LUPUS ERYTHEMATOSUS AND MULTIPLE SCLEROSIS
INTRODUCTION
SLE and Multiple Sclerosis both being autoimmune conditions, still show lot of diversities in their clinical manifestations. SLE is now also termed as Connective Tissue Disease to avoid confusion with other related diseases. Whereas Multiple Sclerosis is more of an inflammatory disease affecting the Central Nervous System.
*Prevalence of SLE : 30 in 100000 in Caucasians to 200/100000 in Afro-Carribeans
*Prevalence of Multiple Sclerosis: 120 in 100000 (in UK)
*Gender Distribution : Both the diseases are commoner in women than in men
ETIOLOGY OF THE DISEASES
Predisposing factors of SLE:
- Genetic predisposition
- Cigarette smoking
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- Oestrogen pill consumption by post menopausal women
- UV rays exposure
- Exposure to silica dust
Predisposing factors of Multiple Sclerosis:
- Genetic predisposition ( very mild )
- Bacterial infection
- Viral infection ( mainly Ebstein Barr Virus)
- Reduced sun exposure
- Geographical influence : commoner along the northern and southernmost parts of the world
PATHOPHYSIOLOGY
Pathophysiology of SLE :
During cellular apoptosis, the intracellular antigens come over the cell surface which are otherwise hidden inside the cells, thus triggering a widespread response leading to massive production of autoantibodies from polyclonal B- cell and T- cell activation.
Pathophysiology of Multiple Sclerosis :
The inflammatory process begins with the access of activated T- lymphocytes into the Central Nervous System, crossing the blood-brain barrier. They come into contact with myelin- derived antigens present on the microglia, initiating clonal proliferation and cytokine release. Resulting in destruction of the oligodendrocyte-myelin unit by macrophages.
The inflammatory plaque commonly forms around the periventricular regions of the brain,optic nerves, and the subpial regions of spinal cord.
CLINICAL MANIFESTATIONS
Clinical manifestations of SLE :
- Raynaud’s phenomena : The most common presentation
- Musculoskeletal features : Symptoms resembling Rheumatoid Arthritis; migratory arthralgia, morning stiffness. Although deformities, as seen in RA are not present in this disease.
- Skin symptoms : a) Oral ulcers
b) Diffuse non scarring alopecia
c) Butterfly rash; occurring along the cheeks sparing the
nasolabial region
d) Subacute Cutaneous Lupus Erythematosus; occurring
as non scarring migratory rashes.
e) Discoid lupus lesions; hyperkeratotic, may lead to
alopecia, if present on scalp, due to plugging of the
follicles
f) Periungual erythema
g) Vasculitis
h) Livedo reticularis
- Renal symptoms : Proliferative glomerulonephritis; accompanied by
haematuria and proteinuria
- Cardiopulmonary features : a) Pericarditis, leading to chest pain.
b) Myocarditis
c) Libmann- Sacks endocarditis
d) Venous thromboembolism
e) Alveolitis
f) Lung fibrosis
- Central Nervous System Features : a) Fatigue
b) Headache
c) Poor concentration
d) Visual hallucinations
e) Chorea
f) Organic Psychosis
g) Lymphocytic meningitis
7) Haematological features : a) Haemolytic anaemia
b) Elevated ESR
c) Normal CRP ( in absence of any infection)
8) Constitutional features : a) Fever
b) Weight loss
c) Gastrointestinal symptoms (rare)
Clinical manifestation of Multiple Sclerosis
- Optic neuritis
- Loss of sensation in upper limbs
- Trigeminal neuralgia
- Lhermitte’s symptom
- Partial Brown Sequard syndrome
- Postural tremor
- Ataxia
INVESTIGATIONS
Investigation for SLE : 1) CRP raised
2) ESR raised
3) Leucopenia
4) ANA present in 98% patients
5) Clinical features
Investigation for Multiple Sclerosis : 1) Exclusions of other disorders
2) MRI of head - to visualise the involved areas
MANAGEMENT
Management of SLE : General- Avoid exposure to sun, use sunscreen of SPF 25-50
Medical- 1) For patient with mild intensity disease- NSAIDS
2) For skin and joint symptoms- Hydrochloroquine ( 200-
400 mg daily)
3) Mild or moderate diseases ( like rashes, synovitis) - Oral
corticosteroids
4) Serious diseases ( like renal and cerebral) – High dose of
corticosteroids like oral prednisolone 40-60mg daily or IV
methylprednisolone 500mg- 1gm in combination with
pulse IV cyclophosphamide
5) Other immunosuppressive drugs ( azathioprine,
methotrexate, cyclosporine, Tacrolimus, mycophenolate
mofetil ) are useful either alone or in combination with
corticosteroids for severe but non life threatening
Manifestations or as step down therapy after
cyclophosphamide.
Management of Multiple Sclerosis : Acute relapses : Pulses of high dose
methylprednisolone, either IV 1g
daily for 3 days or orally ( 500mg daily for 5 days)
Preventing relapses : Azathioprine. Subcutaneous or
IM interferon beta- 1a/b reduces number relapses by
some 30%, with small effect
on long term disability
Nursing care : Physiotherapy or occupational therapy
may bring improvement in disabled patients.
PROGNOSIS
Prognosis of SLE : 5yr survival rate in more than 90% patients; early mortality within 5yrs of diagnosis due to organ failure or sepsis
Prognosis of Multiple Sclerosis: Difficult to predict. 15% with one attack of demyelinating attack do not suffer anymore attacks; while those with relapsing and remitting have on average 1-2 relapses every 2yrs. Approximately 5% patient die within 5yrs of onset. Overall, after 10yrs about one-third of patients disabled to the point of needing help with walking,rising to about 50% after 15yr
REFERENCE
Davidson, S. S. (2006). Davidson's principles and practiced of medicine. Toronto: Churchill livingstone elsevier.
Morris W. Foster, C. E. (April,2003). A practice approach for identifying previously unsuspected enviromental contributors to systemic lupus erythematosus and other complex diseases. Environmental health perspectives, Vol.111, No.4.
Multiple sclerosis
Retrieved from : http://emedicine.medscape.com/article/1146199-overview Soyuer, F. (2006). Balance performance in three forms of multiple sclerosis . Neurological Research .
Systemic lupus erythematosus
Retrieved from : http://emedicine.medscape.com/article/332244-overview
