ocular sarcoidosis,sarcoidosis

1. OCULAR
SARCOIDOSIS
SIVATEJA CHALLA

2. • DEFINITION
• EPIDEMIOLOGY
• PATHOGENESIS
• ETIOLOGY
• HISTOPATHOLOGY
• CLINICAL FEATURES
• DIFFERENTILA DIAGONOSIS
• INVESTIGATIONS
• DIAGONOSTIC CRITERIA
• TREATMENT
• COMPLICATIONS
• COURSE AND OUTCOME

3. Definition
• Multisystem chronic inflammatory disorder
characterised by the presence of non caeseating
granulomas in affected tissues
• Main organs involved are lungs,lymph nodes, eyes,
skin, heart, liver,kidneys, muscles
• Ocular involvement was first observed by schumaker
in patient with nodular iritis in the year 1909

4. EPIDEMIOLOGY
• World wide distribution
• Prevance differs from region to region
• Overall incidence 6-10/ 1 lakh
• Japan 3.2/lakh and finland 28.2/lakh
• Age common is 20-40 yrs (bimodal 20 to 30y and 50-60y)
• Sex females>males
• Ocular incidence is 25 to 60%
• Earlier was disease of developed world,now d/t increased
diagnostic modalities seen in developing countries also

5. PATHOGENESIS
• Caused by antgenic stimulation
• Antigen activates a cascade of reactions in genetically
susceptible individuals
Antigen presented by APC
Activation of CD4+T lymphocytes and cytokines
compartmentalisation at site of inflammation sealing the
antigen L/T granuloma formation

6. ETIOLOGY
• ?BACTERIAL
• ?VIRAL
• ?ENVIRONMENTAL
• Mycobacterial- clinical radiological and histopath
similarities of TB and sarcoidosis implicates
mycobacterium as cause.
-studies demo M.TB DNA and RNA from
sarcoid tissues,but was not able to isolate M.TB on culture
- recently high frequency of M.TB catalase
peroxidase in sarcoid granulomas renewed interest
ANTIGEN STUDIES BUT CAUSE IS NOT
PROVED
Cont…..

7. ETIOLOGY
• Other agents
Proprionobacterium
Hepatitis C
Herpes
EBV
Helicobacter pyroli

8. HISTOPATHOLOGY
• HLADR 17 AND TNF play important role in disease severity
and prognosis
• But no gene has been demonstrated responsible for
sarcoidosis
• HPE classically shows typical NON CASEATING sarcoid
granulomas
centre—epitheloidcells or macrophages
surrounded by –lymphocytes and fibroblasts
• SARCOID and ASTEROID bodies are found

9. HISTOPATHOLOGY

10. CLINICAL FEATURES
• OCULAR FEATURES
• SYSTEMIC FEATURES
PRESENTATION
ACUTE ONSETLOFGRENS SYNDROME-erythema nodosum,
B/L hilar lymphadenopathy, arthritis
 HEERFORDTS SYNDROME-fever, uveitis,
parotid swelling, facial palsy
INSIDIOUS ONSET 5th decade with PUL involvement
resulting in cough,dyspnoea along with extra pulmonary
manifestations

11. OCULAR FEATURES
• Seen in 50% pts with systemic disease
• m/c manifestation is uveitis
• May be asymptomatic
• May present with blurred vision floaters, redness and
discomfort
• Ocular involvement is chronic and has insidious onset

12. Eye lids
• Small millet shaped to large nodules on eyelids
• Lesions are non tender and rarely ulcerate

13. Lacrimal gland and drianage system
• 7% pts with sarcoidosis
• 25% pts with ocular sarcoidosis
• Usually B/L
• Enlargement of lacrimal glands found
• Loss of functionality may lead to keratoconjunctivitis sicca
• NLD afected very rarely,dacrystenosis may occur
secondary to granulomatous inflammation
• May L/T epiphora and acute or chronic dacrocyctitis

14. Orbit
• Uncommon
• Only involves in 1% pts with ocular sarcoidosis
• Granulomatous infl of orbit may L/T U/L OR B/L proptosis
• Involvement of EOM very rare,may lead to painful external
ophthalmoplegia

15. Conjunctiva
• 4-13% cases of sarcoidosis
• 17-20% pts with ocular sarcoidosis
• 25% pts with sarcoid uveitis have conj changes
• M.C lesion is granuloma
• Seen as small round yellow nodules
• Usually present in infero palpebral conj and fornix
• May be single,may be U/L OR B/L
• In some cases cicatrial chnages and symblepheron can
occur

16. CONJUNCTIVA

17. Cornea
• M.C manifestation is band shaped keratopathy
• Also nummular keratitis may be seen,bilateral,not
characteristic
• May also seen as thickening and opacification of
descemets membrane and endothelium in inferior cornea
due to fibrous metaplsia of endothelial cells,may be of
chronic inflammation
• Presencce of inferior corneal opacification in patient with
chronic iridocyclitis should raise the suspicion of sarcoid

18. CORNEA

19. Iris
• Nodules in 11% pts with ocular sarcoidosis
• Koeppes and busaccas nodules
• Not characteristic

20. Anterior uveitis
• M.c manifestation
• Seen on 2/3rd cases
• Typical chronic B/L Non caseating granulomatous uveitis
• Acute or chronic
ACUTE ANTERIOR UVETIS
• Abrupt onset asso with pain redness and photophobia
• Blurred vision
• Usually U/L,exam reveals cells,flare and fine KPs
• Likely to occur at the onset of systemic disease
• Well responds to treatment
Cont….

21. Anterior uveitis
CHRONIC ANTERIOR UVEITIS
• Mutton fat KPs
• Iris nodules
• Trabecular meshwork nodules on gonio
• Tent shaped PAS on gonio
• May lead to glaucoma and cataract
• Mor edifficult to treat and has higher incidence of
complaications and worst prognosis

22. ANTERIOR UVEITIS

23. ANTERIOR UVEITIS

25. Intermediate uveitis
• Inflammation of vitreous,pars plana and peripheral retina
• Snow balls are seen
• Some times snow banking may be seen
• Least common form
• In sarcoidosis anterior uveitis > posterior uveitis >
intermediate uveitis

26. Posterior uveitis
• 25% patients with ocular sarcoidosis have posterior
segment involvement
• Although asso with ant uveitis 20% pts amy have isolated
post sement involvment
CHARACTERISTIC FINDINGS
• Candle wax dripping sign/taches de bouge,there is
periphlebitis with segmental cuffing with extensive
sheatind and perivenous infiltrate
• Capillary closure and ischemia may L/T Neo vas and VH
• Choroidal granulomas are seen,which resolve and form
RPE atrophy or scar formation
• Cystoid macular oedema

27. OTHER RARE COMPLICATIONS
• RETINAL DETACHMENTS
• OPTOCILIARY SHUNTS
• ARTERIAL MACRO ANEURYSMS
• major concern is posterior segment sarcoid
uvetis involvement associated with
neurological involvement in 27% cases,SO
careful fundus examination should be done

28. POSTERIOR UVEITIS

29. POSTERIOR UVEITIS

30. OPTIC NEUROPATHY
• Rare
• Feared complication,may lead to rapid vn loss
• Four types of optic nerve disease described
1.Papilloedema sec to severe raised ICP and CNS involvement
2.Infiltration of optic nerve by non caeseating granulomas
3.Retrobulbar neuritis
4.Glaucomatous optic atrophy secondary to raised IOP FOR
long periods

31. OPTIC NEUROPATHY

33. SYSTEMIC FEATURES
• Predominantly affects
1. Lungs 90%
2. Thoracic lymph nodes
3. Skin
4. CNS
5. ARTHROPATHY
6. Hepatic involvement
7. Renal invlvement
8. Cardiac involvement

34. Lungs and thoracic lymphnodes
• Usually asymptomatic and discovered on CXR
• Patient may also present with fever,malaise,fatigue,weight
loss,cough,dyspnoea,chest pain
SCADDING SCALE CXR and HRCT CHEST
• Stage 1 (a) –Bilateral hilar lymphadenopathy (55-90%)
• Stage 2 (b) –Pulmonary infiltrates with BHL(40-70%)
• Stage 3 (c) – Pulmonary infiltrate alone (10-20%)
• Stage 4 (d) – extensive pulmonary fibrosis (10%)

35. CXR

36. SKIN
• ERYTHEMA NODOSUM– tender erythematous plaques on
knees,shins,occasionally seen on thighs and forearms
• Granulomatous scattered plaques,nodules and papules
• LUPUS PERNIO-- indurated violaceous lesions involving
nose cheeks fingers and ears

37. SYSTEMIC FEATURES
• Neurological involvement is seen on 5-10% pts. M.C is U/L
7th nerve palsy,
• Arthropathy is typically symmetrical and all types of joints
involved
• Hepatic involvement subclinical and altered liver enzymes
may be present
• Renal involvement may lead to hyper calcemia,hyper
calciuria and increased risk for nephrocalcinosis and
nephrolithiasis
• Cardiac seen on <5%,conduction defects occur.be careful
In using beta blockers in these patients for glaucoma
• Peripheral lymphadenopathy in 30% pts

38. DIFFERENTIAL DIAGONOSIS
• Varied presentations and features it shares with other
diseases offer many oppurtunities for misdiagonosis
• So history, clinical corse and associated systemic sgns and
investigations proves clinical clues

39. DIFFERENTIAL DIAGONOSIS
GRANULOMATOUS UVEITIS Tb, Syphilis, leprosy, vkh,
toxoplasmosis, herpetic uveitis
ISOLATED IRIS NODULES Iris neoplasms, metastatic
carcinomas, leukemic infiltrates, seeding from
retinoblastoma
INTERMEDIATE UVEITIS LYMES disease,multiple
sclerosis,pars planitis
CHOROIDAL LESIONS tb, birdshot retinopathy, metastatic
tumours, large cell lymphomas, vkh, serpigrnous
choroidopathy, behcets disease , CMV retinitis, Sympathetic
ophthalmia, histoplasmosis

40. INVESTIGATIONS
• Diagonosis of exclusion
1.ICG csn be done for dx of occult choroidal lesions
2.TUBERCULIN TEST is depressed in pts with sarcoid d/t
cutaneous anergy and may be NEGATIVE
3.Serum ACE levels are elevated,if patient on ACE inhibitors
then do serum LYSOZYME levels,not characteristic
4.LIVER ENZYMES,included in diagonostic criteria for ocular
sarcoidosis (3* inc in ALP or 2* inc in sgot/sgpt)

41. INVESTIGATIONS
• RADIOLOGY  CXR,HRCT for hilar lymphadenopathy and
pul infiltrates,HRCT is more sensitive than CXR
• GALLIUM SCANNING
• BRONCHOALVELOR LAVAGE shows lymphocytois,altered
CD4/CD8 ratio, smears and culture can be made to r/o TB
• BIOPSY any clinically involved tissue can be
biopsied,usuallu lung parenchyma and hilar lymph nodes
are biopsied to look for non caseating granulomas
• HYPERCALCEMIA occurs in <10% pts,is of limited value

42. GALLIUM SCANNING

43. DIAGONOSTIC CRITERIA

45. TREATMENT
• No standardized therapy
• Main is corticosteroids
• If mild then topical corticosteroids
• If severe then start oral corticosteroids and taper
• If not responding then immunosuppresants like MTX,
mycophenolate, azathioprine, cyclosporine and
leflunomide
• If not controlled then anti TNF ALPHA agents like
etanecept, infliximab and alalimumab can be tried

46. COMPLICATIONS
1.CME IN 76% CASES
2.CATARACT 49%
3.GLAUCOMA 36 %
4.RETINAL ISCEMIA 16%
5.NEO VASCULARISATION IN 11%
CATARACT AND GLAUCOMA TO BE TREATED APPROPRIATELY
IVTA can be given for CME

47. COURSE AND OUTCOME
• Overall good prognosis
• Systemic features asso with poor outcome are
1.Diffuse CNS disease
2.Lupus pernio
3.Nephrocalcinosis
4.Late stages of pulmonary disease
5.Bone abnormalities
6.Hepatosplenomegaly
7.Cardiac involvement

48. THANK YOU