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Systemic lupus erythematosus natural history, complications and prognosis

Introduction

Overview

Overview

Common complications of systemic lupus erythematosus include dermatitis, nephritis, and arthritis. Prognosis is generally poor, and the 10-year mortality rate of patients with systemic lupus erythematosus is approximately 40%. The disease’s course can be divided into 4 subcategories: developmental phase, preclinical phase, clinical phase, and comorbid complication phase.

Natural History

Natural History

Systemic lupus erythematosus (SLE) is an autoimmune disease. Several flare-ups may happen in the course of the disease. SLE usually develops in the second and third decades of life, although it can present at any age. It usually starts with mild symptoms such as fatigue, fever, and skin rashes. Without treatment, the patient will develop symptoms of end organ damage, which will eventually lead to death in most patients.

The disease course can be divided into 4 subcategories based on the course of the disease:[1][2][3]

Developmental phase:
Preclinical phase:
Clinical phase:
Comorbidity-complication phase

The phase of damages due to complications of longstanding disease, immunosuppressive therapy, and end organ damage (irreversible damages and complications)

Factors associated with flare up: [4][3][5]

Complications

Complications

Complications that can develop as a result of prolonged activation of systemic lupus erythematosus or the SLE therapy are:[6][7][8][9][10][11][12][13][14][15][16]

(Up arrows represent higher frequencies and down arrows represent lower frequencies)

Organ Disease Description Frequency
Gastrointestinal Dysphagia ↑↑↑
Peptic ulcer disease
  • Due to:
    • The disease itself
    • The adverse effect of SLE treatment
↑
Intestinal pseudo-obstruction
  • May lead to mechanical obstruction of the small or large bowel in the absence of an anatomic lesion
  • Obstructing the flow of intestinal contents
↓↓
Protein-losing enteropathy ↓↓
Hepatitis ↑
Acute pancreatitis
  • Occurs usually in the setting of active SLE
↓
Mesenteric vasculitis ↓↓
Acute cholecystitis ↓↓
Pulmonary Pleural disease ↑
Acute pneumonitis ↓↓
Pulmonary hemorrhage
  • Pulmonary alveolar hemorrhage:
↓↓
Pulmonary hypertension ↑
Thromboembolic disease ↑
Shrinking lung syndrome ↓↓
Cardiac Cardiomegaly ↑↑
Valvular disease ↑↑
Pericardial disease ↓
Myocarditis ↓
Coronary artery disease ↑↑
Neurological Cognitive dysfunction
  • May be temporarily affected by multiple, transient metabolic and systemic processes
↑
Stroke ↓
Seizures ↑
Psychosis ↑↑
Neuropathies ↑↑
Musculoskeletal Arthritis ↑↑↑↑
Osteonecrosis (Avascular necrosis) ↓
Subcutaneous nodules
  • Associated with active disease and flare ups
↑
Osteoporosis ↑
Skin Cutaneous lupus erythematosus ↑
Photosensitivity
  • Common theme for skin lesions associated with SLE
↑↑↑
Non-scarring alopecia
  • May occur at some point during the course of their disease
↑
Oral and nasal ulcers
  • Usually painless
↑↑
Discoid lesions ↑
Very rare disorders Malignancy ↓↓↓
Diabetes mellitus ↓
Prognosis

Prognosis

The prognosis of systemic lupus erythematosus ranges from a benign illness to an extremely rapid progressive disease that can lead to a fulminant organ failure and death. Without treatment, systemic lupus eryhtematosus will result in a very high mortality rate, with a report of higher than a 60% mortality rate during the mid-20th century. The presence of nephritis is associated with a particularly poor prognosis among patients with SLE; SLE is associated with a 10-year mortality of more than 50% among patients with nephritis. The increase in survival rate of patients and better prognosis may be due to increased disease recognition with more sensitive diagnostic tests, earlier diagnosis or treatment, the inclusion of milder cases, increasingly judicious therapy, and prompt treatment of complications. Although improvements in SLE diagnosis have led to better prognosis, the mortality rate among SLE patients is still 5 times higher than the normal population.[17][18][19][20]

Poor prognostic factors for SLE survival:[21]

Prognosis markers: [21]

Serum anti ds-DNA titres correlated with:
Antiphospholipid antibodies correlated with:

SLE in men compared to women: [22]

SLE in the elderly (>65) compared to middle age prevalency: [23]

References

References

  1. ↑ Iwata Y, Furuichi K, Kaneko S, Wada T (2011). “The role of cytokine in the lupus nephritis”. J. Biomed. Biotechnol. 2011: 594809. doi:10.1155/2011/594809. PMCΒ 3199078. PMIDΒ 22028590.
  2. ↑ Rahman A, Isenberg DA (2008). “Systemic lupus erythematosus”. N. Engl. J. Med. 358 (9): 929–39. doi:10.1056/NEJMra071297. PMIDΒ 18305268.
  3. ↑ 3.0 3.1 Deguchi Y, Kishimoto S (1991). “Tumour necrosis factor/cachectin plays a key role in autoimmune pulmonary inflammation in lupus-prone mice”. Clin. Exp. Immunol. 85 (3): 392–5. PMCΒ 1535595. PMIDΒ 1893619.
  4. ↑ Crow MK, Olferiev M, Kirou KA (2015). “Identification of Candidate Predictors of Lupus Flare”. Trans. Am. Clin. Climatol. Assoc. 126: 184–96. PMCΒ 4530671. PMIDΒ 26330673.
  5. ↑ JosiΔ‡ D, Hofmann W, Habermann R, Schulzke JD, Reutter W (1988). “Isolation of immunoglobulins and their use in immunoaffinity HPLC”. J. Clin. Chem. Clin. Biochem. 26 (9): 559–68. PMIDΒ 3199078.
  6. ↑ Gurevitz SL, Snyder JA, Wessel EK, Frey J, Williamson BA (2013). “Systemic lupus erythematosus: a review of the disease and treatment options”. Consult Pharm. 28 (2): 110–21. doi:10.4140/TCP.n.2013.110. PMIDΒ 23395811.
  7. ↑ Zubair A, Frieri M (2013). “Lupus nephritis: review of the literature”. Curr Allergy Asthma Rep. 13 (6): 580–6. doi:10.1007/s11882-013-0394-4. PMIDΒ 24234325.
  8. ↑ Torres A, Askari AD, Malemud CJ (2009). “Cardiovascular disease complications in systemic lupus erythematosus”. Biomark Med. 3 (3): 239–52. doi:10.2217/bmm.09.14. PMIDΒ 20477476.
  9. ↑ Cortes S, Chambers S, JerΓ³nimo A, Isenberg D (2008). “Diabetes mellitus complicating systemic lupus erythematosus – analysis of the UCL lupus cohort and review of the literature”. Lupus. 17 (11): 977–80. doi:10.1177/0961203308091539. PMIDΒ 18852220.
  10. ↑ Doria A, Canova M, Tonon M, Zen M, Rampudda E, Bassi N, Atzeni F, Zampieri S, Ghirardello A (2008). “Infections as triggers and complications of systemic lupus erythematosus”. Autoimmun Rev. 8 (1): 24–8. doi:10.1016/j.autrev.2008.07.019. PMIDΒ 18703174.
  11. ↑ Zizic TM, Classen JN, Stevens MB (1982). “Acute abdominal complications of systemic lupus erythematosus and polyarteritis nodosa”. Am. J. Med. 73 (4): 525–31. PMIDΒ 6127033.
  12. ↑ Cojocaru M, Cojocaru IM, Silosi I, Vrabie CD (2011). “Manifestations of systemic lupus erythematosus”. Maedica (Buchar). 6 (4): 330–6. PMCΒ 3391953. PMIDΒ 22879850.
  13. ↑ Clowse ME, Jamison M, Myers E, James AH (2008). “A national study of the complications of lupus in pregnancy”. Am. J. Obstet. Gynecol. 199 (2): 127.e1–6. doi:10.1016/j.ajog.2008.03.012. PMCΒ 2542836. PMIDΒ 18456233.
  14. ↑ Bhattacharyya S, Helfgott SM (2014). “Neurologic complications of systemic lupus erythematosus, sjΓΆgren syndrome, and rheumatoid arthritis”. Semin Neurol. 34 (4): 425–36. doi:10.1055/s-0034-1390391. PMIDΒ 25369438.
  15. ↑ Alves SC, Fasano S, Isenberg DA (2016). “Autoimmune gastrointestinal complications in patients with systemic lupus erythematosus: case series and literature review”. Lupus. 25 (14): 1509–1519. doi:10.1177/0961203316655210. PMIDΒ 27329649.
  16. ↑ Kang I, Park SH (2003). “Infectious complications in SLE after immunosuppressive therapies”. Curr Opin Rheumatol. 15 (5): 528–34. PMIDΒ 12960476.
  17. ↑ Ren Y, Feng X, Zou Y, Pan W, Wang X, Pan J, Zhang M, Tao J, Zhang Y, Tan K, Li J, Ding X, Qian X, Da Z, Wang M, Chen Z, Sun L (2014). “[Clinical features and prognosis of patients with lupus nephritis]”. Zhonghua Yi Xue Za Zhi (in Chinese). 94 (13): 973–6. PMIDΒ 24851681.
  18. ↑ Ugarte A, Ruiz-Irastorza G (2016). “SLE: the changing prognosis”. Lupus. 25 (12): 1285–7. doi:10.1177/0961203316652948. PMIDΒ 27307448.
  19. ↑ Matsuyama N, Morimoto S, Tokano Y, Amano H, Nozawa K, Isonuma H, Hashimoto H, Takasaki Y (2010). “The long-term prognosis of lupus nephritis patients treated with intravenous cyclophosphamide”. Intern. Med. 49 (9): 823–8. PMIDΒ 20453401.
  20. ↑ Sabio JM (2016). “[Systemic lupus erythematosus today]”. Med Clin (Barc) (in Spanish; Castilian). 146 (4): 160–2. doi:10.1016/j.medcli.2015.08.001. PMIDΒ 26434992.
  21. ↑ 21.0 21.1 Lisnevskaia L, Murphy G, Isenberg D (2014). “Systemic lupus erythematosus”. Lancet. 384 (9957): 1878–88. doi:10.1016/S0140-6736(14)60128-8. PMIDΒ 24881804.
  22. ↑ de Carvalho JF, do Nascimento AP, Testagrossa LA, Barros RT, BonfΓ‘ E (2010). “Male gender results in more severe lupus nephritis”. Rheumatol. Int. 30 (10): 1311–5. doi:10.1007/s00296-009-1151-9. PMIDΒ 19784840.
  23. ↑ Feng X, Zou Y, Pan W, Wang X, Wu M, Zhang M, Tao J, Zhang Y, Tan K, Li J, Chen Z, Ding X, Qian X, Da Z, Wang M, Sun L (2014). “Associations of clinical features and prognosis with age at disease onset in patients with systemic lupus erythematosus”. Lupus. 23 (3): 327–34. doi:10.1177/0961203313513508. PMIDΒ 24297642.

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