2. AUTO IMMUNITY
IMMUNE SYSTEM IS SELECTIVELY
UNRESPONSIVE AGAINST SELF ANTIGENS.
THIS STATE IS CALLED TOLERANCE.
IT IS SUGGESTED THAT THE SELF
ANTIGENS ARE SEQUESTRATED THEREBY
PREVENTING ACCESSS BY IMMUNE SYSTEM
3. AUTO IMMUNITY
• Individuals start mounting Immune Reactions
against Self Antigens.
• This Results from Dysregulation of Immune
System
Tolerance of the Immune System gets broken
down and they start reacting against self
antigens, which results in Autoimmunity.
4. SLE
• TYPICAL PROTOYPE OF AUTI-IMMUNE
DISEASE
• AETIOLOGY IS NOT CERTAIN.
• CAUSED BY INTERACTIONS BETWEEN
GENETIC, ENVIRONMENTAL AND
ENDOCRINE INFLUENCES
• SUCH INTERACTIONS RESULT IN THE
FORMATION OF MULTIPLE AUTO
ANTIBODIES
5. SLE
• ESSENTIALLY A DISEASE OF YOUNG
WOMEN OF CHILD BEARING AGE 19-29
• FEMALE TO MALE RATIO: 20 TO 1
• HENCE IT IS A DISEASE TO BE CONSIDERED
IN YOUNG WOMEN
6. • SLE involves Autoimmune Antibodies and Immune
Complex formation. The Immune complexes get deposited
in small vessels and cause occlusion of vessels and cause
ischemia and necrosis of areas supplied by the vessels. It
causes inflammation at the site of deposition and causes
increased permeability of the vessels and cause exudation
and edema.
• Involvement occurs in areas, which are rich in small
vessels, i.e.arterioles, capillaries and venules. Skin, Mucous
Membranes, Lungs, Eyes and Kidnies are the sites which are
commonly involved. Thus SLE is a Multi System Disease.
7. Because of the Multisystem involvement,
the symptoms are predominated by systemic
symptoms. i.e. Fatigue, Malaise, Fever,
Anemia, Loss of Appetite and weight Loss
Symptoms due to various system involvement
get added to the picture
8. SKIN MANIFESTATIONS
• Two Types of Skin Lesions occur:
• 1. Due to Photosensitivity. Rashes found in
areas of Sunlight Exposure. Face, Neck and
Hands.
• 2. Discoid Lesions. Found over Face and
Scalp. They are neither caused nor
aggrevated by sunlight exposure
9. PHOTOSENSITIVE RASHES
• Occurs over the face over the cheeks and
nose (Butterfly Rash)
• Also may occur over the ears, Upper Neck
(V Area) and extensor surface of forearms
• Lesions have the appearance of slightly
raised erythema
• Central clearing of the lesions present.
Edges are irregular
14. Discoid Lupus Lesions
• Well circumscribed, roughly circular, slightly
raised lesions.
• They have scaly surface. The edges are
hyperpigmented and erythematous. Centrally
they are depigmented and atrophic.
• Found over the Face, Scalp. Trunk and
extremities. They are disfiguring lesions.
25. PEARLS TO REMEMBER
• ALMOST ALL DERMATOLOGICAL CONDITIONS
PRESENTING WITH RASH AND ULCERATIONS
EXCEPT LUPUS SPARE THE ELBOWS AND
EYELIDS. HENCE RASHES INVOLVING THE
ELBOWS AND EYELIDS SHOULD SUGGEST A
DIAGNOSIS OF LUPUS
• MOST LUPUS SKIN LESIONS ARE CAUSED BY
OR AGGREVATED BY SUNLIGHT. HENCE IN
EVERY PHOTOSENSITIVE RASH, LUPUS SHOULD
BE STRONGLY CONSIDERED AS A POSSIBILITY
26. MUSCULO SKELETAL
MANIFESTATIONS
• JOINTS ARE NOT USUALLY INVOLED IN SLE.
JOINT DESTRUCTION SHOULD SUGGEST AN
ALTERNATIVE DIAGNOSIS.
• WHAT IS INVOLVED COMMONLY IS THE
TENDONS. TENDON EDEMA IS THE CAUSE
FOR JOINT STIFFNESS.
27. JACOUD’S DEFORMITY IN SLE
• STIFF TENDONS BY THEMSELVES CAUSE
JOINT DEFORMITIES (JOINTS ARE NOT
AFFECTED)
• THESE DEFORMITIES ARE REVERSIBLE SINCE
THE JOINT SPACES ARE NORMAL AND
THERE IS NO FUSION OF BONES
• SEVERE CONTRACTURES OF TENDONS MAY
OCCUR TO PRODUCE DISFIGURING
DEFORMITIES (JACOUD’S)
28. TENDON INVOLVEMENT
• OCCASIONALLY LARGER TENDONS LIKE
ACILLE’S TENDON MAY BE INVOLVED
WHICH MAY CAUSE RUPTURE OF THE
TENDON
• DIFFICULTY IN STRAIGHTENING THE
FINGERS DUE TO TENDON EDEMA CAN
OCCUR
29. BONE AND JOINT INVOLVEMENT
• INTERMITTENT POLYARTHRITIS USUALLY
INVOLVING HANDS, WRISTS AND KNEES.
• USUALLY MILD INVOLVEMENT.
• SEVERE PAIN IN A JOINT IS NOT A
FEATURE. SEVERE PERSISTING PAIN SHOULD
SUGGEST AVASCULAR NECROSIS.
• AVASCULAR NECROSIS USUALLY INVOLVES
THE FEMORAL HEAD
30. RENAL INVOLVEMENT
RENAL INVOLVEMENT IS ONE OF THE
SERIOUS MANIFESTATIONS.
PROTEINURIA IS THE COMMONEST
MANIFESTATION
MORE SERIOUS INVOLVEMENT WILL LEAD
ON TO NEPHROTIC SYNDROME,
HYPERTENSION AND RENAL FAILRE
REGULAR URINE ANALYSIS WILL DETECT
THE ONSET OF RENAL DISEASE.
31. NERVOUS SYSTEM INVOLVEMENT
NEUROPSYCHIATRIC MANIFESTATIONS, i.e.
PHOBIAS AND PSYCHOSIS ARE COMMON.
HEAD ACHE, MEMORY LOSS AND SEIZURE
EPISODES MAY ALSO OCCUR.
STROKE IS ANOTHER MANIFESTATION.
THUS SLE SHOULD BE CONSIDERED IN
EVERY YOUNG STROKE
32. NERVOUS SYSTEM INVOLVEMENT
ACUTE TRANSVERSE MYELITIS IS A RARE
MANIFESTATION
OTHER MANIFESTATIONS INCLUDE MULTIPLE
SCLEROSIS AND MYASTHENIC SYNDROMES
33. CARDIOVASCULAR INVOLVEMENT
• HEART. ALL THREE LAYERS OF THE HEART
ARE INVOLVED.
• PERICARDIUM. PERICARDITIS & EFFUSION
• MYOCARDIUM. MYOCARDITIS
• ENDOCARDIUM. FIBRINOUS OR LIBMAN-
SACHS ENDOCARDITIS
• HEART FAILURE, ARRYTHMIAS AND
EMBOLIC EVENTS WILL FOLLOW HEART
INVOLVEMENT
34. CARDIOVASCULAR INVOLVEMENT
• ENHANCED ATHEROGENESIS AND EARLY
ATHEROMA FORMATION ARE FEATURES.
THIS PREDISPOSES TO OCCURRENCE OF
MYOCARDIAL INFARCTION
• EARLY ATHEROSCLEROSIS AND KIDNEY
INVOLVEMENT LEADS TO HYPERTENSION
35. VASCULITIS
• SMALL VESSEL VASCULITIS LEADS ON TO
OCCURRENCE OF SMALL ISCHEMIC LESIONS IN
THE TIPS OF FINGERS AND TOES.
• OCCURRENCE OF VASCULITIC LESIONS OVER
THE ELBOW IS VERY SPECIFIC FOR SLE
• OTHER LESIONS DUE TO VASCULITIS ARE:
1. SPLINTER HAEMORRHAGES IN THE
NAIL BED
2. DIGITAL GANGRENE
36. LUNG MANIFESTATIONS
• PLEURISY WITH EFFUSION IS THE MOST
COMMON MANIFESTATION
• INTERSTITIAL INFLAMMATION LEADING ON
TO FIBROSIS
• INTRA-ALVEOLAR HEMORRHAGE
37. HAEMATOLOGIC MANIFESTATIONS
• ANEMIA. IRON DEFICIENCY ANEMIA IS
COMMON BECAUSE OF NSAID THERAPY.
NORMOCHRMIC NORMOCYTIC ANEMIA
OCCUR DUE TO CHRONIC INFLAMMATION
• LEUCOPENIA IS COMMON. IT IS ALWAYS
LYMPHOCYTOPENIA.
• THROMBOCYTOPENIA IS COMMON. COUNTS
LESS THAN 1 LAKH/C.MM ARE COMMON
• HEMOLYTIC ANEMIAS ALSO MAY OCCUR
38. OCULAR MANIFESTATIONS
• CONJUNCTIVITIS IS COMMON
• RETINAL VASCULITIS CAN OCCUR
• OPTIC NEURITIS IS ANOTHER FEARED COM-
PLICATION
• CATARACTS AND GLAUCOMA MAY OCCUR
AS COMPLICATIONS OF STEROID THERAPY
39. GASTROINTESTINAL FEATURES
• PERITONITIS IS A COMPLICATION. IT
CAUSES DIFFUSE ABDOMINAL PAIN AND
ASCITES
• PERFORATIONS AND BLEEDING MAY OCCUR
AS COMPLICATIONS OF STEROID THERAPY.
• ISCHEMIC BOWEL PAIN MAY OCCUR
SECONDARY TO VASCULITIS INVOLVING
VESSELS SUPPLYING THE INTESTINES.
40. LIVER
• INVOLVEMENT OF LIVER IS VERY RARE.
• THE TERM “LUPOID HEPATITIS” IS USED.
• THIS IS NOT CAUSED BY SLE.
• USUALLY CAUSED BY OTHER AUTO-IMMUNE
DISEASES SUCH AS HUGHE’S SYNDROME
42. DIAGNOSIS
• SUSPICION OF SLE IS THE MOST IMPORTANT
REQUIREMENT FOR DIAGNOSIS
• IT SHOULD BE SUSPECTED IN WOMEN IN THE 20
TO 30 AGE GROUP, WHEN ANY OF THE
FOLLOWING CLUES ARE FOUND
1. CLASSICAL BUTTERFLY RASH OVER
THE CHEEKS AND BRIDGE OF THE NOSE
2. SMALL RED SPOTS OR BLISTERS OVER
THE ELBOW
43. DIAGNOSIS
3.CONSTITUTIONAL SYMPTOMS LIKE FEVER,
WEIGHT LOSS, ACHES AND PAIN ALL
OVER THE BODY WHICH REMAIN
UNEXPLAINED.
4.NEUROPSYCHIATRIC SYMPTOMS LIKE
PHOBIAS AND PSYCHOSIS
5. SEIZURES AND STROKE
46. TREATMENT
• 1. AVOID SUNLIGHT/UV LIGHT EXPOSURE
• 2. VIT D SUPPLEMENTATION
• 3. HYDROXYCHLOROQUIN IS THE MAINSTAY
DRUG 200 TO 400 MG/DAY
• 4. STEROIDS CAN BE USED TO TREAT
ACUTE FLARE UPS AND SEROSAL
INVOLVEMENT. METHYL PREDNISOLONE IN
EMERGENCY AND ORAL PREDNISOLONE
FOR CONTINUATION
48. COMPLETE HEART BLOCK AND SLE
• THE MOST IMPORTANT YOU MUST
REMEMBER IS THAT:
CONGENITAL COMPLETE HEART BLOCK
OCCURS IN NEONATES AND CHILDREN
OF MOTHERS SUFFERING FROM SLE
