Ocular Manifestations of Systemic Disorders Practice Questions

Q: Angioid streaks are seen in which of the following conditions?

Acromegaly. **Explanation:** Angioid streaks are crack-like dehiscences in a thickened, calcified, and brittle **Bruch’s membrane**. While they appear as irregular, jagged, reddish-brown lines radiating from the peripapillary area, they are often markers of systemic pathology. **Why "All of the above" is the correct clinical context (and why Option D is the intended answer):** The question asks for conditions where angioid streaks are seen. In clinical practice and standard textbooks (like Kanski), angioid streaks are classically associated with the mnemonic **PEPSI**: * **P – Pseudoxanthoma Elasticum (PXE):** The most common association (Grönblad-Strandberg syndrome). * **E – Ehlers-Danlos Syndrome:** Due to connective tissue defects. * **P – Paget’s Disease of bone:** Seen in about 1-2% of cases due to bone metabolism changes. * **S – Sickle Cell Anemia:** And other hemoglobinopathies like Thalassemia. * **I – Idiopathic:** No systemic cause found. **Analysis of Options:** * **A & C (PXE and Paget’s):** These are classic, high-yield causes of angioid streaks. * **B (Acromegaly):** While much rarer than PXE, Acromegaly is a documented systemic association of angioid streaks due to alterations in connective tissue and growth hormone effects. * **D (All of the above):** Since A, B, and C are all recognized associations, "All of the above" is the most comprehensive and correct choice. **NEET-PG High-Yield Pearls:** 1. **Most common cause:** Pseudoxanthoma Elasticum (PXE). 2. **Vision Loss:** The primary cause of vision loss in these patients is **Choroidal Neovascularization (CNV)** occurring through the cracks in Bruch's membrane. 3. **Appearance:** They mimic blood vessels but lie deeper (sub-retinal) and do not follow the normal branching pattern of retinal vasculature. 4. **Mnemonic:** Remember **"PEPSI"** for your exams to quickly recall the systemic associations.

Q: Koeppe's nodules are seen in which of the following conditions?

Granulomatous uveitis. **Explanation:** **Koeppe’s nodules** are small, cellular aggregates found on the **pupillary margin** of the iris. They are a hallmark clinical sign of **Granulomatous Uveitis**. 1. **Why Granulomatous Uveitis is correct:** Granulomatous inflammation is characterized by the collection of epithelioid cells, lymphocytes, and multinucleated giant cells. In the eye, these inflammatory cells clump together to form nodules on the iris. Koeppe’s nodules are located at the pupillary border, whereas **Busacca’s nodules** (also seen in granulomatous uveitis) are located on the iris stroma away from the pupil. Common causes include Sarcoidosis, Tuberculosis, and Syphilis. 2. **Why the other options are incorrect:** * **Non-granulomatous uveitis:** This typically presents with a diffuse cellular infiltrate and fine Keratic Precipitates (KPs) rather than organized nodules. * **Choroiditis:** This is a form of posterior uveitis involving the choroid. While it can be granulomatous, iris nodules are specific to anterior segment involvement (iridocyclitis). * **Pars planitis:** This is a subset of intermediate uveitis characterized by "snowbanking" and "snowballs" in the vitreous, not iris nodules. **High-Yield Clinical Pearls for NEET-PG:** * **Koeppe’s nodules:** Pupillary border (Think: **K**oeppe = **K**orner/Edge). * **Busacca’s nodules:** Iris surface/stroma (Pathognomonic for granulomatous disease). * **Mutton-fat Keratic Precipitates (KPs):** Large, greasy-looking inflammatory deposits on the corneal endothelium, also diagnostic of granulomatous uveitis. * **Berlin’s Nodules:** Found in the angle of the anterior chamber (seen on gonioscopy).

Q: A chalazion is caused by the obstruction of which gland?

Meibomian gland. **Explanation:** A **Chalazion** is a chronic, non-infectious, granulomatous inflammation of the **Meibomian gland**. It occurs due to the obstruction of the gland's duct, leading to the retention of lipid secretions (sebum). This leakage of lipids into the surrounding tarsal plate triggers a sterile "foreign body" inflammatory reaction. **Analysis of Options:** * **A. Meibomian gland (Correct):** These are modified sebaceous glands located within the tarsal plate. Because they are embedded deep in the lid, a chalazion typically presents as a firm, painless nodule pointing towards the conjunctival side. * **B. Zeis's gland:** These are small sebaceous glands associated with the hair follicles of the eyelashes. Infection or inflammation of the Glands of Zeis (or Glands of Moll) results in a **Hordeolum Externum (Stye)**, which is acute, painful, and superficial, unlike a chalazion. * **C. Lacrimal gland:** This gland is responsible for the production of the aqueous layer of the tear film and is located in the superolateral orbit. Inflammation here is termed **Dacryoadenitis**, which presents with lateral upper lid swelling and a characteristic "S-shaped" ptosis. **High-Yield Clinical Pearls for NEET-PG:** * **Pathology:** Histologically, a chalazion shows a **lipogranuloma** (giant cells, epithelioid cells, and lymphocytes surrounding lipid spaces). * **Treatment:** Small ones may resolve spontaneously; larger ones require **Incision and Curettage (I&C)** via a vertical incision (to avoid damaging adjacent Meibomian glands). * **Red Flag:** Recurrent chalazion in the same location in elderly patients should be biopsied to rule out **Sebaceous Gland Carcinoma**.

Q: Which of the following is NOT a feature of Behcet's disease?

Granulomatous Uveitis. **Explanation:** Behcet’s disease is a chronic, multi-system inflammatory disorder characterized by a relapsing systemic vasculitis. **1. Why "Granulomatous Uveitis" is the correct answer:** Behcet’s disease typically presents with **Non-granulomatous uveitis**. The hallmark ocular finding is a **sterile transient hypopyon** (pus in the anterior chamber that shifts with head position). In contrast, granulomatous uveitis is characterized by large "mutton-fat" keratic precipitates and nodules on the iris, which are typical of conditions like Sarcoidosis or Tuberculosis, not Behcet’s. **2. Analysis of Incorrect Options:** * **Oral Ulcers:** These are the most common and usually the first sign of the disease. They are painful, recurrent, and aphthous-like. * **Erythema Multiforme (Incorrectly listed in options):** While Behcet's is classically associated with **Erythema Nodosum**, some classifications and older texts include various cutaneous vasculitic lesions. However, in the context of this question, the "non-granulomatous" nature of the uveitis is the definitive "False" feature compared to the others. * **HLA B5 Association:** There is a very strong genetic association between Behcet’s disease and **HLA-B5** (specifically the **HLA-B51** subtype), which is a high-yield fact for competitive exams. **Clinical Pearls for NEET-PG:** * **Classic Triad:** Relapsing oral ulcers, genital ulcers, and iridocyclitis (uveitis). * **Pathergy Test:** A unique diagnostic test where a sterile skin papule or pustule forms 24–48 hours after a needle prick. * **Ocular Complication:** It is a leading cause of blindness due to **obliterative retinal vasculitis** (Frosted branch angiitis). * **Treatment:** Systemic steroids and immunosuppressants (like Azathioprine or Cyclosporine) are mainstay therapies.

Q: Which of the following metabolic disorders is associated with the fundoscopic finding shown in the image?

Mitochondrial disorders. ***Mitochondrial disorders*** - **Salt-and-pepper pigmentary retinopathy** is a characteristic fundoscopic finding in mitochondrial disorders, particularly **Kearns-Sayre syndrome**. - These disorders affect retinal pigment epithelium function due to **mitochondrial dysfunction**, leading to scattered pigmentary changes throughout the fundus. *Galactosemia* - Primarily causes **oil-droplet cataracts** in the anterior segment, not posterior segment fundoscopic changes. - Fundoscopic examination is typically **normal** unless secondary complications develop from untreated disease. *Albinism* - Characterized by **hypopigmented fundus** with visible choroidal vessels and **foveal hypoplasia**. - Does not produce the salt-and-pepper pigmentary pattern seen in mitochondrial disorders. *GM1 Gangliosidosis* - Classic fundoscopic finding is a **cherry-red spot** in the macula due to ganglioside accumulation. - This presents as a well-defined red central area, distinctly different from diffuse pigmentary retinopathy.

Q: What is the most common ocular manifestation in rheumatoid arthritis?

Keratoconjunctivitis sicca. **Explanation:** **Keratoconjunctivitis sicca (KCS)**, or secondary Sjögren’s syndrome, is the **most common** ocular manifestation of Rheumatoid Arthritis (RA), occurring in approximately 15–25% of patients. It results from immune-mediated destruction of the lacrimal glands, leading to aqueous tear deficiency. Patients typically present with a foreign body sensation, burning, and dryness. **Analysis of Options:** * **B. Episcleritis:** This is the most common **inflammatory** complication of the sclera/episclera in RA, but its overall prevalence is lower than KCS. It is usually benign and self-limiting. * **A. Scleritis:** While RA is the most common systemic cause of scleritis (especially necrotizing scleritis), it is less frequent than both KCS and episcleritis. Its presence often indicates high systemic disease activity and a poor prognosis. * **D. Anterior Uveitis:** This is more characteristically associated with Seronegative Spondyloarthropathies (like Ankylosing Spondylitis) rather than Rheumatoid Arthritis. **High-Yield Clinical Pearls for NEET-PG:** * **Most common ocular finding:** Keratoconjunctivitis sicca. * **Most specific/serious ocular finding:** Necrotizing scleritis (Scleromalacia perforans), which occurs in long-standing RA and is characterized by painless thinning of the sclera without inflammation. * **Drug-induced toxicity:** Patients on Hydroxychloroquine (HCQ) for RA require baseline and periodic screening for **"Bull’s eye maculopathy."** * **Peripheral Ulcerative Keratitis (PUK):** A serious "melting" of the cornea that can occur in severe RA.

Q: The presence of Kayser-Fleischer rings is pathognomonic of which condition?

Wilson's disease. **Explanation:** **Wilson’s Disease (Hepatolenticular Degeneration)** is the correct answer. The **Kayser-Fleischer (KF) ring** is a pathognomonic sign caused by the deposition of **copper** in the **Descemet’s membrane** of the cornea. It typically appears as a golden-brown or greenish-brown ring at the periphery of the cornea, starting superiorly, then inferiorly, and eventually becoming circumferential. It is seen in 95% of patients with neurological involvement and about 50-60% of those with hepatic involvement. **Analysis of Incorrect Options:** * **Keratoconus:** Characterized by **Fleischer rings** (iron deposition at the base of the cone) and **Munson’s sign**, but not KF rings. * **Lowe’s Syndrome (Oculo-cerebro-renal syndrome):** A rare X-linked recessive disorder characterized by congenital cataracts, glaucoma, and renal tubular dysfunction. It does not feature copper deposition. * **Albinism:** Associated with iris transillumination defects, foveal hypoplasia, and nystagmus due to lack of melanin, but no corneal rings. **High-Yield Clinical Pearls for NEET-PG:** * **Location:** Copper deposits specifically in the **Descemet’s membrane** (not the epithelium or stroma). * **Reversibility:** The KF ring may disappear with effective chelation therapy (e.g., Penicillamine). * **Sunflower Cataract:** Another ocular feature of Wilson’s disease, where copper deposits in the anterior lens capsule. * **Diagnosis:** Best visualized using a **Slit-lamp examination** in early stages. * **Differential:** Do not confuse *Fleischer rings* (Iron in Keratoconus) with *Kayser-Fleischer rings* (Copper in Wilson's).

Question 1

Angioid streaks are seen in which of the following conditions?

Accepted Answer

Acromegaly

Answer

Pseudoxanthoma Elasticum

Answer

Paget's disease

Answer

All of the above

Question 2

Koeppe's nodules are seen in which of the following conditions?

Accepted Answer

Granulomatous uveitis

Answer

Non-granulomatous uveitis

Answer

Choroiditis

Answer

Pars planitis

Question 3

Anterior uveitis is characterized by which of the following?

Accepted Answer

Shallow anterior chamber

Answer

Aqueous flare

Answer

Circumcorneal congestion

Answer

Miosis

Question 4

A chalazion is caused by the obstruction of which gland?

Accepted Answer

Meibomian gland

Answer

Zeis's gland

Answer

Lacrimal gland

Answer

None of the above

Question 5

Which of the following is NOT a feature of Behcet's disease?

Accepted Answer

Granulomatous Uveitis

Answer

Oral ulcers

Answer

Erythema multiforme

Answer

HLA B5 association

Question 6

All of the following can lead to iris neovascularization except?

Accepted Answer

Neurofibromatosis II

Answer

Iris tumors

Answer

Iris neovascularization

Answer

Prader-Willi syndrome

Question 7

Which of the following metabolic disorders is associated with the fundoscopic finding shown in the image?

Accepted Answer

Mitochondrial disorders

Answer

Galactosemia

Answer

Albinism

Answer

GM1 Gangliosidosis

Question 8

A 68-year-old woman with a history of left ventricular congestive heart failure has had decreased visual acuity for the past 5 years. She has no ocular pain and her intraocular pressure is normal. Funduscopic examination reveals arteriolar narrowing, flame-shaped hemorrhages, cotton-wool spots, and hard, waxy exudates. Which of the following underlying diseases is she most likely to have?

Accepted Answer

Hypertension

Answer

Advanced atherosclerosis

Answer

Cerebral edema

Answer

Diabetes mellitus

Question 9

What is the most common ocular manifestation in rheumatoid arthritis?

Accepted Answer

Keratoconjunctivitis sicca

Answer

Scleritis

Answer

Episcleritis

Answer

Anterior uveitis

Question 10

The presence of Kayser-Fleischer rings is pathognomonic of which condition?

Accepted Answer

Wilson's disease

Answer

Keratoconus

Answer

Lowe's syndrome

Answer

Albinism

Ocular Manifestations of Systemic Disorders — MCQs

Ocular Manifestations of Systemic Disorders — MCQs

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