Sjögren's Syndrome — MCQs

10 questions

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

Regarding Sjögren's syndrome, all are true except:

All of the following are associated with secondary Sjogren's syndrome EXCEPT

Development of Lymphoma in Sjogren's syndrome is suggested by all of the following except

A 48-year-old female presents to your office with a 1-year history of dry eyes and difficulty swallowing. She complains of blinking frequently and of eye strain while using her computer at work. She also reports stiffness in her knees and lower back. Past medical history is unremarkable and she does not take medications. She denies cigarette or alcohol use. Family history is notable for Hashimoto's thyroiditis in her mother. Physical exam shows dry oral mucosa and enlargement of the parotid glands. Which of the following serologies is likely to be positive in this patient?

Which of the following is not a primary symptom of Sjögren's syndrome?

A young male presents with acute redness of the eye. His X-ray of the spine is shown below. What is the most likely condition?

Microscopically, epimyoepithelial islands are typically seen in:

Q10

True regarding felty's syndrome is all, except -

Sjögren's Syndrome Indian Medical PG Practice Questions and MCQs

Question 1: 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 2: 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 3: Regarding Sjögren's syndrome, all are true except:

A. Rheumatoid arthritis
B. Keratoconjunctivitis sicca
C. Epiphora (Correct Answer)
D. Autoimmune in nature

Explanation: ***Epiphora*** - **Epiphora** refers to excessive tearing or watery eyes, which is not characteristic of Sjögren's syndrome. - Sjögren's syndrome is instead characterized by **dry eyes** due to decreased tear production. *Rheumatoid arthritis* - Sjögren's syndrome can occur as a **secondary condition** associated with other autoimmune diseases, including rheumatoid arthritis [1]. - This association means that individuals with rheumatoid arthritis have an increased risk of developing Sjögren's syndrome [1]. *Keratoconjunctivitis sicca* - **Keratoconjunctivitis sicca** (dry eyes) is a hallmark symptom of Sjögren's syndrome, resulting from inflammation and damage to the lacrimal glands [1]. - Patients experience chronic dryness, foreign body sensation, and irritation due to insufficient tear production [1]. *Autoimmune in nature* - Sjögren's syndrome is an **autoimmune disease** where the immune system mistakenly attacks the body's own moisture-producing glands, primarily the lacrimal and salivary glands. - This autoimmune attack leads to chronic inflammation and dysfunction of these glands, causing symptoms of dryness.

Question 4: All of the following are associated with secondary Sjogren's syndrome EXCEPT

A. Systemic lupus erythematosus
B. Primary biliary cirrhosis
C. Rheumatoid arthritis
D. Pheochromocytoma (Correct Answer)

Explanation: ***Pheochromocytoma*** - **Pheochromocytoma** is a tumor of the adrenal medulla that causes excessive secretion of **catecholamines** and is not directly associated with **secondary Sjögren's syndrome**. - Its clinical manifestations include **hypertension**, **tachycardia**, and **sweating**, which are distinct from autoimmune features. *Systemic lupus erythematosus* - **Systemic lupus erythematosus (SLE)** is a common autoimmune disease frequently associated with **secondary Sjögren's syndrome** [1]. - Patients with SLE can develop **sicca symptoms** (dry eyes, dry mouth) as a manifestation of secondary Sjögren's [1]. *Primary biliary cirrhosis* - **Primary biliary cirrhosis (PBC)** is an **autoimmune liver disease** that often coexists with other autoimmune conditions, including **secondary Sjögren's syndrome**. - Approximately 70% of patients with PBC have **sicca symptoms**, indicating an association with Sjögren's. *Rheumatoid arthritis* - **Rheumatoid arthritis (RA)** is a chronic inflammatory autoimmune disease that is strongly associated with **secondary Sjögren's syndrome**. - Up to 30% of RA patients develop **dry eyes and mouth**, fulfilling the criteria for secondary Sjögren's.

Question 5: Development of Lymphoma in Sjogren's syndrome is suggested by all of the following except

A. Leukopenia
B. Cryoglobulinemia
C. Persistent parotid gland enlargement
D. High C4 complement levels (Correct Answer)

Explanation: High C4 complement levels - **High C4 complement levels** are not typically associated with an increased risk or development of lymphoma in Sjögren's syndrome. In fact, **low C4** (due to consumption) is more commonly linked to complications like vasculitis or cryoglobulinemia, which can sometimes precede lymphoma. - Decreased complement levels, particularly **C4**, are often seen in Sjögren's patients with **lymphoproliferative disorders** or **cryoglobulinemia**, making elevated levels an unlikely indicator of lymphoma. Leukopenia - **Leukopenia** (low white blood cell count) can be a significant indicator of developing **lymphoma** in patients with Sjögren's syndrome. It may reflect bone marrow infiltration or a systemic effect of the underlying disease. - Persistent or worsening **leukopenia** should prompt further investigation for lymphoproliferative disorders. Cryoglobulinemia - **Cryoglobulinemia** is a well-established risk factor and a paraneoplastic manifestation strongly associated with the development of **B-cell non-Hodgkin lymphoma** in Sjögren's syndrome [1]. - The presence of **cryoglobulins** indicates abnormal B-cell activity, which can evolve into overt lymphoma. Persistent parotid gland enlargement - **Persistent parotid gland enlargement** in a Sjögren's patient is a critical warning sign for potential **lymphoma**, especially **mucosa-associated lymphoid tissue (MALT) lymphoma** [1]. - This symptom warrants a **biopsy** to rule out malignancy, as it often signifies increased lymphoid proliferation within the salivary glands.

Question 6: A 48-year-old female presents to your office with a 1-year history of dry eyes and difficulty swallowing. She complains of blinking frequently and of eye strain while using her computer at work. She also reports stiffness in her knees and lower back. Past medical history is unremarkable and she does not take medications. She denies cigarette or alcohol use. Family history is notable for Hashimoto's thyroiditis in her mother. Physical exam shows dry oral mucosa and enlargement of the parotid glands. Which of the following serologies is likely to be positive in this patient?

A. Anti-Jo-1
B. Anti-dsDNA
C. Anti-SS-A (Correct Answer)
D. Anti-Smith
E. Anti-CCP

Explanation: ***Anti-SS-A*** * This patient's symptoms of **dry eyes**, **difficulty swallowing**, **dry oral mucosa**, and **enlarged parotid glands** are highly indicative of **Sjögren's syndrome** [1]. * **Anti-SS-A (Ro)** antibodies are present in 60-70% of patients with primary Sjögren's syndrome and are recognized as a diagnostic marker [1]. * *Anti-Jo-1* * **Anti-Jo-1** antibodies are primarily associated with **polymyositis** and **dermatomyositis**, particularly the anti-synthetase syndrome, characterized by muscle weakness and interstitial lung disease, which are not described in this patient. * The patient's symptoms are focused on sicca manifestations rather than muscle inflammation. * *Anti-dsDNA* * **Anti-dsDNA** antibodies are highly specific for **Systemic Lupus Erythematosus (SLE)** and are often associated with lupus nephritis. * The patient presents with symptoms more consistent with a primary sicca disorder rather than the multi-systemic involvement typical of SLE. * *Anti-Smith* * **Anti-Smith** antibodies are also highly specific for **Systemic Lupus Erythematosus (SLE)**, though less sensitive than anti-dsDNA [1]. * These antibodies are not typically found in Sjögren's syndrome without co-existing SLE. * *Anti-CCP* * **Anti-CCP (cyclic citrullinated peptide)** antibodies are highly specific for **rheumatoid arthritis** [2]. * While the patient reports knee and lower back stiffness, the primary complaints and physical findings point towards Sjögren's syndrome, not inflammatory arthritis.

Question 7: 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 8: A young male presents with acute redness of the eye. His X-ray of the spine is shown below. What is the most likely condition?

A. Psoriatic Arthritis
B. Rheumatoid Arthritis
C. Sjögren Syndrome
D. Ankylosing Spondylitis (Correct Answer)

Explanation: ***Ankylosing Spondylitis*** - The young male presenting with **acute redness of the eye** (uveitis) and a spinal X-ray showing changes consistent with **bamboo spine** (fusion of vertebrae due to syndesmophyte formation) is highly indicative of ankylosing spondylitis. - This condition is a chronic inflammatory disorder primarily affecting the **axial skeleton** (spine and sacroiliac joints) and is often associated with extra-articular manifestations like **uveitis**. *Psoriatic Arthritis* - While psoriatic arthritis can affect the spine and cause uveitis, the characteristic imaging findings in the provided X-ray, particularly the **bamboo spine**, are more classical for ankylosing spondylitis. - Psoriatic spondylitis often presents with more asymmetric and less diffuse spinal involvement compared to ankylosing spondylitis. *Rheumatoid Arthritis* - Rheumatoid arthritis primarily affects the **peripheral joints** (e.g., small joints of hands and feet) and typically spares the axial skeleton, except for involvement of the **cervical spine**. - **Uveitis** is a rare extra-articular manifestation in rheumatoid arthritis, and the X-ray findings are not typical for this condition. *Sjögren Syndrome* - Sjögren syndrome is a chronic autoimmune disease characterized by **dry eyes** (keratoconjunctivitis sicca) and **dry mouth** (xerostomia) due to lymphocytic infiltration of exocrine glands. - While it can cause some joint pain, it does not typically lead to the characteristic spinal changes seen on the X-ray or acute uveitis.

Question 9: 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.

Question 10: True regarding felty's syndrome is all, except -

A. Nephropathy (Correct Answer)
B. Rheumatoid arthritis
C. Neutropenia
D. Splenomegaly

Explanation: ***Nephropathy*** - **Nephropathy** is not a characteristic component of Felty's syndrome; the triad specifically involves rheumatoid arthritis, neutropenia, and splenomegaly [1]. - While patients with rheumatoid arthritis can develop renal complications, they are not directly linked to the definition of Felty's syndrome itself [1]. *Rheumatoid arthritis* - **Rheumatoid arthritis** is a prerequisite for diagnosing Felty's syndrome, as it represents the underlying chronic inflammatory disease [1]. - Patients typically have severe, long-standing, and often seropositive rheumatoid arthritis [1]. *Neutropenia* - **Neutropenia** (low neutrophil count) is a hallmark of Felty's syndrome, contributing to an increased risk of infections [1]. - The neutropenia is often accompanied by active rheumatoid arthritis and splenomegaly [1]. *Splenomegaly* - **Splenomegaly** (enlarged spleen) is the third essential component of the classic triad in Felty's syndrome [1]. - The enlarged spleen is thought to contribute to the neutropenia by sequestering or destroying neutrophils [1].

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