Systemic lupus erythematosus natural history, complications and prognosis

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


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

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 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 ↓↓
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
Coronary artery disease ↑↑
Neurological Cognitive dysfunction
  • May be temporarily affected by multiple, transient metabolic and systemic processes
Psychosis ↑↑
Neuropathies ↑↑
Musculoskeletal Arthritis ↑↑↑↑
Osteonecrosis (Avascular necrosis)
Subcutaneous nodules
  • Associated with active disease and flare ups
Skin Cutaneous lupus erythematosus
  • 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


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]


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