Necrotizing Scleritis — MCQs
Uveoparotid fever is seen in:
A patient presents with a nodular swelling near the limbus, which does not blanch with topical vasoconstrictors and recurs after treatment. Based on the image and clinical presentation, what is the most probable diagnosis?
Purtscher's retinopathy is associated with -
Pauciarticular JRA is characterized by all except:
The ideal indication for injection of sclerosing agents is:
Cyclosporine is used in the management of -
Most reliable sign of posterior scleritis is:
A patient presents with cutaneous vasculitis, glomerulonephritis and peripheral neuropathy. What investigation is to be performed next for work up?
Most common type of scleritis among the following is
Most common type of scleritis is
Necrotizing Scleritis Indian Medical PG Practice Questions and MCQs
Question 1: Uveoparotid fever is seen in:
- A. Rheumatoid Arthritis
- B. Sjogren's syndrome
- C. SLE
- D. Sarcoidosis (Correct Answer)
Explanation: ***Sarcoidosis*** - **Uveoparotid fever** (also known as Heerfordt's syndrome) is a rare manifestation of **sarcoidosis**, characterized by bilateral **uveitis**, **parotid gland enlargement**, facial nerve palsy, and fever [1]. - Sarcoidosis is a systemic granulomatous disease, and **uveoparotid fever** represents a specific, classic constellation of symptoms within this condition [1]. *Rheumatoid Arthritis* - While **rheumatoid arthritis** can cause **ocular inflammation** (e.g., scleritis, episcleritis), it does not typically present as **uveitis** or **parotid gland enlargement** [2]. - Its primary targets are synovial joints, leading to inflammatory **polyarthritis**. *Sjogren's syndrome* - **Sjogren's syndrome** is well-known for affecting exocrine glands, causing **sicca symptoms** (dry eyes and mouth) due to lymphocytic infiltration of the lacrimal and salivary glands [2]. - However, while **parotid gland enlargement** can occur, it's not typically associated with **uveitis** in the specific combination described as **uveoparotid fever**. *SLE* - **Systemic Lupus Erythematosus (SLE)** is a multi-system autoimmune disease that can affect nearly any organ, including the eyes (e.g., retinal vasculitis, episcleritis) and occasionally salivary glands [3]. - However, **uveoparotid fever** is not a characteristic or specific manifestation of **SLE**.
Question 2: A patient presents with a nodular swelling near the limbus, which does not blanch with topical vasoconstrictors and recurs after treatment. Based on the image and clinical presentation, what is the most probable diagnosis?
- A. Scleritis with rheumatoid arthritis (Correct Answer)
- B. Episcleritis with rheumatoid arthritis
- C. Pinguecula
- D. Dry eye
Explanation: ***Scleritis with rheumatoid arthritis*** - The image shows **deep, violaceous conjunctival injection** with surrounding edema and a nodular appearance near the limbus, consistent with **nodular scleritis**. Scleritis is inflammation of the sclera, often characterized by severe pain and association with systemic autoimmune diseases like **rheumatoid arthritis**, which can cause destructive lesions and recurrence. - The characteristic **deep vessel engorgement that does not blanch with phenylephrine** and the history of recurrence further support scleritis. *Episcleritis with rheumatoid arthritis* - Episcleritis presents with a **more superficial, bright red injection** involving the episclera, which generally **blanches with topical phenylephrine** and is less painful than scleritis. - While episcleritis can be associated with rheumatoid arthritis, the clinical features described (nodular swelling, deep injection) are more typical of scleritis. *Pinguecula* - A pinguecula is a **yellowish patch or bump** on the conjunctiva, typically on the nasal side of the eye, that is **not inflamed** unless irritated. - It consists of **degenerated collagen fibers** and elastic tissue, and does not present with the diffuse, deep vascular injection seen in the image. *Dry eye* - Dry eye is characterized by **ocular dryness, irritation, and sometimes a foreign body sensation**, but it typically causes **diffuse conjunctival hyperemia** rather than a localized, nodular, deep inflammation with surrounding edema as shown. - While dry eye can be associated with autoimmune diseases, its appearance is not consistent with the depicted nodular lesion.
Question 3: Purtscher's retinopathy is associated with -
- A. Head trauma (Correct Answer)
- B. Diabetes Mellitus
- C. Wilson's disease
- D. Rheumatoid arthritis
Explanation: ***Head trauma*** - **Purtscher's retinopathy** is a rare occlusive microvasculopathy characterized by sudden vision loss and specific retinal findings, classically associated with **head or chest trauma**. - The condition occurs due to **fat embolization** and **leukoembolization** with complement activation, leading to occlusion of retinal precapillary arterioles. - Classic findings include **cotton wool spots**, intraretinal hemorrhages, and **Purtscher flecken** (polygonal areas of retinal whitening between the optic disc and macula). - The term "Purtscher's retinopathy" specifically refers to trauma-induced cases, while "Purtscher-like retinopathy" describes similar findings from non-traumatic causes (acute pancreatitis, fat embolism syndrome, amniotic fluid embolism). *Diabetes Mellitus* - **Diabetic retinopathy** is associated with chronic hyperglycemia causing microvascular damage, characterized by microaneurysms, dot-blot hemorrhages, hard exudates, and venous beading. - It involves gradual progressive changes over years, not acute embolic occlusion from trauma. - Advanced stages include **proliferative diabetic retinopathy** with neovascularization and diabetic macular edema. *Wilson's disease* - **Wilson's disease** is an autosomal recessive disorder of copper metabolism affecting the liver, brain, and eyes. - Ocular manifestations include **Kayser-Fleischer rings** (copper deposition in Descemet's membrane) and sunflower cataracts. - It does not cause acute retinal vascular occlusion or the specific findings of Purtscher's retinopathy. *Rheumatoid arthritis* - **Rheumatoid arthritis** is a chronic autoimmune inflammatory disease primarily affecting joints. - Ocular manifestations include **keratoconjunctivitis sicca** (dry eyes), **episcleritis**, **scleritis**, and peripheral ulcerative keratitis. - It is not associated with Purtscher's retinopathy, which is specifically linked to traumatic or embolic events.
Question 4: Pauciarticular JRA is characterized by all except:
- A. Scleritis (Correct Answer)
- B. Uveitis
- C. Keratopathy
- D. Cataract
Explanation: ***Scleritis*** - **Scleritis** is a rare ocular manifestation in juvenile idiopathic arthritis (JIA) and is not a characteristic feature of **pauciarticular JRA**. - Its presence would suggest other systemic inflammatory conditions or more severe forms of JIA, but not typical pauciarticular JRA. *Uveitis* - **Uveitis**, specifically **chronic anterior uveitis**, is a common and often asymptomatic complication in pauciarticular JRA, particularly in ANA-positive girls. - Regular ophthalmologic screening is crucial for early detection and prevention of long-term vision impairment. *Keratopathy* - While not a direct primary manifestation, **keratopathy** (corneal disease) can occur as a secondary complication of chronic **uveitis** in pauciarticular JRA, often due to inflammation and prolonged use of corticosteroids. - Corneal band keratopathy, in particular, is associated with chronic anterior uveitis. *Cataract* - **Cataract formation** is a known complication associated with chronic **uveitis** in pauciarticular JRA, often exacerbated by long-term corticosteroid use. - It results from chronic inflammation affecting the lens of the eye and can lead to significant vision loss if untreated.
Question 5: The ideal indication for injection of sclerosing agents is:
- A. External hemorrhoids
- B. Internal hemorrhoids (Correct Answer)
- C. Immediate surgery for strangulated hemorrhoids
- D. Surgical intervention for prolapsed hemorrhoids
Explanation: ***Internal hemorrhoids*** - Sclerotherapy is most effective for **first- and second-degree internal hemorrhoids**, where symptomatic bleeding is the primary concern. - The injected agent causes **fibrosis** and **scarring**, leading to fixation of the hemorrhoidal tissue and reduced blood flow. *External hemorrhoids* - External hemorrhoids are located **below the dentate line** and are covered by sensitive anoderm. - Sclerosing agents can cause **significant pain** and are generally ineffective for external hemorrhoids. *Immediate surgery for strangulated hemorrhoids* - **Strangulated hemorrhoids** are a medical emergency requiring **urgent surgical intervention** to prevent tissue necrosis. - Sclerotherapy is absolutely **contraindicated** in this scenario due to the risk of exacerbating ischemia and complications. *Surgical intervention for prolapsed hemorrhoids* - While sclerotherapy can be used for some early-stage prolapsed internal hemorrhoids (second degree), **surgical intervention** is more appropriate for **third- and fourth-degree prolapsed hemorrhoids**. - These more advanced hemorrhoids often require techniques like **hemorrhoidectomy** or stapling for definitive treatment.
Question 6: Cyclosporine is used in the management of -
- A. Disciform Keratitis (Correct Answer)
- B. Phacomorphic glaucoma
- C. Anterior Iridocyclitis
- D. Rhegmatogenous retinal detachment
Explanation: ***Disciform Keratitis*** - **Cyclosporine** is an immunosuppressant particularly valuable in managing **disciform keratitis**, which represents immune-mediated stromal inflammation typically associated with herpes simplex virus (HSV). - In disciform keratitis, the primary pathology is **immune-mediated** rather than active viral replication, making cyclosporine an ideal therapeutic choice to suppress the inflammatory response while avoiding steroid dependency. - **Topical cyclosporine 0.05% or 1%** is specifically indicated for this condition and represents a **characteristic indication** in ophthalmic practice. *Anterior Iridocyclitis* - While cyclosporine may be used in **chronic, recurrent, or steroid-dependent anterior uveitis**, it is not a first-line agent for typical acute anterior iridocyclitis. - First-line management involves **topical corticosteroids** and **cycloplegics**; cyclosporine is reserved for refractory cases. - Disciform keratitis remains the more characteristic and commonly tested indication for cyclosporine in ophthalmology. *Rhegmatogenous retinal detachment* - **Rhegmatogenous retinal detachment** is a surgical emergency caused by a retinal tear allowing vitreous fluid into the subretinal space. - Management is **surgical** (scleral buckle, vitrectomy, or pneumatic retinopexy); immunosuppressants like cyclosporine have no role in this mechanical problem. *Phacomorphic glaucoma* - **Phacomorphic glaucoma** is secondary angle-closure glaucoma caused by an intumescent (swollen) lens pushing the iris forward. - Treatment involves **IOP control** followed by **cataract extraction**; cyclosporine has no role in this mechanical lens-induced condition.
Question 7: Most reliable sign of posterior scleritis is:
- A. Disc edema
- B. Choroidal folds
- C. Exudative detachment
- D. T-sign on ultrasound (Correct Answer)
Explanation: ***T-sign on ultrasound*** - The **T-sign** on B-scan ultrasound is considered the most reliable diagnostic sign of **posterior scleritis**. - It represents accumulation of **fluid in Tenon's capsule** and is highly specific for the condition. *Disc edema* - While **disc edema** can be a clinical finding in posterior scleritis due to inflammation, it is not specific and can be caused by various other conditions, such as **optic neuritis** or papilledema. - It is not as pathognomonic as the T-sign on ultrasound for confirming the diagnosis. *Choroidal folds* - **Choroidal folds** can occur in posterior scleritis due to scleral inflammation and thickening, which can indent the choroid. - However, choroidal folds can also be seen in other conditions like **orbital tumors** or **hypotony**, making them a less specific indicator. *Exudative detachment* - An **exudative retinal detachment** can occur in severe cases of posterior scleritis due to inflammation and fluid leakage from the inflamed choroid into the subretinal space. - This is a serious complication and a sign of advanced disease, but not the earliest or most reliable diagnostic sign for the condition itself, as it can also be seen in other inflammatory or vascular conditions.
Question 8: A patient presents with cutaneous vasculitis, glomerulonephritis and peripheral neuropathy. What investigation is to be performed next for work up?
- A. HBsAg
- B. RA factor
- C. CRP
- D. ANCA (Correct Answer)
Explanation: **ANCA** - The combination of **cutaneous vasculitis**, **glomerulonephritis**, and **peripheral neuropathy** points strongly towards a Systemic Vasculitis [1]. **ANCA** (anti-neutrophil cytoplasmic antibodies) are crucial for diagnosing and classifying **ANCA-associated vasculitides**, such as **Granulomatosis with Polyangiitis (GPA)**, **Microscopic Polyangiitis (MPA)**, and **Eosinophilic Granulomatosis with Polyangiitis (EGPA)**, which perfectly fit this clinical picture [1]. - Identification of specific ANCA patterns (p-ANCA or c-ANCA) and their corresponding target antigens (e.g., **MPO** or **PR3**) is essential for confirming the diagnosis and guiding treatment. *HBsAg* - While **Hepatitis B virus (HBV) infection** can cause **polyarteritis nodosa (PAN)**, which may present with vasculitis and neuropathy, it typically does not involve **glomerulonephritis** as a primary feature [1]. - Testing for HBsAg would be relevant if PAN was strongly suspected or if other risk factors for HBV were present, but ANCA testing is more direct for the given clinical triad. *RA factor* - **Rheumatoid factor (RF)** is primarily associated with **Rheumatoid Arthritis**, which is an inflammatory joint disease and does not typically present with this triad of **vasculitis**, **glomerulonephritis**, and **neuropathy** [2]. - Although some connective tissue diseases can have vasculitic manifestations, RF itself is not a primary diagnostic marker for systemic vasculitis. *CRP* - **C-reactive protein (CRP)** is a general **marker of inflammation**, which would undoubtedly be elevated in a patient with systemic vasculitis [1]. - However, CRP is **non-specific** and would not help in narrowing down the diagnosis or determining the etiology of the vasculitis, making it less useful for specific workup compared to ANCA.
Question 9: Most common type of scleritis among the following is
- A. Necrotizing
- B. Granulomatous
- C. Posterior
- D. Non-necrotizing (Correct Answer)
Explanation: ***Non-necrotizing*** - This category, particularly **diffuse non-necrotizing scleritis**, is the most frequently encountered type. - It is often characterized by widespread inflammation of the sclera without tissue loss. *Necrotizing* - This is a severe form of scleritis associated with significant **tissue destruction** and a high risk of vision loss. - While serious, it is thankfully much rarer than the non-necrotizing forms. *Granulomatous* - This term describes a **histopathological feature** of inflammation rather than a distinct clinical type of scleritis. - Granulomatous inflammation can be seen in various forms of scleritis but is not a primary classification of its most common presentation. *Posterior* - **Posterior scleritis** specifically involves inflammation of the sclera behind the equator of the globe. - It is less common than anterior scleritis and can present with different symptoms such as vision loss or pain with eye movement.
Question 10: Most common type of scleritis is
- A. Diffuse anterior
- B. Nodular anterior
- C. Anterior (Correct Answer)
- D. Posterior
Explanation: ***Anterior*** - **Anterior scleritis** accounts for approximately **98%** of all scleritis cases, making it the most common type. - It involves inflammation of the sclera anterior to the **equator of the globe**, visible on external examination. - Anterior scleritis is further subdivided into **diffuse, nodular,** and **necrotizing** forms based on clinical presentation and severity. *Diffuse anterior* - **Diffuse anterior scleritis** is the most common subtype of anterior scleritis, characterized by widespread inflammation. - While common among anterior types, it represents a subset rather than the overall most common anatomical category. *Nodular anterior* - **Nodular anterior scleritis** presents with discrete nodules of inflamed scleral tissue. - It is less common than diffuse anterior scleritis but more common than necrotizing forms. *Posterior* - **Posterior scleritis** is rare, accounting for only about **2%** of all scleritis cases. - It involves inflammation posterior to the **equator of the globe** and can be difficult to diagnose due to its hidden location, often presenting with pain, proptosis, and vision loss.
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