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Ocular sarcoidosis
- 2. • The patient is a 46-year-old female with no significant ocular history who was referred for evaluation of persistent floaters in both eyes for four months. • She denies seeing flashing lights in either eye. She has no history of trauma to either eye. • She also denies decreased vision, eye pain, or eye redness. 2 ChiefComplaint Floaters in both eyes (OU) for four months..
- 3. • Past Ocular History: No history of ocular surgery. She wears reading glasses. • Medical History: The patient has a history of bipolar disorder and GERD. • Past Surgical History: Uterine cryotherapy for dysfunctional uterine bleeding, and surgical decompression of "trigger thumb“. • Medications: Wellbutrin, Risperdal, Lamictal, and Prevacid. 3 ChiefComplaint Floaters in both eyes (OU) for four months..
- 4. • Family History: The patient’s mother and grandmother have diabetes. • Social History: She denies alcohol use, but she has been smoking one pack of cigarettes every week for the last 6 months. • Review of Systems: Negative apart from the ocular symptoms noted above. 4 ChiefComplaint Floaters in both eyes (OU) for four months..
- 5. • VA, without correction: • Right eye (OD)--20/20-1 • Left eye (OS)--20/20-1 • IOP : 40 mmHg OD and 19 mmHg OS • Gonioscopy: Open bilaterally 360 degrees without peripheral anterior synechiae (PAS). No neovascularization of the angle. • Pupils: • OD: 4mm in dark, constricting to 3.5mm in light • OS: 3.5mm in dark, constricting to 2.5mm in light • No relative afferent pupillary defect (RAPD). • Ocular motility: Full, OU. 5 OcularExamination
- 6. • OD: Mild conjunctival hyperemia. Small granulomatous keratic precipitates in the inferonasal aspect of cornea OD . 5-10 WBC per HPF and 1+ flare in anterior chamber OD. Nodular irregularity of the iris at approximately 3 o’clock with surrounding dilated blood vessels and thickening of iris stroma. • OS: Normal 6 Slit Lamp Examination
- 7. 7 Granulomatous keratic precipitates (KP) are seen in the inferonasal cornea of the right eye.
- 8. 8 Iris nodule with surrounding dilated blood vessels and adjacent pupillary irregularity.
- 9. OU: Normal discs with increased cupping of right disc as compared to the left. No pallor or edema, OU. Macula, vasculature, and peripheral retina were normal, OU. 9 Dilated fundus examination
- 11. Non-Infectious • Idiopathic • Fuchs' heterochromic iridocyclitis • Glaucomatocyclitic crisis • Sarcoidosis • Vogt- Koyanagi -Harada syndrome • Reiter syndrome • Ankylosing spondylitis Infectious • Lyme disease • Tuberculosis • Syphilis Theclinicalperspective 11
- 12. RPR Non-reactive ANA < 1:40 ACE 113 U/L (normal: 8-52 U/L) . Chest X-ray Bilateral hilar lymphadenopathy 12 Backtothecase... Prior to the patient’s follow-up visit, the results of the tests returned:
- 13. 13 Chest X-ray revealing bilateral hilar lymphadenopathy, consistent with the diagnosis of sarcoidosis.
- 14. The patient needs an ocular antihypertensive and a topical steroid to control her intraocular inflammation. We chose to prescribe Timolol for her IOP and Prednisolone 1% for the inflammation. 14 What medications does the patient need?
- 15. • VA without correction: 20/20 OD and 20/20 -1 OS. • IOP : 12 OD and 14 OS • Pupils: Unchanged • Slit Lamp Exam: Few very small follicles in the nasal lower palpebral conjunctiva, OS and in the temporal upper palpebral conjunctiva OS. • There were no KPs in either eye. • The anterior chambers were quiet in both eyes. The iris nodule in the right eye had disappeared. • The remainder of the slit lamp examination was normal in both eyes. 15 One-week follow-up visit...
- 16. 16 Few small follicles in the inferior palpebral conjunctiva of the right eye. None of these have the classic granulomatous appearance that can be seen in patients with sarcoidosis.
- 17. • While the ocular, laboratory and radiographic findings are all suggestive of sarcoidosis, they are not diagnostic. • The definitive diagnosis of sarcoidosis can be made by the combination of suggestive clinical findings combined with a tissue biopsy demonstrating non- caseating granulomas (Rothova, 2000). 17 Should we obtain a conjunctival biopsy?
- 18. 18 Pathology of conjunctival biopsy shown here demonstrates several non- caseating granulomas. Special stains for organisms (PAS with diastase and Ziehl-Neelsen acid fast stain) were negative. This is highly suggestive of sarcoidosis.
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- 20. 20 Sarcoidosis a multisystem chronic inflammation causing multifocal non- caseating granulomas
- 21. Declining Market Lorem ipsum dolor sit amet, consectetur adipiscing elit. Financial Lorem ipsum dolor sit amet, consectetur adipiscing elit. Trust Lorem ipsum dolor sit amet, consectetur adipiscing elit. Cost Lorem ipsum dolor sit amet, consectetur adipiscing elit. Margins Lorem ipsum dolor sit amet, consectetur adipiscing elit. 21
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- 23. 23 Ocular inflammation occurs in 25–70% of sarcoid patients depending on ethnicity. Granulomatous anterior uveitis is the most common manifestation.
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- 36. 1. Cataract 2. Glaucoma 3. PAS 4. Band keratopathy 5. Vitreous hemorrhage 6. Maculopathy (CMO, ERM, CNV) 7. RD and phthisis. 36 Complications are those typically seen in idiopathic uveitis, including:
- 37. CXR showing (BHL) Chest radiography is abnormal in 90%. Negative tuberculin skin test in a BCG- vaccinated patient or in a patient having had a positive tuberculin skin test previously. Elevated serum ACE levels and/or elevated serum lysozyme Investigation 37
- 38. Abnormal liver enzyme tests. Chest CT scan in patients with a negative chest X-ray result. Investigation 38
- 39. • Fibreoptic bronchoscopy with biopsy; histopathological confirmation of sarcoidosis is almost always required before starting treatment. • Thoracic endosonography 39 Investigation
- 40. • Calcium and vitamin D levels may be abnormal depending on disease pattern and level of activity. • Hypercalciuria is common. • Pulmonary function testing. • Bronchoalveolar lavage fluid (BALF) shows characteristic changes; CD4/CD8 T cell ratios are a key indicator. • Induced sputum analysis correlates strongly with BALF, and is a noninvasive technique. 40 Investigation
- 41. FourdiagnosticlevelsweredefinedbytheIWOSforocularsarcoidosis: 41 Definite ocular sarcoidosis: biopsy-supported diagnosis in the presence of a compatible uveitis. Presumed ocular sarcoidosis: biopsy not done but chest X-ray shows BHL with a compatible uveitis. Probable ocular sarcoidosis: biopsy not done, no BHL on chest X- ray but >3/7 of the intraocular signs above and >2/5 positive laboratory tests. Possible ocular sarcoidosis: lung biopsy negative but >4/7 signs and >2/5 positive laboratory tests.
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- 43. • Treatment of anterior and intermediate uveitis is approached in a stepwise fashion as for idiopathic inflammation. • Posterior uveitis generally requires systemic steroids and occasionally immunosuppressive agents such as methotrexate, azathioprine, ciclosporin and TNF inhibitors (e.g. adalimumab). 43 Treatment
Editor's Notes
- The goal was to rule out the more serious diseases first. Many of the other diagnoses (Fuchs’, glaucomatocyclitic crisis, for example) could be made primarily by exclusion. Therefore, the decision was made to order ANA (screen for autoimmune dysfunction), ACE (screen for sarcoid), RPR (screen for syphilis) and a chest Xray (screen for TB/sarcoid). We elected not to test for the HLA-B27 diseases (Reiter, Ankylosing spondylitis, psoriatic arthritis) in part because they were lower on the differential and because the patient had no associated clinical findings.
- The granulomatous uveitis combined with the elevated ACE and abnormal chest Xray strongly suggest (but do not confirm) the diagnosis of sarcoidosis.
- The decision was made to perform a conjunctival biopsy of the left eye. A 6 mm strip of inferior palpebral conjunctiva of the left eye was removed. The procedure was done in the minor procedure room under local anesthesia. The pathology of the specimen revealed "non-caseating granulomas, consistent with the diagnosis of sarcoid.
- Sarcoidosis is a chronic disorder of unknown cause, manifesting with non-caseating granulomatous inflammatory foci. It can affect essentially any organ system, but the lungs and lymph nodes are the most commonly involved. It more frequently (10 : 1) affects patients of black than white ethnicity but is more common in colder climates. It is one of the most common systemic associations of uveitis.
- Presentation. Respiratory symptoms (cough, shortness of breath on exertion) and constitutional symptoms (malaise, arthralgia) each occur in about 50% of patients. Löfgren syndrome is an acute presentation carrying a very good prognosis, characterized by the triad of erythema nodosum (see below), bilateral hilar lymphadenopathy (Fig. 11.25A) on chest X-ray, and polyarthralgia, usually seen in women. A minority of patients are asymptomatic (incidentally abnormal chest X-ray). Diagnosis may be made as the result of investigation of extrapulmonary inflammation such as uveitis. • Lung disease ranges from mild parenchymal infiltration to severe pulmonary fibrosis. • Skin lesions are seen in about 25% of patients and can include erythema nodosum (tender erythematous plaques typically involving the shins – Fig. 11.25B), lupus pernio (indurated violaceous lesions involving exposed parts of the body such as the nose, cheeks, fingers and ears – Fig. 11.25C) and granulomatous papules or macules. • Neurological disease is rare; meningitis and cranial nerve palsies may occur. Pituitary involvement can lead to hormonal abnormalities. Fig. 11.25 Sarcoidosis. (A) Bilateral hilar lymphadenopathy; (B) erythema nodosum; (C) lupus pernio (Courtesy of MA Mir, from Atlas of Clinical Diagnosis, Saunders 2003 – fig. C) A Cardiac involvement is relatively uncommon (5% clinically), but is critically important as it may lead to arrhythmia and sudden death. • Lymphadenopathy. Enlargement of superficial nodes is sometimes the initial clinical manifestation.
- Blindness can occur if not adequately managed. AAU typically affects patients with acute-onset sarcoidosis. CAU, typically granulomatous, tends to affect older patients with chronic pulmonary disease.
- The International Workshop on Ocular Sarcoidosis (IWOS), reporting in 2009, identified seven key signs in the diagnosis of intraocular sarcoidosis:
- 1. ‘Mutton fat’ KPs and/or small granulomatous KPs and/
- 2. Trabecular meshwork (TM) nodules and/or tent-shaped PAS.
- 3. Vitreous opacities: snowballs and/or ‘strings of pearls’.
- Choroidal and retinal involvement in sarcoidosis. (A) Small choroidal granulomata; (B) same eye as (A) showing lesion with a punched-out appearance; (C) confluent choroidal infiltration; (D) multifocal choroiditis; (E) multiple small retinal granulomata 4-Multiple chorioretinal peripheral lesions (active and/or atrophic). Multiple small pale-yellow infiltrates, sometimes with a punched-out appearance are the commonest; they are often most numerous inferiorly. Multiple large confluent infiltrates are less common Multifocal choroiditis carries a guarded visual prognosis even after resolution of activity, as a result of secondary choroidal neovascularization associated with macular or peripapillary chorioretinal scarring. Retinal granulomas may also occur, seen as discrete small yellow– white lesions.
- Fig. 11.28 Periphlebitis in sarcoidosis. (A) ‘Candle wax drippings’; (B) occlusive periphlebitis and disc oedema 5- Nodular and/or segmental periphlebitis (± ‘candle wax drippings ’) and/or retinal macroaneurysm in an inflamed eye. Periphlebitis appears as yellowish or grey-white perivenous sheathing. Perivenous exudates referred to as ‘candle wax drippings’ (en taches de bougie) are typical of severe sarcoid periphlebitis. Occlusive periphlebitis is uncommon, but peripheral retinal neovascularization may develop secondary to retinal capillary dropout. In black patients it may be mistaken for proliferative sickle-cell retinopathy.
- Involvement of the optic nerve head in sarcoidosis – granulomata and periphlebitis 6- Optic disc nodule(s)/granuloma(s) and/or solitary choroidal nodule. 7. Bilaterality. Solitary choroidal nodules are less common than multiple lesions in sarcoidosis. Focal optic nerve granulomas do not usually affect vision. Persistent disc oedema is a frequent finding in patients with retinal or vitreous involvement, and papilloedema due to CNS involvement may occur in the absence of other ocular manifestations.
- Conjunctival nodules resembling those of follicular conjunctivitis, Lacrimal gland infiltration Dry eye Eyelid skin nodules Orbital and scleral lesions.
- Figure 1. Both eyes (A, right; B, left) showed multiple lumpy subconjunctival masses infiltrating from the bulbar conjunctiva 360° around the limbus to the fornix. There was also diffuse hyperemia. The cornea was unaffected, and the rest of the ocular examination was normal.
- In addition to the acquisition of histopathological evidence, IWOS judged the following five investigations to be of significant value in the diagnosis of ocular sarcoidosis in patients having a compatible uveitis: A tuberculin skin test is negative in most sarcoid patients; a strongly positive reaction to one tuberculin unit makes a diagnosis of sarcoidosis highly unlikely.
- HR-CT scanning is of considerably greater value than standard resolution imaging.
- the lung is a common site from which to establish this in the presence of clinical or investigational evidence of pulmonary disease, though a more easily accessible superficial lesion should be chosen if available. (endobronchial or esophageal) with needle aspiration has been shown in a large trial to be a more sensitive technique than bronchoscopic biopsy. Miscellaneous biopsy sites include superficial lymph nodes or skin lesions, conjunctival nodules and lacrimal glands (up to 75% of enlarged glands are positive). If the eye is involved, vitreous biopsy is very useful (e.g. CD4/CD8 ratio). Other imaging modalities: include MRI cardiac and CNS imaging. PET scanning and occasionally whole-body gallium scanning.
- Corticosteroids have conventionally been the major treatment modality in ocular and systemic sarcoidosis, though alternative immunosuppressives are being used more commonly, particularly as steroid-sparing agents and in refractory disease. Treatment should be initiated aggressively to prevent sight-threatening complications. Peripheral retinal neovascularization can be treated with scatter photocoagulation to ischaemic areas demonstrated by FA. Cystoid macular oedema may respond to a topical NSAID. Cataract and glaucoma may require treatment; inflammation should be suppressed prior to surgery, preferably for at least 3 m