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: 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 4: 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 5: 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 Flashcard 1 of 5

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

Answer: Non-necrotizing anterior

Diseases of the Sclera Flashcard 2 of 5

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

Answer: Sclera

Diseases of the Sclera Flashcard 3 of 5

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

Answer: Necrotizing

Diseases of the Sclera Flashcard 4 of 5

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 5

Question: Equatorial staphyloma occurs up to _____mm behind the limbus

Answer: 14