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1. GIANT CELL ARTERITIS
( CRANIAL ARTERITIS/ TEMPORAL ARTERITIS)
HISTORY :-
HUTCHISON IN 1890 -- THROMBOTIC ARTERITIS
OF THE TEMPORALARTERY IN AGED INDIVIDUALS
FIRST H-P DESCRIPTION by HORTON IN 1932 --
UNDESCRIBED FORM OFARTERITIS OF TEMPORAL
VESSELS
JENNINGS IN 1938 -- FISRT RECOGNISED VISUAL
LOSS AS SIGNIFICANT COMPLICATION

2. DEFINITION:-
SYSTEMIC VASCULITIS THAT PREDOMINANTLY
INVOLVES THE MEDIUM SIZED EXTRACRANIAL
ARTERIES OF THE CAROTID CIRCULATION AND
SOMETIMES THE AORTAAND IT’S BRANCHES.
EPIDEMIOLOGY:-
AGE > 50yrs ESTIMATED ANNUAL INCIDENCE
ABOUT 15-30/100,000
AGE SPECIFIC PREVALENCE RATES
60yrs-69yrs = 33/100,000
>80yrs = 844/100,000
MALE : FEMALE = 3-4 : 1
MORE COMMON IN THE WHITES

3. PATHOGENESIS :-
UNKNOWN; ABNORMALITY IN THE ARTERIOLAR
ELASTICUM -> DISINTEGRATION OF THE INNER
ELASTIC MEMBRANE
ALTERNATE THEORY : INITIAL DISINTEGRATION
OF THE MUSCULAR LAYER OF MEDIA ->
FRAGMENTATION OF THE ELASTICUM AND
FORMATION OF GIANT CELLLS
HUMORALAND CELLULAR AUTOIMMUNITY
IMPLICATED.
MAY HAVE GENETIC PREDISPOSITION :
a) INCREASED FREQUENCY IN CERTAIN ETHNIC
GROUPS AND GEOGRAPHICALAREA
b) HLA-B8 & HLA-DR4 ANTIGEN ASSOCIATION
NOT DEFINITIVELY ESTABLISHED

4. CLINICAL FEATURES AND ASSOCIATIONS
SYSTEMIC FEATURES :-
MAY OCCUR WITHOUT OPHTHALMIC
MANIFESTATIONS
MAY PRECEDE ANY OPHTHALMIC
MANIFESTATION BY MANY WEEKS
HEADACHE - COMMONEST; UNILATERAL OR
BILATERAL; TEMPORAL, OCCIPITAL OR BOTH;
THROBBING OR SHOOTING; MAY HAMPER SLEEP
SCALP TENDERNESS
JAW CLAUDICATION - ISCHEMIA OF THE
MASSETER MUSCLES; VIRTUALLY PATHOGNOMONIC;
PAIN ON SPEAKING OR CHEWING
NON-SPECIFIC SYMPTOMS - NECK PAIN; WEIGHT
LOSS; NIGHT SWEATS; ANOREXIA; FEVER; MALAISE;
DEPRESSION, etc

5. SUPERFICIAL TEMPORALARTERITIS - TENDER
INFLAMED NODULAR ARTERIES; INITIALLY PULSATION
PRESENT BUT THE ARTERIES CANNOT BE FLATTENED:
LATER PULSATION ALSO LOST; MOST SEVERE CASES,
GANGRENE OF THE SCALP MAY OCCUR; SOMETIMES
THE SCALP VESSELS MAY APPEAR NORMAL BUT ON
BIOPSY SHOW FEATURES OF ARTERITIS.
ARTERITIS OF OTHER ARTERIES - ANEURYSMS,
VALVE INCOMPITENCE, STROKES, RENAL FAILURE,
CVA, etc
POLYMYALGIA RHEUMATICA - RELATIONSHIP
REMAINS UNCERTAIN; MAY BE ANOTHER END OF THE
SAME SPECTRUM; DIFFERENCE MORE QUANTITATIVE
THAN QUALITATIVE;

6. OCULAR FEATURES OF GCA

7. OPHTHALMIC FEATURES :-
VISION - SUDDEN, PAINLESS, PROFOUND LOSS;
MAY RANGE FROM FC TO NO PL COMMONLY; USUALLY
BILATERAL OR THE SECOND EYE MAY FOLLOW
WITHIN DAYS TO WEEKS
ANTERIOR SEGMENT ISCHEMIA - CONJUNCTIVAL
AND EPSCLERAL HYPEREMIA; CORNEAL EDEMA; LOW
IOP; ANTERIOR CHAMBER REACTION; IRIS RUBEOSIS;
RAPID CATARACT, etc
RAPD OR TAPD
DIPLOPIA - MUSCLE ISCHEMIA OR
OPHTHALMOPLEGIA FROM BRAIN STEM INFARCTION
FUNDUS - ALTITUDINAL DISC OEDEMA WITH
PALOR; SMALL FLAME SHAPED HGE EXTENDING
SHORT DISTANCE FROM THE DISC MARGIN;
ARTERIOLAR ATTENUATION; COTTON WOOL SPOTS;
CHOROIDAL INFARCTS, etc

8. VISUAL FIELD DEFECTS - ARCUATE SCOTOMAS
OR ALTITUDINAL HEMIANOPIA ( SUPERIOR OR
INFERIOR) OR NASAL PSEUDO-QUADRANTIC DEFECTS;
DENSE AND EASILY DETECTED BY CONFRONTATION
METHOD;
KESTEMBAUM EMPHASIZED THE VALUE OF LOCALISATION
OF THE VERTEX OF THE DEFECT : IF THE WEDGE ORIGINATES OR
POINTS TOWARDS THE BLIND SPOT, THE DISEASE LIES IN THE
NERVE HEAD OR JUST BEHIND IT.
CENTRAL SCOTOMAS ARE THE PREDOMINANT
DEFECTS
OLD AION - OPTIC ATRPHY ENSUES WITH LOSS OF
DISC TISSUE; MAY GIVE RISE TO CUPPING BUT RARELY
OF GLAUCOMATOUS SIZE; OPHTHALMIC CRITERIA OF
RETROSPECTIVE DIAGNOSIS POST-AION O.A.
ATTENUATION OF THE ARTERIES

9. DIAGNOSIS :-
ESR :- ELEVATED ESR IS SEEN COMMONLY;
MEDIAN WAS 96mm/ hr; RANGE WAS 50-132mm/hr;
VARIOUS STUDIES DONE SHOWING DIFFERENT
LEVELS; MILLER & GREEN - IN ADULTS, MAXIMAL
NORMAL ESR FOR ANY GIVEN AGE = AGE/2
ACCORDING TO GLASER, NORMAL UPPER LIMIT 35-
40mm/hr
CROSS REACTING PROTEIN (CRP) :- MAY BE
MORE SPECIFIC; CRP IS 100% SPECIFIC AND 83%
SENSITIVE IN MALES; 100% AND 79% IN FEMALES;
MEAN = 6.6mg/dl ( 0.5 - 34.7)
ELVATED SERUM ALKALINE PHOSPHATASE &
GAMMA- GLUTARYL TRANSFERASE
DECREASE IN CD8+ LYMPHOCYTES IN BLOOD

10. TEMPORAL ARTERY BIOPSY :-
USUALLY BIOPSIED BUT DIAGNOSIS CAN ALSO
BE MADE FROM FRONTALAND OCCIPITAL ARTERY
BIOPSY; LENGTH OF ATLEAST 1cm ( MAY BE UPTO 3 -
5cm); BILATERAL BIOPSY IMPROVES THE CHANCES OF
DIAGNOSIS; SEVERAL SECTIONS SHOULD BE
EXAMINED AS “SKIP LESIONS” ARE COMMON; SHOULD
BE DONE WITHIN 1 WEEK OF STEROID THERAPY AS
BIOPSY MAY TURN NEGETIVE;
BLOOD FLOW STUDIES :-
OCULAR PNEUMOTONOGRAPHY
PNEUMOPLETHYSMOGRAPHY
COLOUR DUPLEX
DOPLER ULTRASOUND

11. ACCORDING TO IOWA SERIES OF 363 PTS UNDERGOING
TEMPORALARTERY BIOPSY, THE ODDS OF POSITIVE
WAS INCREASED
9 TIMES BY THE PRESENCE JAW CLAUDICATION
3.4 TIMES BY NECK PAIN
3.2 TIMES BY CRP > 2.45mg/dl
AND ONLY 2 TIMES BY ESR 47 -107mm/hr

12. TREATMENT :-
IT IS SAID THAT, “ WHEN ARTERITIS IS SUSPECTED,
THERAPY SHOULD NOT BE DELAYED FOR THE RESULTS OF ESR,
CRP OR TEMPORALARTERY BIOPSY OR OTHER INVESTIGATION.”
THERAPY IS STEROIDS BUT DOSES VARY FROM
VARIOUS INSTITUTIONS;
JACOBEIC = ORAL PREDNISOLONE 60-120mg/DAY
INITIALLY AND THEN TAPERED
GLASER = 80- 100mg/DAY OR
i.v. METHYL PREDNISOLONE 250mg 6hrly
HIGH DOSE MAINTAINED FOR 4-8wks
THE DOSE IS THEN TAPERED WEEKLY BY 5mgs
UNTIL THE ESR COMES DOWN TO 40mm/hr;
Pt. SHOULD BE HOSPITALISED FOR
OBSERVATION OF ANY SIDE-EFFECTS OF STEROIDS
HEAD END SHOULD BE LOWERED

13. STEROID SIDE-EFFECTS LIKE MYALGIA AND
WEAKNESS SHOULD NOT MISTAKEN FOR
WORSENNING OF PMR;
ALTERNATIVES :-
METHOTREXATE(7.5 - 20mg/week) SHOWED NO
STEROID SPARING EFFECT
DAPSONE, CYCLOPHOSPHAMIDE, AZATHIOPRINE
IS UNDER REVIEW
OPTIC NERVE SHEATH FENESTRATION IS NOT
DONE ANY MORE

14. PROGNOSIS :-
IF UNTREATED, MAY LEAD TO BLINDNESS AND
EVEN, DEATH.
BILATERAL BLINDNESS OCCURS IN 9% ON
STEROID THERAPY;
VISION LOSS IN PERMANENT;
VARIOUS STUDIES HAVE BEEN DONE AND
STATISTICS VARY ABOUT RECOVERY WITH STEROIDS
AND PROTECTION OF SECOND EYE WITH STEROIDS.
PROGNOSIS POOR EVEN AFTER THERAPY WITH
STEROIDS ALTHOUGH FEW CASES OF GOOD
RECOVERY HAVE BEEN RECORDED.

15. INFREQUENLY ASSOCIATED CONDITIONS :-
D.M. PAPILLITIS
CATARACT EXTRACTION :-
1973 CAROLL REPORTED ION 4wks - 15m
AFTER CATARACT EXTRACTION. SECOND EYE UN-
AFFECTED IF NOT OPERATED ON.
COLLAGEN VASCULAR OR AUTO-IMMUNE
ARTERITIS:-
PTs IN YOUNGER AGE GROUPS WITHOUT
h/o DM OR HTN, PRESENTING WITH DISC EDEMAAND
ACUTE VISUAL LOSS; BILATERAL AND RECURRENT;
PROLONGED ANASTHESIA, LOW HEMATOCRIT
AND HYPOTENSION (POST ION)

16. CAROTID ARTERY DISEASE :-
THOUGH PREVIOSLY THOUGHT TO BE A
CAUSE, NOW-A-DAYS DOES NOT SEEM TO PLAY ANY
ROLE IN ION
OTHER COLLAGEN DISORDERS RARELY
ASSOCIATED WITH ION :-
LUPUS ARTERITIS
RHEUMATOID ARTERITIS
SJOGREN’S SYNDROME
POLYARTERITIS NODOSA
RELAPSING POLYCHONDRITIS
TAKAYASU’S ARTERITIS
OTHER CONDITIONS RARELY ASSOCIATED:-
ECLAMPSIA; PORPHYRIA;
PSEUDOXANTHOMA ELASTICUM; SICKLE CELL, etc

17. SIGNS OF DISC EDEMA:-
THE FIVE MECHANICAL SIGNS ARE AS FOLLOWS:
1.ANTERIOR EXTENSION OF OPTIC NERVE HEAD (3 D =
1 MM OF ELEVATION)
2.BLURRING OF THE OPTIC DISC MARGINS
3.FILLING IN OF THE PHYSIOLOGIC CUP
4.EDEMA OF THE PERIPAPILLARY NERVE FIBER LAYER
5.RETINAL OR CHOROIDAL FOLDS
THE FIVE VASCULAR SIGNS ARE AS FOLLOWS:
1.HYPEREMIA OF THE OPTIC DISC (THE
KESTENBAUM'S NUMBER MAY BE GREATER THAN 12)
2.VENOUS CONGESTION (VENOUS DILATATION AND
TORTUOSITY)
3.PERIPAPILLARY HEMORRHAGES
4.EXUDATES IN THE DISC OR PERIPAPILLARY AREA
5.NERVE FIBER LAYER INFARCTS

20. ACUTE DISC PALOR
WITH MILKY PALE
OEDEMA
CHOROIDAL
INFARCTS

21. TEMPORALARTERY
BIOPSY SHOWING GIANT
CELLS AND INTIMAL
THICKENING