Autoinflammatory Syndromes — MCQs

10 questions

Abatacept, a drug inhibiting co-stimulation is used for:

Amyloidosis is associated with all of the following conditions except:

A 26-year-old pregnant woman presents with arthritis and a malar rash that worsens with sun exposure. She has a low-grade fever and is admitted to the hospital. What is the likely diagnosis?

A patient in ICU and on ventilator develops cough with fever. The gram-staining on microscopy will show:

Sicca syndrome is associated with all except?

Match the following: A) Caplan syndrome- 1) Found first in coal worker B) Asbestosis- 2) Upper lobe predominance C) Mesothelioma- 3) Involves lower lobe D) Sarcoidosis- 4) Pleural effusion is seen

True regarding felty's syndrome is all, except -

Which condition is most commonly associated with systemic amyloidosis?

Which type of amyloidosis is seen in the patients going through dialysis?

Q10

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?

Autoinflammatory Syndromes Indian Medical PG Practice Questions and MCQs

Question 1: Abatacept, a drug inhibiting co-stimulation is used for:

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

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.

Question 2: Amyloidosis is associated with all of the following conditions except:

A. Multiple myeloma
B. Renal failure
C. Chronic inflammatory conditions
D. Acute inflammatory conditions (Correct Answer)

Explanation: ***Acute inflammatory conditions*** - Amyloidosis is primarily associated with chronic inflammatory conditions rather than acute ones, making this option less likely [1]. - Conditions like **chronic infections** and malignancies are more commonly linked to amyloid deposition than acute inflammation [1]. *Multiple myeloma* - A significant association exists between **multiple myeloma** and amyloidosis, as the production of light chains can lead to **AL amyloidosis** [3]. - Patients with myeloma often develop **renal complications** due to amyloid infiltration. *Renal failure* - **Renal failure** is frequently seen in patients with amyloidosis due to renal amyloid deposits, leading to glomerular damage. - The kidneys are one of the primary organs affected in systemic amyloidosis, resulting in significant clinical manifestations. *Alzheimer's disease* - Alzheimer's disease is associated with **amyloid-beta peptide** accumulation, classifying it as AD-related amyloidosis [2]. - This form of amyloidosis is well documented in the literature, making it more relevant compared to acute inflammatory conditions. **References:** [1] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. (Basic Pathology) introduces the student to key general principles of pathology, both as a medical science and as a clinical activity with a vital role in patient care. Part 2 (Disease Mechanisms) provides fundamental knowledge about the cellular and molecular processes involved in diseases, providing the rationale for their treatment. Part 3 (Systematic Pathology) deals in detail with specific diseases, with emphasis on the clinically important aspects., pp. 136-140. [2] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Diseases of the Immune System, pp. 269-270. [3] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Diseases of the Immune System, pp. 266-267.

Question 3: A 26-year-old pregnant woman presents with arthritis and a malar rash that worsens with sun exposure. She has a low-grade fever and is admitted to the hospital. What is the likely diagnosis?

A. Lyme disease
B. Chloasma
C. Systemic Lupus Erythematosus (Correct Answer)
D. Steven Johnsons syndrome

Explanation: ***Systemic Lupus Erythematosus*** - The combination of **arthritis**, **malar rash** that worsens with sun exposure (photosensitivity), and a **low-grade fever** in a young woman is highly characteristic of systemic lupus erythematosus (SLE) [1]. - SLE is an **autoimmune disease** with diverse clinical manifestations affecting multiple organ systems [1]. *Lyme disease* - Characterized by a **target-like rash (erythema migrans)**, which is distinct from a malar rash, and often presents with flu-like symptoms. - While Lyme disease can cause arthritis, the presence of a classic malar rash and photosensitivity points away from this diagnosis. *Chloasma* - Also known as the **"mask of pregnancy,"** chloasma is a common skin condition in pregnant women causing dark, discolored patches on the face. - It is a **pigmentation disorder** and does not involve arthritis, fever, or an associated malar rash that worsens with sun exposure. *Steven Johnsons syndrome* - An infrequent, serious systemic reaction to medication or infection, that triggers severe skin and mucous membrane reactions, typically presenting with **widespread blistering** and epidermal detachment. - This is an **acute, severe mucocutaneous reaction** and does not present with the chronic arthritis and photosensitive malar rash seen in this patient.

Question 4: A patient in ICU and on ventilator develops cough with fever. The gram-staining on microscopy will show:

A. Gram negative cocci
B. Gram negative bacilli (Correct Answer)
C. Gram variable organism
D. Gram positive bacilli

Explanation: ***Gram negative bacilli*** - **Ventilator-associated pneumonia (VAP)** is most commonly caused by **Gram-negative bacilli**, particularly in late-onset VAP (>5 days of mechanical ventilation). - Common pathogens include *Pseudomonas aeruginosa*, *Klebsiella pneumoniae*, *Escherichia coli*, and *Acinetobacter baumannii*, which are prevalent in the ICU environment. - These organisms commonly colonize the oropharynx of critically ill, intubated patients and can be aspirated into the lower respiratory tract, leading to pneumonia. - Gram-negative bacilli account for the **majority of VAP cases**, making this the most likely microscopic finding. *Gram negative cocci* - **Gram-negative cocci** like *Neisseria meningitidis* and *Neisseria gonorrhoeae* are primary causes of meningitis and sexually transmitted infections, respectively, and are not typical pathogens for VAP. - While *Moraxella catarrhalis* is a Gram-negative coccobacillus that can cause respiratory infections, it is uncommon in severe VAP cases compared to Gram-negative bacilli. *Gram variable organism* - The term **"Gram variable"** is rarely used in clinical microbiology to describe a consistent bacterial morphology; it usually refers to mixed staining results where some cells stain Gram-positive and others Gram-negative. - This description does not fit the typical profile of bacteria causing VAP and is not a standard morphological category for common VAP pathogens. *Gram positive bacilli* - **Gram-positive bacilli**, such as *Bacillus* species or *Corynebacterium* species, are generally not recognized as common causes of VAP. - Note: While **Gram-positive cocci** like *Staphylococcus aureus* (including MRSA) and *Streptococcus pneumoniae* are important VAP pathogens, they would appear as **cocci**, not bacilli, on microscopy. - The question specifically asks about morphology on Gram staining, and Gram-positive **bacilli** are not the predominant morphological pattern in VAP.

Question 5: Sicca syndrome is associated with all except?

A. Chronic active hepatitis
B. Midline granuloma (Correct Answer)
C. Rheumatoid arthritis
D. Scleroderma

Explanation: Sicca syndrome is associated with all except? ***Midline granuloma*** - **Midline granuloma** (now commonly referred to as **extranodal NK/T-cell lymphoma, nasal type**) is a destructive inflammatory process primarily affecting the upper respiratory tract. It is not directly associated with the autoimmune features of sicca syndrome. - Its pathology involves an aggressive **lymphoproliferative disorder**, distinct from the glandular dysfunction seen in sicca syndrome. *Chronic active hepatitis* - **Primary biliary cholangitis** (formerly primary biliary cirrhosis), a form of chronic active hepatitis, is strongly associated with sicca syndrome. Patients often experience dry eyes and mouth alongside liver involvement. - Autoimmune hepatitis can also present with **extraglandular manifestations** including sicca symptoms, highlighting a systemic autoimmune link. *Rheumatoid arthritis* - **Secondary Sjögren's syndrome**, which manifests as sicca symptoms, commonly occurs in patients with **rheumatoid arthritis (RA)**. This is a well-recognized overlap. - The inflammatory processes common to both conditions suggest a shared **autoimmune pathogenesis**. *Scleroderma* - **Systemic sclerosis (scleroderma)** is another autoimmune connective tissue disease that frequently overlaps with Sjögren's syndrome and features of sicca [1]. - Patients with scleroderma often report **dry eyes and mouth**, reflecting autoimmune involvement of the exocrine glands.

Question 6: Match the following: A) Caplan syndrome- 1) Found first in coal worker B) Asbestosis- 2) Upper lobe predominance C) Mesothelioma- 3) Involves lower lobe D) Sarcoidosis- 4) Pleural effusion is seen

A. A-3, B-4, C-2, D-1
B. A-1, B-4, C-3, D-2 (Correct Answer)
C. A-4, B-2, C-3, D-1
D. A-2, B-4, C-3, D-1

Explanation: **A-1, B-4, C-3, D-2** - **Caplan syndrome** was first described in **coal workers** with **rheumatoid arthritis** and progressive massive fibrosis. - **Asbestosis** is often associated with **pleural effusion**, which can be benign or malignant. - **Mesothelioma** typically involves the **lower lobes** of the lungs, specifically the pleura, and is strongly linked to asbestos exposure. - **Sarcoidosis** is characterized by **non-caseating granulomas**, which have a predilection for the **upper lobes** of the lungs. *A-3, B-4, C-2, D-1* - This option incorrectly states that Caplan syndrome involves the lower lobe; **Caplan syndrome** is defined by the presence of large nodules in the lungs of coal workers with rheumatoid arthritis, and their specific lobar distribution is not a defining characteristic. - This option incorrectly states that Mesothelioma has an upper lobe predominance; **Mesothelioma** is a pleural malignancy and typically involves the **lower lobes**, extending along the pleura. *A-4, B-2, C-3, D-1* - This option incorrectly associates Caplan syndrome with pleural effusion; **Caplan syndrome** manifests as rheumatoid nodules in the lungs, not primarily pleural effusion. - This option incorrectly states that Asbestosis has an upper lobe predominance; **Asbestosis** predominantly affects the **lower lobes** of the lungs, causing interstitial fibrosis. *A-2, B-4, C-3, D-1* - This option incorrectly states that Caplan syndrome has an upper lobe predominance; the defining feature of **Caplan syndrome** is the combination of rheumatoid arthritis and pneumoconiosis, not specific lobar involvement. - This option correctly identifies pleural effusion with asbestosis and lower lobe involvement with mesothelioma, but **Caplan syndrome** is not characterized by upper lobe predominance.

Question 7: 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].

Question 8: Which condition is most commonly associated with systemic amyloidosis?

A. Chronic Kidney Disease (Correct Answer)
B. Multiple Myeloma
C. Rheumatoid Arthritis
D. Familial Mediterranean Fever

Explanation: Type II DM - Amyloidosis is commonly associated with **chronic diseases** like type II diabetes mellitus due to insulin resistance leading to amyloid deposition [4]. - The presence of **amiloid in the pancreas** often correlates with the complications of this type of diabetes [3]. *Maturity onset DM* - While maturity-onset diabetes can lead to complications, it is often synonymous with **type II DM**, making this distinction inaccurate regarding amyloidosis. - This term is less commonly used and does not emphasize the direct link to amyloidosis seen in type II diabetes. *HTN* - Hypertension itself is not a direct cause of amyloidosis; it typically results from other underlying conditions. - The association of amyloidosis with hypertension is usually **secondary**, not a primary condition leading to amyloid deposits [2]. *Type I DM* - Type I diabetes is primarily **autoimmune**, resulting in insulin-deficient states and does not strongly associate with amyloidosis like type II [1]. - The amyloid deposits seen in type I are much less common compared to type II or associated chronic conditions.

Question 9: Which type of amyloidosis is seen in the patients going through dialysis?

A. A-beta
B. AL
C. A-beta 2 (Correct Answer)
D. aTTR

Explanation: ***A-beta 2*** - **A-beta 2 microglobulin amyloidosis** (also known as dialysis-related amyloidosis) occurs because **beta-2 microglobulin** is not effectively cleared by dialysis and accumulates in tissues [1]. - This condition primarily affects **joints, bones**, and **tendons** in long-term dialysis patients, leading to carpal tunnel syndrome, arthropathy, and bone cysts. *A-beta* - **A-beta amyloidosis** refers to the accumulation of **amyloid-beta peptides** that are characteristic of **Alzheimer's disease**, primarily affecting the brain. - This type of amyloidosis is not directly associated with renal dialysis or systemic amyloid deposits in other organs. *AL* - **AL (light chain) amyloidosis** results from the deposition of **monoclonal immunoglobulin light chains** produced by plasma cells, often associated with multiple myeloma. - While it can affect the kidneys, it is a primary amyloidosis and not caused by dialysis itself, though it can occur in patients who also have kidney failure. *aTTR* - **aTTR (transthyretin) amyloidosis** involves the deposition of **abnormal transthyretin protein**, which can be hereditary (mutated TTR) or wild-type (aging-related) [1]. - This form primarily affects the heart and nervous system and is not typically associated with chronic dialysis as its direct cause.

Question 10: 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.

Want unlimited practice?

Get full access to all questions, explanations, and performance tracking.

Start For Free