SYSTEMIC LUPUS ERYTHEMATOUS

         SYSTEMIC LUPUS ERYTHEMATOUS

What is Systemic Lupus Erythematous?

 It is an autoimmune disorder characterized by inflammation of blood vessels and connective tissue resulting in multi system involvement .The clinical manifestations are variable and the course unpredictable. Childhood SLE is usually more severe and has a poorer prognosis than adult SLE. the hallmark of SLE is the presence of antinuclear antibodies. The female predominance characteristic of adult SLE usually not apparent in young children.

   What can be the causes of it?

(i)Genetic factors 
(ii)Environmental agents
(iii)Infectious agents
(iv)Ultraviolet rays
(v)Dietary factor
(vi)Effect of drug or chemical agents

 SLE is predominately a disease of women with frequency in 1in 700 among women between ages of 20-64 anf female to male ratio of 9:1,so age and gender is also a factor for systemic lupus erythematous.

What is diagnosis criteria for it?

  1  Positive immunoserology(antibodies to dsDNA/smith nuclear antigen) 

 2 Positive findings of  antiphospholipid antibodies(IgG/IgM anticardiolipin)   

 3 Lupus anticoagulants 

4  Positive antinuclear antibody test

5 Veneral disesase research laboratory test(VDRL)

What can be the symptoms of this disease? 

The symptoms of SLE is can be checked by physical examination of  the patient as a lot of signs of it can be identified through it, for example:

(i)The malar rash which is virtually pathognomic of SLE, may not be apparent initially.It involves the cheek, bridge of nose and lower eyelids but spares the nasolabial folds .

(ii)Discoid lesions are rare in childhood onset SLE.

(iii)Oral ulcerations may involve the buccal mucosa or palate and characteristically painless
(iv)Some children may have frontal alopecia.
(v)Arthritis is usually mild and always non erosive.
(vi)Renal involvement is a dreaded complication of SLE and one the commonest causes of mortality in children.

•  Neurological features may include psychosis ,seizures and chorea .There may be no correlation between the severity of clinical involvement and findings on neuroimaging .

• Hematologic abnormalities include a Coombs' positive hemolytic anemia,leukopenia,lymphopenia and thrombocytopenia . In addition there may be coagulation abnormalities due to secondary antiphospholipid antibody syndrome .Cardiac manifestations may include pericarditis,myocarditis or verrucous(Liebman-sacks) endocarditis.                                               

 Serological diagnostic values in diagnosis of this disease

+Almost all patients with SLE have demonstrable antinuclear antibodies.Presence of anti-double stranded DNA antibodies is highly specific of SLE.The titers of these antibodies usually correlate with disease activity .

+Anti-histone antibodies are characteristic of drug-induced lupus(e.g following phenytoin,isoniazid,hydralazine) but in such cases anti dsDNA antibodies are usually absent and serum complement level is not decreased.

+Anti-ro antibodies are believed to play a role in the development of congenital heart block characteristic of neonatal lupus syndromes.These heart blocks are permanent.Anti-Sm antibodies are a marker for CNS lupus.

 What shall be the treatment and therapies for such disease?

• Glucocorticoids and hydroxychloroquine form the main stay of therapy of lupus .

• Prednisolone is started in doses of 1-2 mg/kg/day and gradually tapered over several months ,according to disease activity.

• Arthritis usually responds to NSAID's.

• Life threatening complications (such as nephritis,myocarditis) may warrant use of intravenous pulses of methlyprednisolone(30mg/kg/day)for 3-5days 

• Rituximab, monoclonal antibody to CD20 has also been found to be effective in such situations .

• Use of monthly pulses of IV cyclophosphamide has considerably improved the long term outcomes

• Infections must be treated aggressively with appropriate antimicrobial s and the steroid dose hiked up during such episodes .

                                                         -BY DR. RAVI RANJAN

                                                        -BY DR. TANYA SHARMA