Conjunctival Manifestations of Systemic Diseases — MCQs
What are the extra-articular manifestations of RA?
Which of the following is NOT a cause of crystal keratopathy?
What condition is associated with copper deposition in the cornea?
What is the most likely complication of the condition shown in the image below?
Arthritis mutilans is seen in?
Which condition is characterized by conjunctival injection, pharyngeal injection, polymorphic rash, and cervical lymphadenopathy?
What is the term for the fusion of the palpebral and bulbar conjunctiva?
Uveoparotitis is seen in
Which of the following can be associated with sarcoidosis?
Statement 1 - A 59-year-old patient presents with flaccid bullae. Histopathology shows a suprabasal acantholytic split. Statement 2 - The row of tombstones appearance is diagnostic of Pemphigus vulgaris.
Conjunctival Manifestations of Systemic Diseases Indian Medical PG Practice Questions and MCQs
Question 1: What are the extra-articular manifestations of RA?
- A. Pericarditis
- B. Pulmonary fibrosis
- C. Scleritis
- D. All of the options (Correct Answer)
Explanation: ***All of the options*** - **Rheumatoid arthritis (RA)** is a systemic inflammatory disease that can affect multiple organs beyond the joints [1]. Therefore, **pericarditis**, **pulmonary fibrosis**, and **scleritis** are all recognized extra-articular manifestations [2]. - The presence of various extra-articular features often indicates more severe or long-standing disease and is associated with increased morbidity and mortality in RA patients. *Pericarditis* - **Pericarditis** is a recognized cardiovascular manifestation of RA, involving inflammation of the **pericardium**. - While many cases are asymptomatic, it can cause chest pain and, rarely, **pericardial effusion** or **constrictive pericarditis**. *Pulmonary fibrosis* - **Interstitial lung disease (ILD)**, including **pulmonary fibrosis**, is a significant pulmonary complication of RA [2]. - It can lead to progressive shortness of breath, cough, and is a major cause of morbidity and mortality in RA patients. *Scleritis* - **Scleritis** is an ocular manifestation of RA, presenting as severe inflammation of the sclera (the white outer layer of the eyeball) [1]. - It can cause severe pain, redness, and vision changes, and requires prompt treatment to prevent visual impairment [1].
Question 2: Which of the following is NOT a cause of crystal keratopathy?
- A. Schnyder's Dystrophy
- B. Bietti's Dystrophy
- C. Diabetes (Correct Answer)
- D. Cystinosis
Explanation: ***Diabetes*** - While diabetes can cause various ocular complications, such as **diabetic retinopathy**, **neovascular glaucoma**, and **cataracts**, it is **not associated** with crystal keratopathy. - Corneal changes in diabetes might include epithelial defects or reduced corneal sensitivity, but **not the deposition of crystalline substances in the cornea**. - **This is the correct answer** as diabetes does NOT cause crystal keratopathy. *Cystinosis* - **Cystinosis** is a metabolic disorder characterized by the accumulation of **cystine crystals** throughout the body, including the **cornea**. - These crystals can lead to significant **photophobia**, **corneal erosions**, and visual impairment, making it a **classic cause of crystal keratopathy**. - Corneal involvement typically appears in childhood with diffuse crystalline deposits. *Schnyder's Dystrophy* - **Schnyder's corneal dystrophy** is an inherited corneal dystrophy characterized by the deposition of **cholesterol and lipid crystals** in the central and peripheral cornea. - This leads to **dense corneal opacification** and can significantly impair vision over time, representing a **classic cause of crystal keratopathy**. - The cornea shows characteristic subepithelial and stromal crystal deposits. *Bietti's Crystalline Dystrophy* - **Bietti's crystalline dystrophy** is a rare, inherited chorioretinal dystrophy characterized by **crystalline deposits primarily in the retina and choroid**. - While the name suggests crystalline involvement, these crystals are predominantly **intraretinal and do NOT typically cause corneal crystal deposits**. - Therefore, Bietti's is **NOT a typical cause of crystal keratopathy** (corneal disease), though minimal peripheral corneal crystals have been rarely reported in advanced cases. - The confusion arises from the word "crystalline" in its name, but the pathology is retinal, not corneal.
Question 3: What condition is associated with copper deposition in the cornea?
- A. Keratoglobus
- B. Keratoconus
- C. Siderosis
- D. Wilson's disease (Correct Answer)
Explanation: ***Wilson's disease*** - Wilson's disease is a genetic disorder of **copper metabolism** leading to excess copper accumulation in various tissues, including the cornea [1]. - This copper deposition in the posterior Descemet's membrane of the cornea forms a distinctive golden-brown or greenish-brown ring known as the **Kayser-Fleischer ring**. *Keratoconus* - This condition is characterized by progressive thinning and steepening of the cornea, causing it to bulge into a **cone-like shape**. - It primarily affects vision due to irregular astigmatism and does not involve copper deposition. *Keratoglobus* - Keratoglobus is a rare corneal ectatic disorder where the entire cornea is thinned and bulges forward, giving it a **globe-like appearance**. - It is congenital and typically runs in families, and it is not associated with copper deposition. *Siderosis* - Siderosis refers to the deposition of **iron** in various tissues, often due to chronic hemorrhage or metallic foreign bodies. - In the eye, it can occur after intraocular iron foreign bodies, leading to retinal degeneration and other ocular complications, but it does not involve copper.
Question 4: What is the most likely complication of the condition shown in the image below?
- A. Exposure Keratitis (Correct Answer)
- B. Difficulty in eye movement
- C. Cataract
- D. Glaucoma
Explanation: ***Exposure Keratitis*** - The image shows **proptosis** (exophthalmos) of the right eye, where the eyeball protrudes forward. This condition often leads to incomplete eyelid closure (lagophthalmos). - **Exposure keratitis** occurs when the cornea is inadequately covered by the eyelids, leading to drying and damage due to constant exposure to air and environmental factors. *Difficulty in eye movement* - While **proptosis** can sometimes be associated with restricted eye movements (e.g., in severe Graves' ophthalmopathy due to muscle swelling), it is not the **most likely direct complication** of the exposure itself. - The image primarily depicts the physical displacement of the globe, which predisposes to corneal issues, not necessarily oculomotor dysfunction as the primary complication. *Cataract* - **Cataracts** are opacities of the lens and are typically associated with aging, trauma, or certain systemic conditions (e.g., diabetes, steroid use). - They are not a direct or common complication of **proptosis** or the resulting **exposure of the ocular surface**. *Glaucoma* - **Glaucoma** is a group of conditions characterized by damage to the optic nerve, often due to elevated intraocular pressure. - While severe **proptosis** leading to orbital congestion can theoretically increase intraocular pressure, it is not the most direct or prevalent complication compared to **exposure keratitis**, which is a direct consequence of inadequate globe protection.
Question 5: Arthritis mutilans is seen in?
- A. Rheumatoid arthritis
- B. Spondyloarthropathy
- C. Reactive arthritis
- D. Psoriatic arthropathy (Correct Answer)
Explanation: ***Psoriatic arthropathy*** - **Arthritis mutilans** is a severe, destructive form of psoriatic arthritis characterized by marked **osteolysis** and telescoping deformities of the digits [1]. - This condition is almost exclusively associated with **psoriatic arthritis**, representing its most aggressive subtype [1]. *Rheumatoid arthritis* - While rheumatoid arthritis can cause severe joint destruction, it typically manifests as **erosive arthritis** with joint deformities like **swan-neck** and **boutonnière deformities**, but not true arthritis mutilans [3]. - The pattern of bone destruction (osteolysis) seen in arthritis mutilans is distinct from the erosions in rheumatoid arthritis. *Spondyloarthropathy* - This is a broad category that includes diseases like ankylosing spondylitis and reactive arthritis, which primarily affect the **axial skeleton** and entheses. - While some spondyloarthropathies can cause peripheral joint involvement, they generally do not lead to the extreme osteolysis and telescoping digits characteristic of arthritis mutilans. *Reactive arthritis* - Reactive arthritis is an aseptic inflammatory arthritis that often follows infection, characterized by **oligoarthritis**, dactylitis, and enthesitis [2]. - This condition does not typically cause the severe, mutilating joint destruction seen in arthritis mutilans.
Question 6: Which condition is characterized by conjunctival injection, pharyngeal injection, polymorphic rash, and cervical lymphadenopathy?
- A. Kawasaki syndrome (Correct Answer)
- B. Measles
- C. Scarlet fever
- D. Mumps
Explanation: ***Kawasaki syndrome*** - **Kawasaki syndrome** is characterized by a constellation of symptoms including **conjunctival injection**, **pharyngeal injection**, a **polymorphic rash**, and **cervical lymphadenopathy**, often described as the CRASH and burn criteria (Conjunctivitis, Rash, Adenopathy, Strawberry tongue, Hand/foot changes, and Fever). - It is an acute systemic vasculitis, primarily affecting young children, and without treatment, it can lead to **coronary artery aneurysms**. *Measles* - Measles is characterized by a maculopapular rash that typically starts on the face and spreads downwards (cephalocaudal), along with the presence of **Koplik spots** on the buccal mucosa. - While it presents with conjunctivitis and rash, the rash is not polymorphic in the same way as Kawasaki, and cervical lymphadenopathy is less prominent. *Scarlet fever* - **Scarlet fever** is caused by Group A Streptococcus and presents with pharyngitis, fever, and a characteristic **sandpaper-like erythematous rash** with circumoral pallor. - While it has pharyngeal involvement and rash, it lacks the **conjunctival injection** and **polymorphic nature of the rash** seen in Kawasaki syndrome. The rash is typically fine and blanching. - Cervical lymphadenopathy may be present but the overall constellation differs from Kawasaki. *Mumps* - Mumps is an acute viral infection primarily characterized by the swelling of the **parotid glands** (parotitis), often accompanied by fever, headache, and malaise. - It does not typically present with conjunctival injection, a polymorphic rash, or prominent cervical lymphadenopathy as seen in Kawasaki syndrome.
Question 7: What is the term for the fusion of the palpebral and bulbar conjunctiva?
- A. Trichiasis (inward growth of eyelashes)
- B. Ectropion (outward turning of eyelid)
- C. Symblepharon (Correct Answer)
- D. Tylosis (thickening of skin on palms and soles)
Explanation: ***Symblepharon*** - **Symblepharon** is the term for the adhesion between the **palpebral conjunctiva** (lining the eyelid) and the **bulbar conjunctiva** (covering the eyeball). - This condition can limit eye movement and cause chronic irritation, often resulting from severe conjunctival inflammation or injury. *Trichiasis (inward growth of eyelashes)* - **Trichiasis** refers to the misdirection of eyelashes such that they rub against the cornea or conjunctiva. - It causes irritation, foreign body sensation, and can lead to corneal abrasion, but it does not involve fusion of conjunctival layers. *Ectropion (outward turning of eyelid)* - **Ectropion** is a condition where the lower eyelid turns outward or sags away from the eyeball. - This exposes the conjunctiva, causing dryness, irritation, and epiphora (excessive tearing), but it is not a fusion of conjunctival tissues. *Tylosis (thickening of skin on palms and soles)* - **Tylosis** is a medical term referring to diffuse **hyperkeratosis** or thickening of the skin, typically observed on the palms and soles. - This condition is completely unrelated to the conjunctiva or eye structures.
Question 8: Uveoparotitis is seen in
- A. Sjogren's syndrome
- B. Rheumatoid arthritis
- C. SLE
- D. Sarcoidosis (Correct Answer)
Explanation: ***Sarcoidosis*** - **Uveoparotitis** (inflammation of the uveal tract of the eye and the parotid glands) is a classic manifestation of **sarcoidosis**, particularly when associated with **facial nerve palsy (Heerfordt's syndrome)**. - Sarcoidosis is a multisystem granulomatous disease that can affect various organs, including the eyes, salivary glands, and lungs. *Sjogren's syndrome* - Characterized by **dry eyes** (keratoconjunctivitis sicca) and **dry mouth** (xerostomia) due to immune-mediated destruction of exocrine glands. - While it can involve the parotid glands, it typically causes chronic inflammation leading to dryness, not necessarily acute uveoparotitis. *Rheumatoid arthritis* - Primarily an inflammatory arthritis affecting **synovial joints**, leading to joint destruction and deformity. - Ocular involvement can include **scleritis** or **episcleritis**, but not typically uveitis or parotitis as a combined syndrome. *SLE* - A systemic autoimmune disease with diverse manifestations, including kidney disease, skin rashes, and arthritis. - Ocular manifestations can include **retinal vasculitis** or **conjunctivitis**, but uveitis and parotitis as a specific syndrome like uveoparotitis are not characteristic.
Question 9: Which of the following can be associated with sarcoidosis?
- A. Band keratopathy
- B. Diabetic retinopathy
- C. Angioid streaks
- D. Granulomatous anterior uveitis (Correct Answer)
Explanation: ***Granulomatous anterior uveitis*** - **Granulomatous anterior uveitis** is the **most common and characteristic** ocular manifestation of **sarcoidosis**, characterized by inflammatory cells clumping together in the anterior chamber. - Classic findings include **mutton-fat keratic precipitates**, iris nodules (Koeppe and Busacca nodules), and posterior synechiae. - It can lead to complications such as glaucoma, cataracts, and posterior segment involvement. *Band keratopathy* - **Band keratopathy** is a corneal degeneration characterized by calcium deposits in the superficial cornea, typically seen in chronic ocular inflammation, hypercalcemia, or certain systemic disorders. - While sarcoidosis can cause **hypercalcemia** (in 10-20% of cases) which may lead to band keratopathy, this is an **indirect and uncommon** association. - **Granulomatous anterior uveitis** is the **direct, hallmark** ocular manifestation of sarcoidosis, making it the better answer. *Angioid streaks* - **Angioid streaks** are breaks in Bruch's membrane, associated with systemic conditions such as **pseudoxanthoma elasticum**, Ehlers-Danlos syndrome, Paget's disease, and sickle cell disease, but **not sarcoidosis**. - They can lead to choroidal neovascularization and vision loss. *Diabetic retinopathy* - **Diabetic retinopathy** is a microvascular complication of **diabetes mellitus**, characterized by damage to the retinal blood vessels. - It is **completely unrelated to sarcoidosis**.
Question 10: Statement 1 - A 59-year-old patient presents with flaccid bullae. Histopathology shows a suprabasal acantholytic split. Statement 2 - The row of tombstones appearance is diagnostic of Pemphigus vulgaris.
- A. Statements 1 & 2 are correct, 2 is not explaining 1 (Correct Answer)
- B. Statements 1 and 2 are correct and 2 is the correct explanation for 1
- C. Statements 1 and 2 are incorrect
- D. Statement 1 is incorrect
Explanation: ***Correct: Statements 1 & 2 are correct, 2 is not explaining 1*** **Analysis of Statement 1:** - A 59-year-old patient with **flaccid bullae** and **suprabasal acantholytic split** on histopathology is the classic presentation of **Pemphigus vulgaris** - The flaccid (easily ruptured) nature of bullae distinguishes it from tense bullae seen in bullous pemphigoid - The suprabasal location of the split (just above the basal layer) with acantholysis (loss of cell-to-cell adhesion) is pathognomonic - **Statement 1 is CORRECT** ✓ **Analysis of Statement 2:** - The **"row of tombstones" or "tombstone appearance"** is indeed a diagnostic histopathological feature of Pemphigus vulgaris - This appearance results from basal keratinocytes remaining attached to the basement membrane while suprabasal cells separate due to acantholysis - The intact basal cells standing upright resemble a row of tombstones - **Statement 2 is CORRECT** ✓ **Does Statement 2 explain Statement 1?** - Statement 2 describes a **histopathological appearance** (tombstone pattern) that is a **consequence** of the suprabasal split - However, it does NOT explain the **underlying cause** of the flaccid bullae or the suprabasal split - The true explanation involves **IgG autoantibodies against desmoglein 3 (and desmoglein 1)**, which attack intercellular adhesion structures (desmosomes), causing **acantholysis** - Therefore, **Statement 2 does NOT explain Statement 1** ✗ *Incorrect: Statement 2 is the correct explanation for Statement 1* - While both statements describe features of Pemphigus vulgaris, the tombstone appearance is a descriptive finding, not an explanatory mechanism *Incorrect: Statements 1 and 2 are incorrect* - Both statements are medically accurate descriptions of Pemphigus vulgaris features *Incorrect: Statement 1 is incorrect* - Statement 1 correctly describes the cardinal clinical and histopathological features of Pemphigus vulgaris
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