SLE
LUPUS (Systemic Lupus Erythematosus)
SLE is an autoimmune disease, affecting young people all over the world. As the name suggests systemic lupus erythematosus is a systemic disorder associated with a characteristic butterfly rash and multiple system involvement.
The disorder is usually seen in the second to fourth decade and affects women almost 6-7 times more than men. Though environmental triggers may play a role SLE is largely influenced by genetics with upto 50% of identical twins and 10% of first degree relatives affected by the disorder. Due to multiple genes and complicated polygenic inheritance, the HLA MHC human leukocyte antigen major histocompatibility complex is considered the most important genetic factor. Factors that can precipitate a flare up include infections, stress, sun exposure, pregnancy and surgery among many other stressors.
SLE is a chronic inflammatory disorder with flare ups or relapses and remissions, affecting multiple organ systems including the musculoskeletal system, skin, lungs, hematologic, renal, and even the nervous system. Symptoms consist of malaise, fatigue, fever, loss of appetite and weight changes. Muscle and joint aches, a butterfly rash over the cheeks, cold extremities (Raynaud’s phenomenon), GI ulcers, lung problems (inflammation of the pleura), heart problems ( chest pain due to inflammation of the pericardium or covering of the heart, pericarditis), confusion and seizures, dry eyes, enlarged lymph nodes and blood abnormalities including anemia, low platelet counts, etc, are other manifestations.
Diagnosis:
After a thorough history and clinical examination preferably by a rheumatologist to evaluate the above symptoms testing includes the following:
Blood tests for SLE with Antinuclear Ab or ANA testing (rarely ANA neg patients with high suspicion of lupus may need repeat ANA testing using indirect immunofluorescence using Hep 2 cells), double stranded DNA dsDNA. Less sensitive tests are also performed including Anti Smith, SSA, SSB Ab, (usually positive in Sjogrens syndrome), Anti RNP (seen in mixed connective tissue diseases MCTD), Anti-ribosomal proteins and RF( usually positive in Rheumatoid arthritis) are all positive in approx 25% of SLE patients. Cyclic citrullinated peptide CCP Ab helps in excluding Rheumatoid Arthritis RA patients. Scl 70 helps exclude systemic sclerosis. C3, C4 and CH50 complement levels are usually low. P and C ANCA help exclude other forms of vasculitis. CBC helps pick up anemia and leukopenia as well as thrombocytopenia( In contrast to still’s disease in which leukocytosis is usually seen). Urinanalysis is done to check for proteins or red cells in the urine to ascertain renal involvement.
Tests to exclude Hepatitis B and C and parvovirus 19 are performed. In addition inflammatory markers erythrocyte sedimentation rate ESR and C reactive protein or CRP are performed. CK levels are also helpful in diagnosing muscle breakdown.
Imaging is performed to confirm certain organ involvement.
CT abdomen and Chest helps ascertain pancreatic, renal, lung involvement
MRI for brain involvement in cases with neurologic symptoms
Renal Ultrasound for renal involvement.
Chest X ray
Hand X ray
Echocardiogram and Electrocardiogram to find cardiac abnormalities
Rarely organ specific biopsies help make the diagnosis, when it affects the skin, kidney, etc. Rarely lymph node biopsies may be required in patients with lymphadenopathy (kikuchis syndrome, Lymphoma).
There are many diseases as mentioned above that need to be considered before making a diagnosis of SLE including various forms of vasculitis ( wegeners, MCTD, Sjogrens and Systemic Sclerosis, etc), arthritis (rheumatoid arthritis, etc), transient viral illnesses( EBV infections, CMV, Hep B and C and parvovirus B19) and other rare disorders (stills, Kikuchis, myositis, MS and finally fibromyalgia)
Once the diagnosis is confirmed then treatment is started.
Treatment is usually supervised by a rheumatologist with frequent monitoring of lab and clinical parameters.
Usually for mild disease, a low dose of a steroid (prednisone- screening for latent TB prior to use is recommended) along with hydroxychloroquine (ophthalmic screening is required for side effects) is started. The steroid dose may be increased in moderately severe cases and Methotrexate or Azathioprine (Immuran) may be added. In more severe cases rituximab, belimumab (which target B cells) cellcept, cyclosporine, or cyclophosphamide (screen prior to use for latent TB, Hep B and C) is used in addition to high dose steroids with gradual taper. Monthly labs including CBC, Liver function tests and renal functions may often be required to monitor for side effects.
Tips for SLE
Avoid exposure to sun
Avoid stress if possible
Follow with a rheumatologist
Maintain an adequate Vit D level
Healthy diet
Health physical activity
Smoking cessation
