Systemic lupus erythematosis SLE

Systemic connective tissue disease

Systemic lupus erythematosis (SLE )
Most common systemic connective tissue disease , characterized by a wide variety of clinical features and a diverse spectrum of auto antibodies production 90 % of affected individuals are female Age of affection 20-30 yrs

Etiology and pathogenesis

Wide spectrum of auto antibody production results from polyclonal B and T cell activation The trigger that lead to auto antibody production is unknown Environmental features that associates with flares of lupus increase oxidative stress and subsequent apoptosis , as exposure to sunlight and UV light , pregnancy and infection

Clinical Features

Raynaud’s phenomenon is a common presentation , associated with arthralgia or arthritisSecondary type ( occur after age of 30 yrs )Loss of normal loop pattern and capillary fall out with hemorrhage and dots observed by ophthalmoscope

Raynaud’s phenomenon

Clinical Features
Musculoskeletal featuresMigratory arthralgiaMild morning stiffness Tenosynovitis and small joint synovitisJoint deformities are rare , deformities that occur result from tendon inflammation and damage rather than from bone erosion (Jaccoud’s arthropathy )

Arthritis of PIP Js

(Jaccoud’s arthropathy )

Clinical Features

Muco cutaneous features Painful oral ulcers ( may scarring ) Diffuse non scarring alopecia Classic butter fly facial rash ( 20-30 %) raised , painful , or pruritic that spares the nasolabial fold

Oral ulcers

non scarring alopecia

Diffuse scarring alopecia

Classic butter fly facial rash

Clinical Features

Muco cutaneous features Subacute cutaneous lupus erythematosis (SCLE ) migratory rash , non scarring papulosequamous or annular Discoid lupus (hyperkeratosis and follicular plugging , may lead to scarring alopecia Periungual erythema , vasculitis and livedo reticularis

Discoid lupus

Discoid lupus

Discoid lupus in the back

vasculitis

Periungual erythema

vasculitis

Clinical Features

Renal features Affected in more than 50 % Pathlogical forms of lupus nephritis Normal Mesengeal nephritis Focal prolifrtive nephritis Diffuse prolifarative niphritis Membranous nephropathy

Clinical Features

Cardiopulmonary features Chest pain from pleurisy or pericarditisMyocarditis and sterile endocarditis ( Libman – Sacks endocarditis )Venous thrombo embolismLung fibrosis

Clinical Features

CNS features Fatigue , headache Visual hallucination Chorea , organic psychosis , transverse myelitis and lymphocytic meningitis


Clinical Features
Hematological features Neutropenia , lymphopenia thrombocytopenia , and hemolytic anemia Elevated ESR , normal CRP ( unless there is serositis or infection ) Other manifestations Fever weight loss and lymphadenopathy GI involvement is rare

Diagnosis

Revised American Rheumatism Association criteria for SLE : Malar rash Discoid lesion Photosensitivity Oral ulcers Arthritis Serositis Renal disorders : proteinurea >0.5 g/ day Cellular cast

Diagnosis

Neurological disorders Seizures Psychosis Hematological disorders Immunological disorders Anti- DNA antibodies Antibody to SM antigen Positive antiphospholpid antibodies ( anticardiolipin Ab , lupus anticoagulant ) Anti nuclear Ab (ANA Ab )

Diagnosis

Diagnosis depends on the presence of 4 of 11 criteria Patients who are ANA –ve are very unlikely to have SLE unless presence of extractable nuclear antigen (anti Ro ) which clinically presented with skin rash (SCLE ) , secondary Sjogren syndrome , delivered baby with congenital heart block

Management

Avoidance of sun and UV light NSAIDs and mild analgesics Hydroxycloroquine used in cutaneous and joint symptoms Short coarse steroid may be used for skin rash , synovitis and pleuropericarditis

Management

Acute or life threatening disease ( renal or cerebral ) requires high dose steroids (40-0 mg daily ) or IV methylprednisolone ( 500-1000 mg ) in combination with IV syclophosphamide Other immunosupressive drugs (azathioprine , MTX , tacrolimus and mycophenolate mofetil ) are used for severe but non – life threatening manefistation or as step – down therapy after cyclophosphomide

Management

Lupus patients with the anti phospholipid antibody syndrome who have had previous thrombosis will reqire life – long warfarin


Prognosis
5 year is greater than 90 % Early mortality ( within 5 years ) due to organ failure or sepsis Fivefold increased late mortality mainly due to premature cardiovascular disease