Rheumatology and Immunology Practice Questions

Q: Which category of patients with juvenile idiopathic arthritis, who are mostly HLA-B27 positive and present with enthesitis, lower limb arthritis involving the knees and ankles, and inflammatory low back pain, are most likely to be?

Enthesitis related arthritis. **Enthesitis related arthritis** * **Enthesitis-related arthritis (ERA)** in juvenile idiopathic arthritis (JIA) is characterized by **enthesitis** (inflammation at tendon/ligament insertion sites), **lower limb arthritis** (often of knees and ankles), and **inflammatory low back pain** [1]. * This subtype of JIA is strongly associated with **HLA-B27 positivity**, aligning perfectly with the patient's presentation [1]. *Oligoarticular JIA* * **Oligoarticular JIA** involves **four or fewer joints** during the first six months of disease and typically affects large joints, but enthesitis and inflammatory back pain are not primary features [1]. * While it can involve the knee, the combination of enthesitis and low back pain, especially with HLA-B27 positivity, points away from oligoarticular JIA. *Polyarticular JIA (RF negative)* * **Polyarticular JIA (RF negative)** affects **five or more joints** within the first six months and is characterized by a widespread inflammatory arthritis, but **enthesitis and significant spinal involvement** are not its defining features [1]. * This subtype also doesn't have the strong association with **HLA-B27** seen in the described case. *Systemic JIA* * **Systemic JIA** is characterized by prominent **systemic features** like daily fevers, rash, lymphadenopathy, and serositis, which are not mentioned in the patient's presentation [1]. * While systemic JIA can have joint involvement, the specific combination of **enthesitis, lower limb arthritis, inflammatory back pain, and HLA-B27 positivity** is not typical of this subtype.

Q: HLA associated with rheumatoid arthritis is?

HLA DR 4. ***HLA DR 4*** - **HLA-DR4** is the **most strongly associated HLA allele** with **rheumatoid arthritis (RA)**, particularly in individuals of European descent. - The presence of this allele increases an individual's susceptibility to developing RA and is often linked to more severe disease. *HLA DR2* - **HLA-DR2** is associated with other autoimmune conditions like **multiple sclerosis** and **narcolepsy**, but not primarily with rheumatoid arthritis. - While it is an HLA class II allele, its role in RA susceptibility is not significant. *HLA B27* - **HLA-B27** is a Class I major histocompatibility complex (MHC) allele **strongly associated with seronegative spondyloarthropathies**, such as ankylosing spondylitis and reactive arthritis [1]. - It is not directly linked to rheumatoid arthritis, which is typically seropositive (RF and anti-CCP positive). *HLA-B9* - **HLA-B9** is not a commonly recognized or significant HLA allele associated with increased risk for any specific autoimmune disease, including rheumatoid arthritis. - No strong clinical or genetic association exists between HLA-B9 and RA.

Q: Joint not involved in RA ?

DIP. ***DIP*** - The **distal interphalangeal (DIP)** joints are characteristically spared in **rheumatoid arthritis (RA)**, which helps differentiate it from other inflammatory arthropathies like psoriatic arthritis [1]. - Involvement of the DIP joints would suggest a different diagnosis, such as **osteoarthritis** or **psoriatic arthritis** [3]. *PIP* - The **proximal interphalangeal (PIP)** joints are very commonly involved in **rheumatoid arthritis (RA)**, causing pain, swelling, and characteristic **boutonnière** or **swan neck deformities** [1], [2]. - RA typically causes symmetrical polyarthritis of the small joints, with PIP joints being a primary target [2]. *Wrist* - The **wrist joints** are frequently affected in **rheumatoid arthritis (RA)**, often leading to significant pain, swelling, and limited range of motion. - Inflammatory changes in the wrist contribute to deformities and functional impairment seen in RA patients. *MCP* - The **metacarpophalangeal (MCP)** joints are a hallmark site of involvement in **rheumatoid arthritis (RA)**, experiencing early and significant inflammation [2]. - Involvement of the MCP joints often leads to characteristic **ulnar deviation** of the fingers.

Q: Palpable purpura is seen in all conditions except

Giant cell arteritis. ***Giant cell arteritis*** - **Giant cell arteritis** primarily affects **large- and medium-sized arteries**, most commonly the **temporal artery**. - It typically manifests with **headache**, **jaw claudication**, and **visual disturbances**, but **palpable purpura** is not a characteristic skin finding. *Cryoglobulinuria* - **Cryoglobulinemia** is characterized by the presence of **cryoglobulins** in the blood, which can precipitate in cold temperatures. - This often leads to **cutaneous small vessel vasculitis**, manifesting as **palpable purpura**, particularly on the lower extremities [1]. *H. S. Purpura* - **Henoch-Schonlein Purpura (HSP)**, now known as **IgA vasculitis**, is a systemic vasculitis primarily affecting small vessels [1]. - Its hallmark dermatological feature is **palpable purpura**, typically found on the **buttocks and lower extremities**, often accompanied by arthralgia, abdominal pain, and renal involvement [1]. *Drug induced vasculitis* - **Drug-induced vasculitis** is an immune-mediated reaction to certain medications that can cause inflammation of blood vessels. - This condition frequently presents with **palpable purpura**, along with other symptoms like fever, arthralgia, and myalgia, due to the acute inflammation of small blood vessels in the skin.

Q: BASDAI criteria is indicated in

Ankylosing spondylitis. ***Ankylosing spondylitis*** - The **Bath Ankylosing Spondylitis Disease Activity Index (BASDAI)** is a commonly used patient-reported outcome measure specifically designed to assess disease activity and symptoms in patients with **ankylosing spondylitis** [1]. - It evaluates five key symptoms: **fatigue, spinal pain, peripheral joint pain/swelling, enthesitis, and morning stiffness**, along with the duration of morning stiffness [1]. *Any autoimmune disease* - While BASDAI is used in an autoimmune disease, it is **not applicable to all autoimmune diseases** due to their diverse manifestations and affected systems. - Each autoimmune disease often has its own **specific criteria or indices** to measure disease activity and monitor treatment response. *Kawasaki Disease* - **Kawasaki disease** is a childhood vasculitis primarily affecting medium-sized arteries, and its diagnostic and activity criteria are distinct from BASDAI. - Disease activity in Kawasaki disease is monitored by different clinical features and laboratory markers such as **fever, rash, conjunctivitis, and inflammatory markers**. *Rheumatoid Arthritis* - **Rheumatoid arthritis (RA)** is a chronic inflammatory autoimmune disease primarily affecting synovial joints, and its disease activity is assessed using scales like **DAS28 (Disease Activity Score 28)**. - Although RA and ankylosing spondylitis are both arthritic conditions, their clinical presentation, affected joints, and systemic involvement differ, necessitating different assessment tools.

Q: Which of the following statements regarding Rheumatoid arthritis is false?

Pulmonary nodules are present. Everything regarding Rheumatoid arthritis follows a predictable clinical pattern. [1] ***Pulmonary nodules are present*** - While **rheumatoid arthritis** can cause pulmonary manifestations, the presence of **pulmonary nodules** is a specific, though less common, finding. [1] This statement is framed as generally true, but in the context of a "false" question about RA characteristics, it's considered false because not all RA patients have them, and this is a distractor to a clear, established finding in RA like **juxta-articular osteoporosis**, leading to further options like "none of the above". It's often used to differentiate it from other conditions. - The phrasing "pulmonary nodules are present" suggests a universal presence, which is not accurate. **Rheumatoid nodules** can occur in the lungs but are not a defining or constant feature across all cases. [1] *Juxta articular osteoporosis may be present* - **Juxta-articular osteoporosis** is a common and early radiological feature of **rheumatoid arthritis** due to chronic inflammation affecting bone density near the joints. [1] - This statement is true; **bone erosions** and **osteoporosis** are characteristic findings in RA. [1] *None of the above* - This option is incorrect because the statement "Pulmonary nodules are present" is indeed false when interpreted as a universal characteristic of rheumatoid arthritis. - Therefore, at least one of the previous statements (the first one) is false, making this option inappropriate. *It is symmetrical involving small and large joints* - **Rheumatoid arthritis** typically affects joints in a **symmetrical pattern**, affecting both small joints (hands, feet) and larger joints (knees, shoulders) over time. [1] - This statement accurately describes a key clinical characteristic of **rheumatoid arthritis**, making it a true statement. [1]

Q: In a patient with gouty arthritis, strongly birefringent needle-shaped crystals with negative elongation in synovial fluid aspiration are composed of:

Monosodium urate. ***Monosodium urate*** - **Monosodium urate (MSU)** crystals are characteristic of **gouty arthritis** [1]. They appear as **needle-shaped** crystals with **strong negative birefringence** when viewed under a polarized light microscope, which helps in differentiating them from other crystals. - These crystals typically precipitate in joints due to **hyperuricemia**, causing an acute inflammatory response [2]. *Homogentisic acid* - **Homogentisic acid** crystals are associated with **alkaptonuria (ochronosis)**, a rare metabolic disorder, not gout. - These crystals impart a dark color to cartilage and urine upon oxidation, and their microscopic appearance is distinct from MSU crystals. *Calcium pyrophosphate* - **Calcium pyrophosphate dihydrate (CPPD)** crystals are associated with **pseudogout**, a condition similar to gout but caused by different crystals [3]. - CPPD crystals appear as **rhomboid or rod-shaped** and exhibit **weakly positive birefringence**, differentiating them from MSU crystals. *Sodium pyrophosphate* - **Sodium pyrophosphate** is not a commonly recognized crystal type found in synovial fluid that causes inflammatory arthritis. - While pyrophosphate is involved in bone metabolism, its sodium salt does not typically form characteristic crystals in joints like MSU or CPPD.

Q: A young man back from leisure trip has swollen knee joints & foreign body sensation in eyes. Likely cause is -

Reiter's syndrome. REITER'S SYNDROME [1] - Reiter's syndrome, also known as **reactive arthritis**, is characterized by the classic triad of **arthritis**, **conjunctivitis** (foreign body sensation in eyes), and **urethritis** [1]. - It often develops after a **genitourinary or gastrointestinal infection**, which aligns with a recent leisure trip [1]. *Behcet's disease* - Behcet's disease primarily presents with recurrent **oral and genital ulcers**, along with **uveitis** and **skin lesions**. - While arthritis can occur, the combination of specific eye foreign body sensation and a recent trip isn't as characteristic as for Reiter's. *SLE* - **Systemic lupus erythematosus (SLE)** is a systemic autoimmune disease with diverse manifestations, including polyarthritis and serositis. - **Conjunctivitis** as a primary eye symptom or a direct association with a preceding infection/trip is less typical compared to Reiter's syndrome. *Sarcoidosis* - **Sarcoidosis** is a multisystem inflammatory disease often involving the **lungs, skin, and lymph nodes**, and can cause arthritis. - However, its acute presentation with foreign body sensation in the eyes and post-trip history is not its common debut.

Q: A 24-year-old woman presents with abdominal pain, joint discomfort, and lower limb rash. She was well until 1 week before presentation. On examination, she has a palpable purpuric rash on her legs, nonspecific abdominal discomfort, and no active joints. She has 3+ proteinuria, normal WBC, no eosinophils, and elevated creatinine of 1.6 mg/dL. Biopsy of the rash confirms vasculitis with immunoglobulin A (IgA) 3+and C3 (complement 3) deposition on immunofluorescence.For the above patient with vasculitis syndrome, select the most likely diagnosis.

Henoch-Schonlein purpura. ***Henoch-Schonlein purpura*** - The classic triad of **palpable purpura**, **arthralgia**, and **abdominal pain**, along with **renal involvement (proteinuria, elevated creatinine)**, strongly suggests Henoch-Schonlein purpura (HSP) [1]. - **IgA deposition** on biopsy of the rash is the hallmark of HSP, confirming its diagnosis [1]. *vasculitis associated with infectious diseases* - While infections can trigger vasculitis, the specific presentation with **IgA deposition**, palpable purpura, and prominent gastrointestinal/renal involvement points away from this broad category [1]. - No clear infectious trigger or specific pathogen is mentioned, making a more specific diagnosis like HSP more appropriate given the findings. *Churg-Strauss disease* - Characterized by **asthma**, **eosinophilia**, and **granulomatous inflammation**, none of which are present in this patient (normal WBC, no eosinophils mentioned) [1]. - Typically involves **ANCA-positive vasculitis (MPO-ANCA)**, which is not indicated here, and IgA deposition is not a characteristic feature [1]. *polyarteritis nodosa (PAN)* - Primarily affects **medium-sized arteries** and can cause **aneurysms** and **organ ischemia**, but classically spares the capillaries and does not involve IgA deposition or palpable purpura. - Often associated with **hepatitis B infection** and lacks the prominent skin and renal manifestations of HSP with IgA deposition.

Q: Which one of the following does not cause small vessel vasculitis?

Kawasaki disease. ***Kawasaki disease*** - **Kawasaki disease** is a **medium vessel vasculitis** that primarily affects the coronary arteries in children, not small vessels [1]. - It is characterized by fever, rash, lymphadenopathy, and mucocutaneous involvement, with a risk of coronary artery aneurysms [1]. *Churg-Strauss syndrome* - **Churg-Strauss syndrome** (now known as **Eosinophilic Granulomatosis with Polyangiitis** or **EGPA**) is a type of **ANCA-associated vasculitis** that frequently involves small vessels [1]. - It is characterized by asthma, **eosinophilia**, and granulomatous inflammation, often affecting the lungs, skin, and kidneys [1]. *Henoch-Schönlein purpura* - **Henoch-Schönlein purpura** (**IgA vasculitis**) is a common form of **small vessel vasculitis** in children and young adults [1]. - It is characterized by a classic tetrad of palpable **purpura**, **arthralgia**, **abdominal pain**, and **renal disease** [1]. *Microscopic polyangiitis* - **Microscopic polyangiitis** (MPA) is a severe form of **ANCA-associated small vessel vasculitis**. - It typically causes necrotizing glomerulonephritis and pulmonary capillaritis without granuloma formation [2].

Question 1

Which category of patients with juvenile idiopathic arthritis, who are mostly HLA-B27 positive and present with enthesitis, lower limb arthritis involving the knees and ankles, and inflammatory low back pain, are most likely to be?

Accepted Answer

Enthesitis related arthritis

Answer

Oligoarticular JIA

Answer

Polyarticular JIA (RF negative)

Answer

Systemic JIA

Question 2

HLA associated with rheumatoid arthritis is?

Accepted Answer

HLA DR 4

Answer

HLA DR2

Answer

HLA B27

Answer

HLA-B9

Question 3

Joint not involved in RA ?

Accepted Answer

DIP

Answer

PIP

Answer

Wrist

Answer

MCP

Question 4

Palpable purpura is seen in all conditions except

Accepted Answer

Giant cell arteritis

Answer

Cryoglobulinuria

Answer

H. S. Purpura

Answer

Drug induced vasculitis

Question 5

BASDAI criteria is indicated in

Accepted Answer

Ankylosing spondylitis

Answer

Any autoimmune disease

Answer

Kawasaki Disease

Answer

Rheumatoid Arthritis

Question 6

Which of the following statements regarding Rheumatoid arthritis is false?

Accepted Answer

Pulmonary nodules are present

Answer

Juxta articular osteoporosis may be present

Answer

None of the above

Answer

It is symmetrical involving small and large joints

Question 7

In a patient with gouty arthritis, strongly birefringent needle-shaped crystals with negative elongation in synovial fluid aspiration are composed of:

Accepted Answer

Monosodium urate

Answer

Homogentisic acid

Answer

Calcium pyrophosphate

Answer

Sodium pyrophosphate

Question 8

A young man back from leisure trip has swollen knee joints & foreign body sensation in eyes. Likely cause is -

Accepted Answer

Reiter's syndrome

Answer

Behcet's disease

Answer

SLE

Answer

Sarcoidosis

Question 9

A 24-year-old woman presents with abdominal pain, joint discomfort, and lower limb rash. She was well until 1 week before presentation. On examination, she has a palpable purpuric rash on her legs, nonspecific abdominal discomfort, and no active joints. She has 3+ proteinuria, normal WBC, no eosinophils, and elevated creatinine of 1.6 mg/dL. Biopsy of the rash confirms vasculitis with immunoglobulin A (IgA) 3+and C3 (complement 3) deposition on immunofluorescence.For the above patient with vasculitis syndrome, select the most likely diagnosis.

Accepted Answer

Henoch-Schonlein purpura

Answer

vasculitis associated with infectious diseases

Answer

Churg-Strauss disease

Answer

polyarteritis nodosa (PAN)

Question 10

Which one of the following does not cause small vessel vasculitis?

Accepted Answer

Kawasaki disease

Answer

Churg - strauss syndrome

Answer

Henoch - Schonlein purpura

Answer

Microscopic polyangiitis

Rheumatology and Immunology — MCQs

Rheumatology and Immunology — MCQs

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