Systemic lupus erythematosus (SLE) is a complex autoimmune disease with multisystem involvement. It is also sometimes classified as a vasculitis.
There is an overall increased female predilection. In adults, women are affected 9-13 times more than males. In children, this ratio is reversed, and males are affected two to three times more often. While it can affect any age group, the peak age at onset around the 2nd to 4th decades.
The disease is sometimes classified according to early and late onset groups 8-9.
SLE can affect multiple components of the immune system, including the complement system, T-suppressor cells, and cytokine production. It can result in a generation of autoantibodies, which can circulate for many years prior to the development of clinical SLE. The disease tends to have a relapsing and remitting course.
SLE has a myriad of clinical features and radiographic presentations that are probably best discussed under individual subtopics:
- CNS manifestations of systemic lupus erythematosus (CNS lupus): neuropsychiatric events can occur in ~45% (range 14-75%) of cases 6,10
- renal manifestations of systemic lupus erythematosus
- gastrointestinal manifestations of systemic lupus erythematosus: there may be GI involvement in ~20% of cases 5
- thoracic manifestations of systemic lupus erythematosus
- cardiovascular manifestations of systemic lupus erythematosus
- musculoskeletal manifestations of systemic lupus erythematosus
Approximately 85% (range 70-98%) of patients test positive for anti-dsDNA antibodies 11, and ~20% of Caucasians and ~35% of Asians/blacks test positive for anti-Smith antibodies 12.
The diagnosis of SLE made be if four of eleven ACR (American College of Rheumatology) criteria are present, either serially or simultaneously 2. These criteria were initially published in 1982 but have been revised in 1997.
- malar rash: fixed, flat or raised, occurs over malar eminences and tends to spare the nasolabial folds.
- discoid rash: erythematous raised patches with adherent keratotic scaling and follicular plugging
- oral ulcers
- non-erosive arthritis: where there is involvement of two or more peripheral joints with tenderness, swelling or joint effusions; can occur in ~80% of cases 1
- pleuritis and/or pericarditis: present in ~35% (range 17-50%) of cases 1
- proteinuria: >0.5 grams/day
- haematological abnormality
- haemolytic anaemia or
- leukopenia: <4000/mm3 on more than two occasions
- lymphopenia: <1500/mm3 on more than two occasions
- thrombocytopenia: <100,000/mm3 without any precipitant medications
- neuropsychiatric manifestations
- seizures or psychotic events in the absence of any underlying precipitating drugs or metabolic abnormalities
- immune abnormality on serology
- anti-DNA antibodies or
- anti-Sm antibodies or
- antiphospholipid antibodies (requires certain criteria)
- 27-42% of SLE patient can have the anti-phospholipid antibody syndrome 1
- positive antinuclear antibodies
- 1. Lalani TA, Kanne JP, Hatfield GA et-al. Imaging findings in systemic lupus erythematosus. Radiographics. 24 (4): 1069-86. doi:10.1148/rg.244985082 - Pubmed citation
- 2. Goh YP, Naidoo P, Ngian GS. Imaging of systemic lupus erythematosus. Part I: CNS, cardiovascular, and thoracic manifestations. Clin Radiol. 2013;68 (2): 181-91. doi:10.1016/j.crad.2012.06.110 - Pubmed citation
- 3. Goh YP, Naidoo P, Ngian GS. Imaging of systemic lupus erythematosus. Part II: gastrointestinal, renal, and musculoskeletal manifestations. Clin Radiol. 2013;68 (2): 192-202. doi:10.1016/j.crad.2012.06.109 - Pubmed citation
- 4. Sanna G, Piga M, Terryberry JW et-al. Central nervous system involvement in systemic lupus erythematosus: cerebral imaging and serological profile in patients with and without overt neuropsychiatric manifestations. Lupus. 2000;9 (8): 573-83. Lupus (link) - Pubmed citation
- 5. Xu D, Yang H, Lai CC et-al. Clinical analysis of systemic lupus erythematosus with gastrointestinal manifestations. Lupus. 2010;19 (7): 866-9. doi:10.1177/0961203310365883 - Pubmed citation
- 6. Oku K, Atsumi T, Furukawa S et-al. Cerebral imaging by magnetic resonance imaging and single photon emission computed tomography in systemic lupus erythematosus with central nervous system involvement. Rheumatology (Oxford). 2003;42 (6): 773-7. doi:10.1093/rheumatology/keg216 - Pubmed citation
- 7. Lim MK, Suh CH, Kim HJ et-al. Systemic lupus erythematosus: brain MR imaging and single-voxel hydrogen 1 MR spectroscopy. Radiology. 2000;217 (1): 43-9. Radiology (full text) - Pubmed citation
- 8. Braunstein EM, Weissman BN, Sosman JL et-al. Radiologic findings in late-onset systemic lupus erythematosus. AJR Am J Roentgenol. 1983;140 (3): 587-9. doi:10.2214/ajr.140.3.587 - Pubmed citation
- 9. Lalani S, Pope J, De leon F et-al. Clinical features and prognosis of late-onset systemic lupus erythematosus: results from the 1000 faces of lupus study. J. Rheumatol. 2010;37 (1): 38-44. doi:10.3899/jrheum.080957 - Pubmed citation
- 10. Aisen AM, Gabrielsen TO, Mccune WJ. MR imaging of systemic lupus erythematosus involving the brain. AJR Am J Roentgenol. 1985;144 (5): 1027-31. doi:10.2214/ajr.144.5.1027 - Pubmed citation
- 11. Conti F, Ceccarelli F, Perricone C et-al. Systemic Lupus Erythematosus with and without Anti-dsDNA Antibodies: Analysis from a Large Monocentric Cohort. Mediators Inflamm. 06;2015: 328078. doi:10.1155/2015/328078 - Free text at pubmed - Pubmed citation
- 12. Systemic Lupus Erythematosus. Academic Press. ISBN:B00AXW76ZC. Read it at Google Books - Find it at Amazon