How sarcoidosis can affect the eye

1. AhmedMohamedEweidah
Presented by:
Faculty of Medicine, Alexandria University, Egypt

2. Alexandria, Egypt

3. • A 37 y/o AAF saying “I have cysts in the upper orbit of both eyes for 2
months and I would like to have them removed”.
Chief Complaint

4. Over the last 2 months, there have been swellings of upper outer portion
on the both orbits. She also notes that the cyst on the right eye is making
her right eye red with blurry vision and soreness. She also notes she is a
little light sensitive in sunlight. Patient denies diplopia, floaters, flashes
of light.
History of Present Illness

5.  Past Ocular History: unremarkable
 Medical History: HTN and anemia
 Medications: 1) amlodipine besylate 10mg, levrofloxacin and ciprofloxacin
2) ocular: tobramycin bid OU
 Family History: No family history of glaucoma, macular degeneration,
blindness or known ocular diseases.
 daughter had a cyst on side of her eye removed at age 1-2
 Social History: Does not use tobacco or drink alcohol.
 Allergies: NKDA
 Review of Systems: bilateral parotid gland enlargement and Rt submandibular
salivary gland enlargement. No symptoms or signs of facial nerve palsy. Denies
fever, chills, n/v, SOB, chest pain, rash, weakness, numbness .

6. Visual acuity (VA) with correction:
 Right eye (OD): 20/40-, pinhole to 20/25
 Left eye (OS): 20/20
Manifest Refraction
 OD -2.25 + 1.25 x 160 VA 20/30
 OS -1.25 sphere x 165 VA 20/25
Intraocular pressure (applanation): OD 15 mmHg, OS 16 mmHg
Visual fields: Full to confrontation (OU)
Motility: Full OU
Ocular Exam

7.  External:
- Orbital lacrimal gland approx 10 x 6mm RT with mild prolapse and approx. 12x 10mm with moderate
prolapse LT
- palpebral lacrimal gland prolapse bilaterally with flipping of the lid
- Hertel base 102: 22/22
Lids & lashes: normal OU
Conjunctiva & sclera: tri injection (OD) Clear and quiet (OS)
Cornea:
 OD: moderate pigmentary KPs
 OS: clear
Anterior chamber: 1+ WBC (OD), 1-2 WBCs post dilation (OS)
Iris and pupil: 30 degrees inferior synechia (OD), round/reactive (OS)
Lens: Pigmentary clumping on anterior capsule(OD), clear OS
Slit Lamp Exam

8. Vitreous: anterior vitreous reaction (OD) , Vitreous rare cell (OS)
Disc: Normal, cup-to-disc ratio 0.3 OU with temporal white lesion extending off border (OS)
Vessels: mild toutsiousity no sheathing (OU)
Macula: flat OU with focal pigmentary changes (OS)
Periphery: white chorioretinal changes no RT/RD (OD), peripheral whitening and vitreous clumping
noted but no RT/RD (OS).
 Optos :
OD: white chorioretinal changes.
OS: white peripheral retinal changes with vitreous clumping.
 OCT :
OD: 106 NL
OS: 100 NL
Dilated Fundus Exam

9.  Bilateral Lacrimal Gland enlargement.
 Bilateral Parotid and Submandibular Gland Enlargement.
 Anterior iritis with vitreous pigmentary clumping (OD).
Assessment

10.  Sarcoidosis
 Wegener granulomatosis
 Tuberculosis
 Syphilis
 NSOI
 Thyroid disease
 Lymphoma
 SS
D.D.

11.  Order Lab tests ( ACE, lysozyme, CBC)
 Order imaging ( CT head and nesk)
 Schedule for a lacrimal gland biopsy likely LT (larger size) and if needed RT
with frozen section. ( what about the size of the gland). Risks and benefits
discussed including but not limited to scar, lacrimal gland prolapse, infection.
 Prednisolone Acetate OD every 1-2 h for the first week for anterior uveitis.
 HA OD TID
 Return after one week for follow up.
Plan
C/W Sarcoidosis

12. Patient states she has been having difficulty talking and chewing since she started
the PF, not using Atropine. She is still a little light sensitive in sunlight and
soreness.
On January 12th (Pre-operative Evaluation)

13. Visual acuity (VA) with correction:
 (OD): 20/30-, pinhole to 20/25
 (OS): 20/40, pinhole to 20/30
Intraocular pressure (applanation): OD 11 mmHg, OS 12 mmHg
 OD: 1-2+ cell ,mild pigment on endothelium; synechiae broken
 OS: occ cell; mild pigment on endothelium, no synechiae
Orbicularis 4+ RT/2+ LT
Oralis 3-4+ RT/2-3+LT

14.  ACE : 135 U/L
 Lyzozyme: 7 ug/ml
 Hb: 10.5 ( Microcytic hypochromic anemia)
 Bilateral lacrimal glands enlargement.
 Bilateral parotid glands enlargement.
 Rt submandibular gland enlargement.
Lab results
CT results

15.  Biopsy: Soft tissue Lt lacrimal gland biopsy.
 Result: Lacrimal gland with granulomatous inflammation, focally
necrotizing. Special stains with GMS, AFB and FITE are negative for
fungal elements and acid-fast bacilli respectively.
Lacrimal gland biopsy Results

16. Kyoto, Japan

17. Sarcoidosis is a multisystem non-caseating granulomatous disorder of unknown
etiology with protean systemic and ocular manifestations. Although intrathoracic
manifestations are most common (90%), other organs frequently involved include
the lymph nodes, skin, eyes, CNS, bones and joints, liver, and heart. Ocular
involvement may be seen in up to 50% of patients with systemic disease, with
uveitis being the most frequent manifestation. In most large series, sarcoidosis
accounts for up to 10% of all cases of uveitis
Sarcoidosis

18.  The highest prevalence seen in the northern European countries (40 cases
per 100,000 people)
 In United States the disease is up to 20 times more prevalent among
African Americans than whites.
 Both sexes are affected, with a slight female predominance
 Onset usually occurs usually between the ages of 20 and 50 years
Epidemiology

19. • The recently completed ACCESS project suggests that specific occupations and
workplace exposures associated with microbe-rich environments may modestly
increase the risk of developing
• Molecular studies of tissue specimens of patients with sarcoidosis provide
evidence that suggests that mycobacterial and, less convincingly,
propionibacterial organisms may be important etiologic factors.
• A genetic predisposition for the development of the disease is suggested by the
increased expression of class I and II HLA molecules, especially HLA-DRB1,
in patients with biopsy-confirmed sarcoidosis. Familial clustering is observed,
with siblings of patients having a fivefold increased risk of developing the
disease.
Risk Factors

20. Figure 1: Microscopy of the biopsy specimens (H&E) showing non-
caseating granuloma formation characterized by epithelioid histiocytes,
multinucleated giant cells and lymphocytes.

21. Figure 2: Asteroid bodies are stellate inclusions with
numerous rays radiating from a central core that are present
in the cytoplasm of giant cells by H&E stain

22. Figure 3: Schaumann bodies are large concentric calcifications that
form within the cytoplasm of giant cells in sarcoidosis by H&E stain.

23. Neuro-ophthalmic manifestations
 Cranial nerve palsies ---- diplopia and ptosis.
 Optic nerve involvement occurring in 5-38% of patients with neurosarcoid.
Nonspecific optic nerve head swelling (papillitis), direct invasion of the optic disc
or nerve, or papilledema due to increased intracranial pressure may be present.
 Examination of the optic nerve head may reveal edema, vascular engorgement,
nerve head elevation, hemorrhage, or optic nerve pallor or frank atrophy.
Retrobulbar optic nerve lesions may masquerade as optic nerve glioma or
meningioma.
Ocular Manifestations

24. • In the conjunctiva, granulomatous nodules are the most common lesions. Keratoconjunctivitis
sicca presumably results from infiltration of the lacrimal and minor lacrimal glands or from
chronic inflammation of the lacrimal excretory ductules.
• In the cornea, interstitial keratitis rarely may occur. Band keratopathy is infrequent, and scleritis
is rare. Cataract is a frequent complication of uveitis and/or the corticosteroids used in
treatment of uveitis.
• Anterior uveitis is the most common ocular manifestation of sarcoidosis, sings
include granulomatous or mutton-fat keratic precipitates, iris nodules, posterior synechiae, and
peripheral anterior synechiae.
 Glaucoma also may occur. Either or both secondary open-angle and secondary angle-closure
glaucoma mechanisms may be seen.
 Löfgren syndrome is characterized by erythema nodosum, bilateral hilar adenopathy, febrile
polyarthralgias and anterior uveitis.
Anterior Segment Disease

25. • Heerfordt syndrome (uveoparotid fever) is characterized by uveitis,
parotitis and fever.

26.  Posterior findings occur in 25-30% of patients with sarcoidosis.
 Vitreous cavity may show cellular infiltrates, opacities, haze, syneresis, posterior vitreous
detachment, and hemorrhages. Grayish green ̶ white opacities (or snowballs) may be seen in the
anterior inferior vitreous. Vitreous hemorrhage is rare RNV.
 Periphlebitis is not uncommon which is limited to the retinal veins, tends to be segmental, and
involves the small branch veins. En taches de bougie (candle-wax drippings). CRVO is rare
with subsequent retinal neovascularization and vitreous hemorrhrage.
 Cystoid macular edema may occur.
 Focal and discrete subretinal mottling
 Pars plana exudates
Posterior Segment Disease

27. Approximately 20% of patients with ophthalmic findings of sarcoid have soft tissue
involvement of the orbit or lacrimal gland. Lacrimal gland involvement manifests as
a painless, bilateral, palpable swelling of the gland. Moderate to severe keratitis
sicca may result.
Orbital Disease

28. 1) ACE assay (73% sensitive and 83% specific) N: 8-52 U/L
2) Lyzozyme ( N: 9-17ug/ml)
3) Ca++ ( in blood and urine)
4) Vitreous Fluid Analysis In patients with vitreous inflammation. A recent study by Kojima et al
showed that a CD4/CD8 ratio of vitreous-infiltrating lymphocytes greater than 3.5 provided a
diagnosis of ocular sarcoidosis with a sensitivity of 100% and a specificity of 96.3%
5) Tuberculin skin test
6) RPR
7) FTA-Abs
8) ANCA screen
Diagnosis – Laboratory Tests

29. 1. Chest X-ray --- bilateral hilar lymphadenopathy +/- parenchymal involvement
2. HRCT
3. Gallium 67- citrate scan --- panda sign
4. Pulmonary function tests.
5. MRI in neurosarcoid
Diagnosis – Imaging Studies

30. Typically, the most accessible site with the lowest associated morbidity is chosen,
such as a palpable lymph node. Conjunctival biopsies and lacrimal gland biopsies
can also be performed.
Diagnosis – Biopsy

31. • Corticosteroids are the mainstay of treatment. Topical corticosteroids, depot periocular
injections, and oral corticosteroids may be used.
• Anterior uveitis responds well to topical corticosteroids (the treatment of choice for this
condition) and cycloplegia. Pred Forte 1% (prednisolone acetate) is the criterion standard.
• 1 drop every 1-2 hours while awake for 1 week.
• 1 drop 4 times daily for 1 week.
• 1 drop 3 times daily for 1 week.
• 1 drop 2 times daily for 1 week.
• 1 drop daily for 1 week.
• Discontinue if anterior chamber cells and flare are eliminated.
Treatment

32.  Factors favoring the oral route of administration for uveitis include optic nerve
involvement; intermediate uveitis, posterior uveitis, and panuveitis; bilateral
disease; and coexisting threshold systemic disease.
 Orbital sarcoidosis usually requires oral corticosteroids, but retrobulbar injections
of corticosteroids may be helpful.

33.  In cases of steroid failures or intolerance, methotrexate, cyclosporine A and
infliximab can be used.
 If uveitis-associated choroidal/retinal neovascularization is present,
intravitrealinjection of anti VEGF agents may help.
Corticosteroid Sparing Agents

34. Cataract surgery may be performed when the intraocular inflammation is
absolutely controlled. Vitrectomy may be required for severe vitreous opacification.
If secondary glaucoma is unresponsive to maximal medical therapy, patients may
require either trabeculectomy or glaucoma drainage device implant procedures.
Surgical Treatment

35. • https://www.flickr.com/photos/pulmonary_pathology/6151512527/i
n/set-72157627642508464/
• Eyerounds.ord
• Eyewiki.aao.org
• www.nejm.org
References