Diseases of the Sclera

Diseases of the Sclera Indian Medical PG Question 1: What are the extra-articular manifestations of RA?

• A. Pericarditis
• B. Pulmonary fibrosis
• C. Scleritis
• D. All of the options (Correct Answer)

Diseases of the Sclera 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].

Diseases of the Sclera Indian Medical PG Question 2: Abatacept, a drug inhibiting co-stimulation is used for:

• A. Rheumatoid arthritis (Correct Answer)
• B. SLE
• C. Sjogren syndrome
• D. Scleroderma

Diseases of the Sclera Explanation: ***Rheumatoid arthritis***- **Abatacept** is a **selective T-cell co-stimulation modulator** that inhibits the activation of T lymphocytes, which are crucial in the pathogenesis of rheumatoid arthritis.- By binding to **CD80 and CD86** on antigen-presenting cells, it prevents their interaction with **CD28** on T cells, thus blocking a key co-stimulatory signal required for full T-cell activation [1].*SLE (Systemic Lupus Erythematosus)*- While T-cell activation plays a role in SLE, abatacept has shown **limited efficacy** in clinical trials for this condition, and its use is not standard.- The dominant mechanism in SLE often involves **B-cell overactivity** and autoantibody production, making other targeted therapies more effective.*Sjogren syndrome*- Sjogren syndrome is characterized by lymphocytic infiltration of exocrine glands, but abatacept is **not a primary treatment** or widely indicated for this condition.- The main focus of treatment for Sjogren's is typically symptomatic management and immunosuppression with other agents.*Scleroderma (Systemic Sclerosis)*- Scleroderma involves **fibrosis** and vascular damage with complex immunological mechanisms, but abatacept has **not demonstrated significant benefit** in clinical studies.- The disease often has more prominent involvement of **fibroblasts** and different immunological pathways that are not adequately targeted by abatacept.

Diseases of the Sclera Indian Medical PG Question 3: A 35 year old lady complains dysphagia, Raynaud's phenomenon, sclerodactyly. Investigations show antinuclear antibody. The likely diagnosis is ?

• A. Rheumatoid arthritis
• B. Systemic lupus erythematosus
• C. Systemic sclerosis (Correct Answer)
• D. Mixed connective tissue disorder

Diseases of the Sclera Explanation: ***Systemic sclerosis*** - The classic triad of **Raynaud's phenomenon**, **sclerodactyly**, and **維持dysphagia** (due to esophageal dysmotility) is highly characteristic of systemic sclerosis [1]. - The presence of **antinuclear antibodies (ANA)** further supports this diagnosis, as ANA is positive in the majority of systemic sclerosis patients. *Rheumatoid arthritis* - Characterized by **symmetric polyarthritis**, often affecting small joints of the hands and feet, which is not described here. - While ANA can be positive, **rheumatoid factor** and **anti-CCP antibodies** are more specific markers, and the clinical features presented are not typical for RA. *Systemic lupus erythematosus* - This condition presents with a wide range of symptoms, including **arthritis**, malar rash, serositis, and renal involvement, which are not mentioned [2]. - While ANA is a hallmark, the specific combination of **sclerodactyly** and **significant dysphagia due to esophageal dysmotility** is less characteristic of SLE compared to systemic sclerosis [2]. *Mixed connective tissue disorder* - This syndrome features a combination of features from **SLE**, **systemic sclerosis**, and **polymyositis**, with high titers of **anti-U1-RNP antibodies** [1]. - While it can include Raynaud's phenomenon and sclerodactyly, the strong emphasis on prominent dysphagia along with isolated sclerodactyly points more specifically towards systemic sclerosis.

Diseases of the Sclera Indian Medical PG Question 4: 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

Diseases of the Sclera 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.

Diseases of the Sclera Indian Medical PG Question 5: A 65-year-old patient presents with severe headache, temporal artery tenderness, and decreased pulse. What is the most likely diagnosis?

• A. Giant cell arteritis (Correct Answer)
• B. Wegener's granulomatosis
• C. Microscopic polyangiitis
• D. Takayasu arteritis

Diseases of the Sclera Explanation: ***Giant cell arteritis*** - This presentation with **severe headache**, **temporal artery tenderness**, and a **decreased pulse** in a 65-year-old patient is highly classic for giant cell arteritis (GCA). GCA characteristically affects **medium and large arteries**, often the **temporal artery**. - **Decreased pulse** can indicate involvement of other large vessels, such as the subclavian artery, which can occur in GCA. Urgent diagnosis and treatment are crucial due to the risk of **permanent vision loss** [1]. *Wegener's granulomatosis* - This condition (**granulomatosis with polyangiitis**) is characterized by **upper and lower respiratory tract granulomatous inflammation**, **glomerulonephritis**, and small vessel vasculitis. - While it can manifest with systemic symptoms, **temporal artery tenderness** and a **decreased pulse** are not primary features of Wegener's. *Microscopic polyangiitis* - This is a **small vessel vasculitis** that primarily affects capillaries, venules, and arterioles. - It typically presents with **glomerulonephritis** and **pulmonary capillaritis**, but without granuloma formation, and does not involve the temporal arteries or lead to a decreased pulse in the manner described. *Takayasu arteritis* - Takayasu arteritis primarily affects the **aorta and its major branches**, leading to **claudication**, **pulse deficits** in the extremities, and often occurs in **younger women**. - While it can cause a decreased pulse, it is less likely to present with **temporal artery tenderness** and severe headache in a 65-year-old, as these symptoms are more characteristic of GCA.

Diseases of the Sclera Indian Medical PG Question 6: Most reliable sign of posterior scleritis is:

• A. Disc edema
• B. Choroidal folds
• C. Exudative detachment
• D. T-sign on ultrasound (Correct Answer)

Diseases of the Sclera 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.

Diseases of the Sclera Indian Medical PG Question 7: A patient presents with proptosis, restriction of eye movements, and is found to be euthyroid. What is the most likely diagnosis?

• A. Orbital cellulitis
• B. Orbital lymphoma
• C. Orbital pseudotumor (Correct Answer)
• D. Thyroid eye disease

Diseases of the Sclera Explanation: ***Orbital pseudotumor*** - **Orbital pseudotumor** (also known as idiopathic orbital inflammation) presents with **proptosis** and **restriction of eye movements**. - Typically presents with **acute or subacute onset** of **painful** ophthalmoplegia and proptosis. - It is a **diagnosis of exclusion** after ruling out other causes of orbital inflammation, including thyroid eye disease, orbital cellulitis, and orbital neoplasms. - **Euthyroid status** does not exclude this diagnosis, and it is the **most common painful orbital mass** in adults. *Orbital cellulitis* - **Orbital cellulitis** presents with **rapid onset** of **painful proptosis**, **chemosis**, **ophthalmoplegia**, and signs of **acute infection** (fever, periorbital erythema, recent sinusitis). - The absence of infectious signs and acute inflammatory markers makes this less likely. *Orbital lymphoma* - **Orbital lymphoma** typically presents with **slowly progressive, painless proptosis** in older patients. - It is a **chronic, indolent process** and less likely to cause acute, painful restriction of eye movements. - Usually presents as a palpable mass in the superotemporal orbit. *Thyroid eye disease* - **Thyroid eye disease** (Graves' ophthalmopathy) commonly causes **proptosis** and **restricted eye movements** due to extraocular muscle enlargement. - While **90% of TED patients have hyperthyroidism**, approximately **5% are euthyroid at presentation** (euthyroid Graves' ophthalmopathy). - However, TED typically has a **subacute to chronic onset**, bilateral involvement, lid retraction, and characteristic imaging findings (muscle belly enlargement sparing tendons). - The clinical presentation with acute symptoms and euthyroid state makes **orbital pseudotumor more likely** as the initial diagnosis.

Diseases of the Sclera Indian Medical PG Question 8: In blowout fractures, which of the following is seen?

• A. Bulbar hemorrhage
• B. None of the options
• C. Enophthalmos (Correct Answer)
• D. Exophthalmos

Diseases of the Sclera Explanation: ***Enophthalmos*** - A **blowout fracture** of the orbit typically involves the orbital floor or medial wall from direct trauma to the eye or periorbital region. - The fracture allows orbital contents (fat and muscle) to herniate into the maxillary sinus or ethmoid sinuses, **increasing orbital volume**. - This increased volume causes the eye to recede backward into the orbit, resulting in **enophthalmos** (sunken eye appearance). - **Key clinical features**: enophthalmos, diplopia (due to inferior rectus/medial rectus entrapment), restricted eye movements, infraorbital nerve hypoesthesia. *Exophthalmos* - **Exophthalmos** (proptosis) is forward protrusion of the eye, occurring when orbital volume is **decreased** or orbital contents are **increased** (e.g., thyroid eye disease, orbital tumors, orbital hemorrhage). - This is the **opposite** of enophthalmos and would not occur in a blowout fracture where orbital volume increases. *Bulbar hemorrhage* - **Subconjunctival hemorrhage** may occur as an associated finding from ocular trauma but is not a characteristic or defining feature of blowout fractures. - Many types of blunt ocular trauma can cause conjunctival hemorrhage without orbital fracture. *None of the options* - Incorrect because **enophthalmos** is the classic and characteristic finding of orbital blowout fractures.

Diseases of the Sclera Indian Medical PG Question 9: Enophthalmos can be caused by all of the following EXCEPT:

• A. Orbital floor fracture
• B. Loss of orbital fat
• C. Horner's syndrome (Correct Answer)
• D. Cicatricial changes

Diseases of the Sclera Explanation: ***Horner's syndrome*** - **Horner's syndrome** is characterized by **ptosis**, **miosis**, and **anhidrosis** on the affected side. - While it can manifest with a mild degree of **apparent enophthalmos**, this is primarily due to the **ptosis creating an illusion** of globe retraction and **not true enophthalmos**. - It is caused by disruption of the **sympathetic nervous supply**, not by actual posterior displacement of the globe. *Cicatricial changes* - **Cicatricial changes** (scarring) within the orbit can cause **traction on the globe**, pulling it inward and resulting in **true enophthalmos**. - This scarring can occur following **trauma**, **inflammation**, or **surgery** affecting the orbital tissues. *Orbital floor fracture* - An **orbital floor fracture** typically leads to **enophthalmos** due to **herniation of orbital contents** (fat, muscle) into the maxillary sinus. - This involves a **structural defect** with increased orbital volume and loss of support for the globe. *Loss of orbital fat* - **Loss of orbital fat**, often seen in conditions like **Romberg's disease**, severe dehydration, or aging, causes the globe to sink backward. - This is due to a **reduction in volume supporting the globe**, resulting in **true enophthalmos**.

Diseases of the Sclera Indian Medical PG Question 10: Dalen-Fuchs nodules are seen in which of the following?

• A. Retinal detachment
• B. Spring catarrh
• C. Sympathetic ophthalmitis (Correct Answer)
• D. Vogt-Koyanagi-Harada disease

Diseases of the Sclera Explanation: ***Sympathetic ophthalmitis*** - **Dalen-Fuchs nodules** are characterized by accumulations of **lymphocytes, epithelioid cells, and pigment** in the choroid, specifically between Bruch's membrane and the retinal pigment epithelium. - They are a **pathognomonic sign** of sympathetic ophthalmitis, an autoimmune inflammatory reaction in the contralateral eye after penetrating trauma or surgery to the other eye. - These nodules represent a **granulomatous inflammatory response** and are a key histopathological finding. *Retinal detachment* - This condition involves the **separation of the neurosensory retina** from the underlying retinal pigment epithelium. - It is typically characterized by symptoms such as **flashes of light, floaters**, and a **darkening peripheral visual field**, rather than specific nodular formations in the choroid. *Spring catarrh* - Also known as **vernal keratoconjunctivitis**, this is a severe, chronic, bilateral inflammation of the conjunctiva, primarily affecting children and young adults, often with a history of atopy. - Its characteristic features include **papillae on the upper tarsal conjunctiva** and **Trantas dots** (collections of eosinophils and epithelial cells) at the limbus, not Dalen-Fuchs nodules. *Vogt-Koyanagi-Harada disease* - While VKH disease can also show Dalen-Fuchs nodules as it shares similar granulomatous uveitis features, it is a **distinct bilateral panuveitis** with systemic manifestations including **poliosis, vitiligo, dysacusia, and meningismus**. - The key differentiating factor is that **sympathetic ophthalmitis requires prior trauma or surgery to one eye**, whereas VKH disease has no such requirement and presents with characteristic extraocular manifestations.

Diseases of the Sclera Flashcard 1 of 7

Question: _____ scleritis is the most common type of scleritis.

Answer: Non-necrotizing anterior

Diseases of the Sclera Flashcard 2 of 7

Question: What part of eye is affected by nevus of Ota?_____

Answer: Sclera

Diseases of the Sclera Flashcard 3 of 7

Question: _____ scleritis without inflammation is seen in long standing cases of rheumatoid arthritis

Answer: Necrotizing

Diseases of the Sclera Flashcard 4 of 7

Question: Surgically induced _____ (SINS) may occur following any surgery involving the creation of a scleral wound

Answer: necrotizing scleritis

Diseases of the Sclera Flashcard 5 of 7

Question: Equatorial staphyloma occurs up to _____mm behind the limbus

Answer: 14

Diseases of the Sclera Flashcard 6 of 7

Question: Ciliary staphyloma occurs up to _____mm behind the limbus

Answer: 8

Diseases of the Sclera Flashcard 7 of 7

Question: Scleromalacia perforans is a pain_____ condition

Answer: less