Sjögren's Syndrome — MCQs
A 45-year-old female presents with dry eyes, dry mouth, and joint pain. Which of the following antibodies is most indicative of the suspected condition?
Which of the following is NOT a connective tissue disorder?
A 45-year-old woman with long-standing rheumatoid arthritis complains of dry eyes and dry mouth. Bilateral enlargement of the parotids is noted on physical examination. The syndrome described here is best described as
35 year old female patient complains of dry mouth and scratchy feeling in eyes. She is found to have antibodies against anti- SSA/Ro. Which of the following can be the most accurate sole criterion to diagnose the condition
Which of the following drugs is least likely to cause sicca syndrome?
A patient presents with eye ache and difficulty in vision after watching a movie. What will be the first line of management?
Which of the following is a characteristic feature of primary Sjogren's syndrome?
Which of the following is not a primary symptom of Sjögren's syndrome?
The technique involving injection of contrast material for evaluation of salivary glands is called:
Microscopically, epimyoepithelial islands are typically seen in:
Sjögren's Syndrome Indian Medical PG Practice Questions and MCQs
Question 1: A 45-year-old female presents with dry eyes, dry mouth, and joint pain. Which of the following antibodies is most indicative of the suspected condition?
- A. Anti-Ro/SSA antibodies (Correct Answer)
- B. Anti-histone antibodies
- C. Anti-thyroid peroxidase antibodies
- D. Anti-phospholipid antibodies
Explanation: ***Anti-Ro/SSA antibodies*** - The patient's symptoms of **dry eyes (xerophthalmia)**, **dry mouth (xerostomia)**, and **joint pain** are classic manifestations of **Sjögren's syndrome**, a chronic autoimmune disease. [1] - **Anti-Ro/SSA antibodies** are highly specific for Sjögren's syndrome, particularly in patients with primary Sjögren's. [1] *Anti-histone antibodies* - These antibodies are most commonly associated with **drug-induced lupus erythematosus**, a condition characterized by lupus-like symptoms that develop after exposure to certain medications. - The clinical presentation of dry eyes and dry mouth is not the primary distinguishing feature of drug-induced lupus. *Anti-thyroid peroxidase antibodies* - These antibodies are a hallmark of **Hashimoto's thyroiditis**, an autoimmune disease affecting the thyroid gland and leading to hypothyroidism. [2] - While Sjögren's syndrome can coexist with other autoimmune conditions, anti-thyroid peroxidase antibodies do not directly indicate Sjögren's. *Anti-phospholipid antibodies* - These antibodies are primarily associated with **antiphospholipid syndrome**, a thrombophilic disorder characterized by arterial or venous thrombosis and/or adverse pregnancy outcomes. - They are not directly indicative of the sicca symptoms seen in Sjögren's syndrome.
Question 2: 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 3: A 45-year-old woman with long-standing rheumatoid arthritis complains of dry eyes and dry mouth. Bilateral enlargement of the parotids is noted on physical examination. The syndrome described here is best described as
- A. Metastatic
- B. Metabolic
- C. Infectious
- D. Autoimmune (Correct Answer)
Explanation: ***Autoimmune*** - The combination of **dry eyes (xerophthalmia)**, **dry mouth (xerostomia)**, **parotid enlargement**, and a history of **rheumatoid arthritis** points to **Sjögren's syndrome**, which is a systemic autoimmune disease characterized by lymphocytic infiltration of exocrine glands. - Sjögren's syndrome is frequently associated with other autoimmune diseases, with **rheumatoid arthritis** being the most common secondary autoimmune condition [1]. *Metastatic* - **Metastatic** refers to the spread of cancer from its primary site to other parts of the body. - The patient's symptoms of dry eyes, dry mouth, and parotid enlargement are not typical manifestations of metastatic disease. *Metabolic* - **Metabolic** conditions involve disturbances in the body's chemical processes, such as diabetes or thyroid disorders. - While some metabolic conditions can cause systemic symptoms, the specific constellation of dry eyes, dry mouth, and parotid enlargement is not characteristic of a primary metabolic disorder. *Infectious* - **Infectious** diseases are caused by pathogenic microorganisms. - Although some infections can cause dry mouth or parotid gland enlargement (e.g., mumps), the chronic nature of the symptoms, associated dry eyes, and chronic rheumatoid arthritis are inconsistent with an acute infectious process.
Question 4: 35 year old female patient complains of dry mouth and scratchy feeling in eyes. She is found to have antibodies against anti- SSA/Ro. Which of the following can be the most accurate sole criterion to diagnose the condition
- A. Objective measurement of lacrimal and salivary gland secretion
- B. Lip tissue biopsy (Correct Answer)
- C. Lymph node biopsy
- D. Positive rheumatoid factor
Explanation: Lip tissue biopsy - A lip biopsy demonstrating focal lymphocytic sialadenitis in the minor salivary glands is a gold standard for diagnosing Sjogren's syndrome, especially when other criteria are met. - This pathological finding provides definitive evidence of the characteristic autoimmune inflammation affecting the exocrine glands. Objective measurement of lacrimal and salivary gland secretion - While important for evaluating the severity of dryness (e.g., Schirmer's test for eyes, sialometry for saliva), these are functional measurements and not a sole diagnostic criterion. - They can be affected by other factors and do not directly confirm the autoimmune pathology like a biopsy does. Lymph node biopsy - A lymph node biopsy is not a standard diagnostic procedure for Sjogren's syndrome. - It might be performed if lymphoma is suspected, as Sjogren's patients have an increased risk, but it's not used for initial diagnosis of the syndrome itself. Positive rheumatoid factor - A positive rheumatoid factor (RF) is common in Sjogren's syndrome (present in 75-90% of patients), but it is not specific and can be elevated in other autoimmune diseases (e.g., rheumatoid arthritis) or even in healthy individuals. - Therefore, it cannot be used as a sole diagnostic criterion. Antibodies to Ro (SSA) are characteristic of the condition and are often found in association with anti-La antibodies [1].
Question 5: Which of the following drugs is least likely to cause sicca syndrome?
- A. Atropine
- B. Nicotine (Correct Answer)
- C. Olanzapine
- D. Clonidine
Explanation: ***Nicotine*** - **Nicotine** is a **nicotinic cholinergic receptor agonist** that acts primarily at autonomic ganglia and the neuromuscular junction. - Unlike antimuscarinic drugs, nicotine **does NOT block parasympathetic glandular secretion** and is **not associated with causing sicca syndrome**. - While nicotine has complex autonomic effects, it does **not significantly reduce salivary or lacrimal gland secretion**, making it the **least likely** among these options to cause sicca syndrome. - The question asks for the drug *least likely* to cause sicca syndrome, and nicotine's mechanism of action does not involve blocking secretory glands. *Atropine* - **Atropine** is a potent **muscarinic acetylcholine receptor antagonist** with strong anticholinergic effects. - It directly **blocks parasympathetic stimulation** of salivary and lacrimal glands, leading to marked reduction in secretions. - **Dry mouth (xerostomia) and dry eyes (xerophthalmia)** are classic and prominent side effects, making atropine one of the **most likely drugs to cause sicca syndrome**. *Olanzapine* - **Olanzapine** is an atypical antipsychotic with **moderate anticholinergic properties** due to muscarinic M1 receptor blockade. - **Dry mouth is a common side effect** occurring in 20-30% of patients taking olanzapine. - While less potent than atropine, olanzapine's anticholinergic effects make it **likely to contribute to sicca symptoms**. *Clonidine* - **Clonidine** is an **alpha-2 adrenergic agonist** that reduces sympathetic outflow centrally. - **Dry mouth (xerostomia)** is one of the **most common side effects** of clonidine, occurring in up to 40% of patients. - The mechanism involves decreased salivary gland secretion, making clonidine **highly likely to cause sicca syndrome symptoms**.
Question 6: A patient presents with eye ache and difficulty in vision after watching a movie. What will be the first line of management?
- A. Mannitol with Moxifloxacin
- B. Mannitol with Atropine
- C. Mannitol with lubricating eye drops
- D. Mannitol with Pilocarpine (Correct Answer)
Explanation: ***Mannitol with Pilocarpine*** - This combination is appropriate for **acute angle-closure glaucoma (AACG)**, which can be triggered by pupillary dilation (e.g., in a dark movie theater). **Mannitol** is an osmotic diuretic that rapidly reduces intraocular pressure. - **Pilocarpine** is a miotic agent that constricts the pupil, pulling the iris away from the trabecular meshwork and opening the drainage angle to facilitate aqueous humor outflow. *Mannitol with Moxifloxacin* - While mannitol helps with intraocular pressure, **Moxifloxacin is an antibiotic** used to treat bacterial infections. - There is no indication of an ocular infection in this scenario, so an antibiotic would not be the first-line treatment for sudden eye pain and vision difficulty after watching a movie. *Mannitol with Atropine* - Adding **Atropine, a cycloplegic agent**, would cause further pupillary dilation, which would worsen acute angle-closure glaucoma and increase intraocular pressure. - Atropine is contraindicated in AACG and would exacerbate the patient's condition. *Mannitol with lubricating eye drops* - While mannitol helps with intraocular pressure, **lubricating eye drops** are used for dry eyes or surface irritation, not for acute angle-closure glaucoma. - Lubricating drops do not address the underlying pathology of increased intraocular pressure due to angle closure.
Question 7: Which of the following is a characteristic feature of primary Sjogren's syndrome?
- A. Can occur in children
- B. Increased complement C4 is associated with thymoma
- C. Can be associated with rheumatoid arthritis
- D. Enlargement of salivary glands (Correct Answer)
Explanation: ***Enlargement of salivary glands*** - **Sjögren's syndrome** is characterized by chronic inflammation of **exocrine glands**, leading to swelling and dysfunction, most notably in the **parotid** and **submandibular glands**. - This glandular enlargement often presents as recurrent or persistent **bilateral swelling** of the major salivary glands. *Can occur in children* - While possible, Sjögren's syndrome is predominantly a disease of **adults**, with incidence peaking in individuals between **40 and 60 years old**. - **Juvenile Sjögren's syndrome** is rare and typically presents with more variable symptoms, making it less of a characteristic feature of the primary disease in the general population. *Increased complement C4 is associated with thymoma* - **Increased complement C4** levels are generally not associated with Sjögren's syndrome; instead, **hypocomplementemia** (low C4) can be seen in some patients, indicating immune complex activity. - **Thymoma** is primarily linked to **myasthenia gravis** and other paraneoplastic syndromes, not a direct or characteristic association with Sjögren's syndrome or C4 levels in this context. *Can be associated with rheumatoid arthritis* - Sjögren's syndrome can be classified as **primary** (occurring alone) or **secondary** (occurring in conjunction with another autoimmune disease). - Its association with **rheumatoid arthritis** defines **secondary Sjögren's syndrome**, meaning it's not a characteristic feature of the *primary* form of the disease itself.
Question 8: Which of the following is not a primary symptom of Sjögren's syndrome?
- A. Parotid gland enlargement
- B. Dry eyes
- C. Dry mouth
- D. Systemic manifestations (Correct Answer)
Explanation: ***Systemic manifestations*** - While Sjögren's syndrome can have **systemic manifestations**, they are secondary complications or features, not among the **primary, defining symptoms** that establish the diagnosis. - The primary symptoms directly relate to **exocrine gland dysfunction**, specifically lacrimal and salivary glands. *Dry eyes* - This is a cardinal symptom, resulting from damage to the **lacrimal glands** leading to decreased tear production. - Patients typically report a **gritty or burning sensation** in their eyes. *Dry mouth* - Another core symptom, resulting from dysfunction of the **salivary glands** and reduced saliva flow. - This can lead to difficulties in **speaking, swallowing**, increased dental carries, and oral discomfort. *Parotid gland enlargement* - This is a common and often **visible clinical sign** of Sjögren's syndrome, indicating inflammation of the **major salivary glands**. - It results from lymphocytic infiltration and is typically recurrent or persistent.
Question 9: The technique involving injection of contrast material for evaluation of salivary glands is called:
- A. Angiogram
- B. Tomography
- C. Ultrasound
- D. Sialography (Correct Answer)
Explanation: **Sialography** - This technique specifically involves the injection of **radiopaque contrast material** into the salivary ducts (typically Stensen's or Wharton's ducts). - It is used to visualize the **ductal system and parenchyma** of the major salivary glands, aiding in the diagnosis of conditions like **sialolithiasis**, strictures, or tumors. *Angiogram* - An angiogram is a medical imaging technique used to visualize the **blood vessels**, including arteries and veins, using contrast material. - It is primarily for evaluating **vascular pathology** and not the ductal system of salivary glands. *Tomography* - Tomography is a general term for imaging techniques (like CT or MRI) that produce **cross-sectional images** of the body. - While it can visualize salivary glands, it does not involve the direct injection of contrast into the salivary ducts for ductal evaluation unless specifically combined with sialography. *Ultrasound* - Ultrasound uses **sound waves** to create images of internal body structures. - It is a useful non-invasive tool for evaluating salivary glands for masses or inflammation but does not involve **contrast injection into the ducts** to map the ductal system.
Question 10: Microscopically, epimyoepithelial islands are typically seen in:
- A. Epithelial-myoepithelial carcinoma
- B. Myoepithelioma
- C. Mucoepidermoid carcinoma
- D. Sjögren's syndrome (Correct Answer)
Explanation: ***Sjögren's syndrome*** - **Epimyoepithelial islands** are a characteristic histopathological feature of **Sjögren's syndrome**, particularly in affected salivary glands. [1] - These islands represent **benign lymphoepithelial lesions** where ducts are surrounded by lymphocytes, eventually forming true islands. *Epithelial-myoepithelial carcinoma* - This is a **malignant salivary gland tumor** with dual differentiation, but it typically presents as distinct inner epithelial and outer myoepithelial layers around ducts or cords, not as true "islands." - While it involves both epithelial and myoepithelial cells, its arrangement and neoplastic nature differ from the benign epimyoepithelial islands of Sjögren's. *Myoepithelioma* - This is a **benign tumor composed predominantly of myoepithelial cells**, often appearing in various morphological patterns (spindle, plasmacytoid, epithelioid, clear cell). - It does not typically form the well-defined **lymphoepithelial islands** seen in Sjögren's, as its characteristic is the proliferation of myoepithelial cells in a different architectural pattern. *Mucoepidermoid carcinoma* - This is a common **malignant salivary gland tumor** characterized by a mixture of mucin-producing cells, epidermoid cells, and intermediate cells. - Its histological features are distinct and do not include the formation of **epimyoepithelial islands**, which are diagnostically specific to Sjögren's syndrome. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Diseases of the Immune System, pp. 235-236.
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