Scleral Manifestations of Systemic Disease — MCQs
What are the extra-articular manifestations of RA?
Recurrent anterior uveitis with increased intraocular tension is seen in which of the following conditions?
All of the following conditions are associated with raised ANCA, except:
Which of the following is NOT a connective tissue disorder?
Blue sclera is seen in:
All of the following features may be used to distinguish PAN from microscopic polyangitis, except:
The most common variety of scleritis is:
Most common type of scleritis among the following is
Neurological manifestation of Whipple's disease is:
Vertebra Plana is associated with all of the following conditions except -
Scleral Manifestations of Systemic Disease 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: Recurrent anterior uveitis with increased intraocular tension is seen in which of the following conditions?
- A. Posner-Schlossman syndrome (Correct Answer)
- B. Foster-Kennedy syndrome
- C. Vogt-Koyanagi-Harada syndrome
- D. Fuchs heterochromic iridocyclitis
Explanation: ***Posner-Schlossman syndrome*** - Characterized by **recurrent, unilateral, non-granulomatous anterior uveitis** associated with markedly **elevated intraocular pressure (IOP)**. - The condition is also known as **glaucomatocyclitic crisis**, highlighting the episodic inflammation and glaucoma. - Key features include **acute attacks** lasting hours to weeks with **dramatic IOP elevation** (often >40 mmHg). *Foster-Kennedy syndrome* - This syndrome is defined by ipsilateral **optic atrophy**, contralateral **papilledema**, and often **anosmia**, typically due to a frontal lobe tumor. - It does not involve anterior uveitis or primary elevated intraocular tension. - This is a neuro-ophthalmologic syndrome, not an inflammatory ocular condition. *Vogt-Koyanagi-Harada syndrome* - An autoimmune disorder affecting pigmented tissues, leading to **bilateral granulomatous panuveitis**, often with hearing loss, vitiligo, poliosis, and neurological symptoms. - While it involves uveitis, it is typically **bilateral and panuveitis**, not recurrent unilateral anterior uveitis. - IOP may be elevated but not the defining feature with dramatic episodic rises. *Fuchs heterochromic iridocyclitis* - A chronic, **unilateral, low-grade anterior uveitis** with characteristic iris heterochromia. - May have mild IOP elevation but **not recurrent episodic attacks** with marked pressure spikes. - Inflammation is typically **quiet and chronic** rather than acute and recurrent.
Question 3: All of the following conditions are associated with raised ANCA, except:
- A. Polyarteritis Nodosa (Correct Answer)
- B. Wegener's granulomatosis
- C. Microscopic Polyangitis
- D. Churg-Strauss syndrome
Explanation: ***Polyarteritis Nodosa*** - Polyarteritis Nodosa is not typically associated with **positive ANCA** (Anti-Neutrophil Cytoplasmic Antibodies) and is mainly characterized by systemic vasculitis affecting medium-sized arteries. [1] - This condition usually presents with symptoms like **weight loss**, **fever**, and **muscle pain**, without the underlying ANCA association. [1] *Wegener's granulomatosis* - Also known as Granulomatosis with Polyangiitis, this condition is strongly associated with **c-ANCA** positivity, often targeting **proteinase 3**. - Clinical features include **respiratory symptoms** and renal involvement, particularly **glomerulonephritis**. *Microscopic Polyangitis* - This vasculitis is associated with **p-ANCA** positivity, commonly targeting **myeloperoxidase** (MPO). - It frequently presents with **pulmonary hemorrhage** and **renal vasculitis**. *Churg-Strauss syndrome* - Known as Eosinophilic Granulomatosis with Polyangiitis, it is associated with **p-ANCA** and involves asthma, eosinophilia, and systemic vasculitis [2]. - Typical manifestations include **respiratory involvement** and peripheral neuropathy, further linking it to ANCA positivity.
Question 4: Which of the following is NOT a connective tissue disorder?
- A. Osteoarthritis
- B. Fibromyalgia
- C. Sjogren's syndrome (Correct Answer)
- D. SLE
Explanation: ***Sjogren's syndrome*** - Sjogren's syndrome is an **autoimmune disease** characterized by **dry eyes** and **dry mouth**, resulting from immune-mediated destruction of exocrine glands [1]. - While it can involve multiple organ systems and is classified as a **systemic autoimmune disorder**, it is primarily an **exocrinopathy**, and not exclusively a disorder of connective tissue structure or function. *Osteoarthritis* - Osteoarthritis is a **degenerative joint disease** primarily affecting the **articular cartilage** and underlying bone. - While it involves destruction of cartilage, which is a connective tissue, it is considered a **degenerative rather than a systemic inflammatory or autoimmune connective tissue disorder**. *Fibromyalgia* - Fibromyalgia is a chronic pain condition characterized by **widespread musculoskeletal pain**, fatigue, and sleep disturbances, without evidence of inflammation or tissue damage [3]. - It is considered a **central sensitization syndrome** rather than a disorder of the connective tissue itself [3]. *SLE* - Systemic lupus erythematosus (SLE) is a **prototypical systemic autoimmune connective tissue disorder** affecting multiple organ systems [2]. - It is characterized by **autoantibody production** and immune complex deposition, leading to inflammation and damage in joints, skin, kidneys, and other tissues [2].
Question 5: Blue sclera is seen in:
- A. Rheumatoid arthritis
- B. Diabetes mellitus
- C. Osteogenesis imperfecta (Correct Answer)
- D. Hypertension
Explanation: ***Osteogenesis imperfecta*** - Blue sclera in **osteogenesis imperfecta** is due to the underlying defect in **collagen type I synthesis**, which causes the sclera to be thin and translucent [1]. - This thinness allows the underlying **choroidal pigmentation** to show through, giving it a characteristic blue hue. *Rheumatoid arthritis* - While rheumatoid arthritis can affect the eyes (e.g., scleritis, episcleritis), it does not typically cause **blue sclera**. - Ocular manifestations are usually related to inflammation and can lead to **scleral thinning** or perforation but are distinct from the blue appearance seen in osteogenesis imperfecta. *Diabetes mellitus* - Diabetes mellitus can lead to various ocular complications, such as **diabetic retinopathy**, **cataracts**, and **glaucoma**. - It does not cause a change in the color of the sclera to blue. *Hypertension* - Hypertension can affect the eyes, particularly the **retinal vasculature** (hypertensive retinopathy), and increase the risk of conditions like **glaucoma**. - It has no direct association with the phenomenon of **blue sclera**.
Question 6: All of the following features may be used to distinguish PAN from microscopic polyangitis, except:
- A. RBC cast in urine
- B. ANCA positivity
- C. HBV infection
- D. Necrotizing vasculitis (Correct Answer)
Explanation: ***Necrotizing vasculitis*** - Both **polyarteritis nodosa (PAN)** and **microscopic polyangiitis (MPA)** are characterized by **necrotizing vasculitis**, making it a shared feature rather than a distinguishing one. [1] - This pathological finding describes the **inflammation** and **necrosis** of vessel walls, which is central to the pathogenesis of both conditions. [1] *RBC cast in urine* - **Red blood cell (RBC) casts** in the urine are indicative of **glomerulonephritis**, which is a prominent feature of **microscopic polyangiitis (MPA)** but typically absent in **polyarteritis nodosa (PAN)**. [1] - The presence of RBC casts points to **renal involvement**, particularly in the small vessels of the glomeruli, which distinguishes MPA's pattern of injury. [1] *ANCA positivity* - **Anti-neutrophil cytoplasmic antibodies (ANCAs)**, particularly **p-ANCA (MPO-ANCA)**, are frequently positive in **microscopic polyangiitis (MPA)** but usually negative in **polyarteritis nodosa (PAN)**. - ANCA positivity helps classify MPA as an **ANCA-associated vasculitis**, a distinction not typically applied to PAN. *HBV infection* - **Hepatitis B virus (HBV) infection** is strongly associated with a significant subset of **polyarteritis nodosa (PAN)** cases, whereas this association is rare in **microscopic polyangiitis (MPA)**. - Serological testing for HBV can therefore help differentiate between the two conditions, with a positive result favoring PAN.
Question 7: The most common variety of scleritis is:
- A. Non-necrotizing anterior nodular
- B. Anterior necrotizing without inflammation
- C. Anterior necrotizing with inflammation
- D. Non-necrotizing anterior diffuse (Correct Answer)
Explanation: ***Non-necrotizing anterior diffuse*** - This is the **most common type of scleritis**, characterized by widespread inflammation of the anterior sclera without tissue loss. - It presents with **redness**, **pain**, and **tenderness** over a large area of the sclera. *Non-necrotizing anterior nodular* - This type features **localized, tender nodules** on the sclera, which are less common than the diffuse form. - While also non-necrotizing and anterior, the nodular presentation is not the most frequent variety. *Anterior necrotizing without inflammation* - This severe form, known as **scleromalacia perforans**, is rare and typically seen in patients with long-standing rheumatoid arthritis. - It is characterized by **scleral thinning and necrosis** without significant inflammatory signs. *Anterior necrotizing with inflammation* - This is a **severe and destructive form** of scleritis associated with significant pain and potential vision loss. - While serious, it is rare compared to diffuse non-necrotizing anterior scleritis.
Question 8: 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 9: Neurological manifestation of Whipple's disease is:
- A. Encephalopathy (Correct Answer)
- B. Cerebellar ataxia
- C. Seizures
- D. Focal neurological deficits
Explanation: ***Encephalopathy*** - **Encephalopathy** is the most common neurological manifestation of Whipple's disease, involving cognitive dysfunction, memory loss, and altered mental status. - This widespread brain involvement is due to the dissemination of the bacterium *Tropheryma whipplei* throughout the central nervous system. *Cerebellar ataxia* - While **cerebellar ataxia** can occur in Whipple's disease, it is less common than encephalopathy and typically presents as part of a broader neurological syndrome rather than an isolated finding. - Ataxia, when present, often suggests more advanced or disseminated disease. *Seizures* - **Seizures** are a less frequent neurological manifestation and are typically seen in cases with focal lesions or significant inflammatory changes within the brain. - They are not considered the predominant or most common neurological symptom of the disease. *Focal neurological deficits* - **Focal neurological deficits**, such as cranial nerve palsies or hemiparesis, can occur but are generally less common than the diffuse cognitive and mental status changes associated with encephalopathy. - These focal signs usually indicate specific areas of inflammation or granuloma formation.
Question 10: Vertebra Plana is associated with all of the following conditions except -
- A. Leukemia
- B. Excessive use of systemic steroids
- C. Scheurmanns Disease (Correct Answer)
- D. Histiocytosis X
Explanation: ***Scheurmanns Disease*** - **Scheuermann's disease** is characterized by **vertebral wedging** and **kyphosis**, not a complete flattening of the vertebral body (vertebra plana). - It involves irregularities of the vertebral endplates and Schmorl's nodes, differing from the destructive process seen in vertebra plana. *Histiocytosis X* - **Histiocytosis X** (Langerhans cell histiocytosis) can cause destructive lesions in the vertebral body, leading to its collapse and the appearance of **vertebra plana**. - This condition is common among young children and is associated with eosinophilic granuloma. *Leukemia* - **Leukemic infiltration** of bone marrow can weaken vertebral bodies, causing **osteopenia** and eventual collapse, which may present as vertebra plana. - This is often seen in pediatric patients with acute lymphoblastic leukemia. *Excessive use of systemic steroids* - Long-term or excessive use of **systemic corticosteroids** can lead to **osteoporosis**, which weakens bones and makes vertebral bodies prone to compression fractures and collapse into vertebra plana. - This iatrogenic cause results from the negative impact of steroids on bone formation and increased bone resorption.
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