017

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Raviteja Guddeti, M.B.B.S. [2]

Overview

The mainstay of therapy for systemic lupus erythematosus (SLE) is to control disease activity and prevent organ damage. The treatment choice for systemic lupus erythematosus (SLE) varies based on the severity of the disease and symptoms. Generally, all the patients with any type of SLE manifestation should be treated with hydroxychloroquine regardless of the level of their disease. Other pharmacologic medical therapies for SLE include glucocorticoids like oral prednisone or intravenous methylprednisolone, NSAIDs like celecoxib, and immunosuppressive therapy with mycophenolate, cyclophosphamide, or rituximab, particularly in severe cases. Cutaneous lupus erythematosus (CLE), if presented separately without any other system involvement, can be treated with topical corticosteroids. Other organ-related complications of SLE should be treated separately.

Medical Therapy

Treatment goals in systemic lupus erythematosus (SLE) include:

  • Ensure long-term survival
  • Achieve the lowest possible disease activity
  • Prevent organ damage
  • Minimize drug toxicity
  • Improve quality of life
General treatment
  • Hydroxychloroquine: 200 to 400 mg daily as a single daily dose or in 2 divided doses
    • Generally, all the patients with any type of SLE manifestation should be treated with hydroxychloroquine regardless of the level of their disease

The treatment choice for systemic lupus erythematosus (SLE) is varied based on the severity of the disease and symptoms:

  • Severe cases are defined as presentation of the disease with life threatening complications and multiple (more than 2) organ involvements.
  • Mild cases are defined as disease pattern in which body just makes a small attack on just one or two organs.
  • Moderate cases are defined as more than 2 organ involvement during disease flares with low grade of involvement and complications or one or two organ involvement with more extensive involvements.

Severe disease

  • Preferred regimen (1): Hydroxychloroquine (oral): 200 to 400 mg daily as a single daily dose or in 2 divided doses AND methylprednisolone as intravenous "pulse"; 0.5 to 1 g/day for three days in acutely ill patients, or 1 to 2 mg/kg/day in more stable patients
    • Alternative regimen(1): Hydroxychloroquine (oral): 200 to 400 mg daily as a single daily dose or in 2 divided doses AND prednisone oral; 40-60 mg/day
  • Alternative regimen (2): Mycophenolate
    • For induction: 1 g twice daily for 6 months in combination with a glucocorticoid
    • For maintenance: 0.5-3 g daily or 1 g twice daily
  • Alternative regimen (3): Cyclophosphamide IV: 500 mg once every 2 weeks for 6 doses or 500 to 1,000 mg/m2 once every month for 6 doses or 500 to 1,000 mg/m2 every month for 6 months, then every 3 months for a total of at least 2.5 years
  • Alternative regimen (4): Rituximab IV: 375 mg/m2 once weekly for 4 doses or 1,000 mg (flat dose) on days 0 and 15 or 500 to 1,000 mg on days 1 and 15

Less Severe (mild and moderate) disease

  • Preferred regimen (1): Hydroxychloroquine (oral): 200 to 400 mg daily as a single daily dose or in 2 divided doses
  • Preferred regimen (2): Prednisone (oral) low doses of 10 mg/d or less for a short term therapy
    • For milder SLE
    • For treatment of cutaneous and musculoskeletal symptoms not responding to other therapies
    • It should be tapered once hydroxychloroquine has taken effect
  • Alternative regimen (1): Azathioprine (oral) initial 2 mg/kg/day; may reduce to 1.5 mg/kg/day after 1 month
    • Can be used to control symptoms
  • Alternative regimen (2): Methotrexate (oral) initial therapy with 7.5 mg once weekly; may increase by 2.5 mg increments weekly
    • Can be used to control symptoms

Other organ specific treatments

Fever management[1][2]

Raynaud phenomenon treatment[3]

Chronic pain management[5]
  • Moderate pain should be treated with mild prescription opiates such as:
  • Moderate to severe chronic pain should be treated with stronger opioids such as:
    • Preferred regimen (1): Hydrocodone: Single doses >40 mg or >60 mg with a total daily dose ≥80 mg
    • Preferred regimen (2): Oxycodone: 5 to 15 mg every 4 to 6 hours as needed
    • Alternative regimen (1): MS Contin: Opioid naive patients can have 5 to 10 mg every 4 hours; usual dosage range between 5 to 15 mg every 4 hours
      • Higher initial doses in patients with prior opioid exposure
    • Alternative regimen (2): Methadone: Maximum initial dose 30 mg
    • Alternative regimen (3): Fentanyl Duragesic Transdermal patch: A convenient treatment option for lupus chronic pain. It has a long lasting effect as well
Cutaneous lupus erythematosus[6][7][8][9][10]
Lupus nephritis treatment[11][12][13]
  • Severe active disease: 
    • Preferred regimen: Glucocorticoid therapy is initiated with intravenous pulse methylprednisolone (250 mg to 1000 mg given over 30 minutes daily for three days) to induce a rapid immunosuppressive effect, followed by conventional doses  
    • Alternative regimen: Conventional doses of oral glucocorticoids (eg, 0.5 to 1 mg/kg per day of prednisone) without a pulse.
      • Oral prednisolone at a dose of 60 mg/day, tapered every two weeks by 10 mg/day until 40 mg/day is reached, then tapered by 5 mg/day until 10 mg/day is reached 
Considerations[13]

References

  1. Jordan N, D'Cruz D (2016). "Current and emerging treatment options in the management of lupus". Immunotargets Ther. 5: 9–20. doi:10.2147/ITT.S40675. PMC 4970629. PMID 27529058.
  2. Cobo-Ibáñez T, Loza-Santamaría E, Pego-Reigosa JM, Marqués AO, Rúa-Figueroa I, Fernández-Nebro A, Cáliz Cáliz R, López Longo FJ, Muñoz-Fernández S (2014). "Efficacy and safety of rituximab in the treatment of non-renal systemic lupus erythematosus: a systematic review". Semin. Arthritis Rheum. 44 (2): 175–85. doi:10.1016/j.semarthrit.2014.04.002. PMID 24830791.
  3. Challenor VF, Waller DG, Francis DA, Francis JL, Mani R, Roath S (1987). "Nisoldipine in primary Raynaud's phenomenon". Eur. J. Clin. Pharmacol. 33 (1): 27–30. PMID 3691593.
  4. Pardy BJ, Hoare MC, Eastcott HH, Miles CC, Needham TN, Harbourne T, Ellis BW (1982). "Prostaglandin E1 in severe Raynaud's phenomenon". Surgery. 92 (6): 953–65. PMID 6890719.
  5. Di Franco M, Guzzo MP, Spinelli FR, Atzeni F, Sarzi-Puttini P, Conti F, Iannuccelli C (2014). "Pain and systemic lupus erythematosus". Reumatismo. 66 (1): 33–8. PMID 24938194.
  6. DOEGLAS HM (1964). "CHRONIC DISCOID LUPUS ERYTHEMATOSUS TREATED WITH TRIAMCINOLONE AND PLASTIC OCCLUSION". Dermatologica. 128: 384–6. PMID 14162995.
  7. Rothfield N, Sontheimer RD, Bernstein M (2006). "Lupus erythematosus: systemic and cutaneous manifestations". Clin. Dermatol. 24 (5): 348–62. doi:10.1016/j.clindermatol.2006.07.014. PMID 16966017.
  8. Sárdy M, Ruzicka T, Kuhn A (2009). "Topical calcineurin inhibitors in cutaneous lupus erythematosus". Arch. Dermatol. Res. 301 (1): 93–8. doi:10.1007/s00403-008-0894-6. PMID 18797893.
  9. BJORNBERG A, HELLGREN L (1963). "Treatment of chronic discoid lupus erythematosus with fluocinolone acetonide ointment". Br. J. Dermatol. 75: 156–60. PMID 13971327.
  10. Ritschel WA, Hammer GV, Thompson GA (1978). "Pharmacokinetics of antimalarials and proposals for dosage regimens". Int J Clin Pharmacol Biopharm. 16 (9): 395–401. PMID 359493.
  11. Schwartz N, Goilav B, Putterman C (2014). "The pathogenesis, diagnosis and treatment of lupus nephritis". Curr Opin Rheumatol. 26 (5): 502–9. doi:10.1097/BOR.0000000000000089. PMC 4221732. PMID 25014039.
  12. Hogan J, Appel GB (2013). "Update on the treatment of lupus nephritis". Curr. Opin. Nephrol. Hypertens. 22 (2): 224–30. doi:10.1097/MNH.0b013e32835d921c. PMID 23328501.
  13. 13.0 13.1 Tunnicliffe DJ, Singh-Grewal D, Kim S, Craig JC, Tong A (2015). "Diagnosis, Monitoring, and Treatment of Systemic Lupus Erythematosus: A Systematic Review of Clinical Practice Guidelines". Arthritis Care Res (Hoboken). 67 (10): 1440–52. doi:10.1002/acr.22591. PMID 25778500.

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