Rheumatology and Immunology Practice Questions

Q: Which condition is characterized by the triad of skin lesions, mononeuritis multiplex, and elevated eosinophils?

Churg - Strauss syndrome. ***Churg - Strauss syndrome*** - **Churg-Strauss syndrome** (now known as **Eosinophilic Granulomatosis with Polyangiitis, EGPA**) is characterized by the triad of **skin lesions**, **mononeuritis multiplex** (nerve damage), and **elevated eosinophils** [1]. - It is a **small-vessel vasculitis** with a strong association with **asthma** and **allergic rhinitis** [1]. *Alports syndrome* - **Alport syndrome** is an inherited disorder affecting **type IV collagen**, primarily manifesting as **glomerulonephritis**, **sensorineural hearing loss**, and **ocular abnormalities**. - It does not present with skin lesions, mononeuritis multiplex, or eosinophilia. *Wegeners granulomatosus* - **Wegener's granulomatosis** (now **Granulomatosis with Polyangiitis, GPA**) is characterized by **granulomatous inflammation** of the upper and lower respiratory tracts, **glomerulonephritis**, and **systemic vasculitis** [2]. - While it can cause skin lesions and neuropathy, it is typically associated with **antineutrophil cytoplasmic antibodies (ANCAs)** and does not typically involve eosinophilia as a defining feature [1]. *Cryoglobulinemia* - **Cryoglobulinemia** is a condition where abnormal immunoglobulins (cryoglobulins) precipitate in cold temperatures, leading to **vasculitis-like symptoms** in small and medium-sized vessels. - It often manifests as **palpable purpura**, **arthralgia**, and **glomerulonephritis**, but mononeuritis multiplex and elevated eosinophils are not primary or defining features.

Q: Anti Jo-1 antibodies are a feature associated with?

Polymyositis. Polymyositis - **Anti-Jo-1 antibodies** are a specific type of **anti-synthetase antibody** and are a key serological marker for polymyositis [1]. - Their presence often indicates a more severe form of the disease with a higher likelihood of **interstitial lung disease**, **Raynaud's phenomenon**, and **"mechanic's hands"** [1]. *Systemic sclerosis (scleroderma)* - While systemic sclerosis is also an **autoimmune connective tissue disease**, it is typically associated with antibodies such as **anti-Scl-70** (topoisomerase I) or **anti-centromere antibodies**. - **Anti-Jo-1 antibodies** are not characteristic of systemic sclerosis; their presence would suggest a different or overlapping diagnosis. *Rheumatoid arthritis (RA)* - **Rheumatoid arthritis** is primarily diagnosed by the presence of **rheumatoid factor (RF)** and **anti-cyclic citrullinated peptide (anti-CCP) antibodies**. - **Anti-Jo-1 antibodies** are not found in the typical serological profile of rheumatoid arthritis. *Systemic lupus erythematosus (SLE)* - **Systemic lupus erythematosus** is characterized by a wide array of autoantibodies, most notably **anti-nuclear antibodies (ANA)**, **anti-dsDNA antibodies**, and **anti-Sm antibodies** [2]. - **Anti-Jo-1 antibodies** are rarely, if ever, found in SLE and would point away from this diagnosis.

Q: In patients with the limited cutaneous form of CREST syndrome, which autoantibody is most commonly found?

Anti-centromere antibodies. ***Anti-centromere antibodies*** - **Anti-centromere antibodies (ACA)** are highly specific for the **limited cutaneous systemic sclerosis (lcSSc)**, often referred to as CREST syndrome. - They are associated with a milder course of the disease and a lower risk of serious internal organ involvement, especially **pulmonary fibrosis**. *Anti-DNA topoisomerase I* - **Anti-DNA topoisomerase I (Scl-70 antibodies)** are classically associated with the **diffuse cutaneous systemic sclerosis (dcSSc)**. - Patients with Scl-70 antibodies are at higher risk for **interstitial lung disease** and more generalized skin involvement. *Anti-double-stranded DNA antibodies* - **Anti-double-stranded DNA (anti-dsDNA) antibodies** are a hallmark of **systemic lupus erythematosus (SLE)**, not systemic sclerosis [1]. - Their presence often correlates with **lupus nephritis** and disease activity in SLE [1]. *Anti-Golgi antibodies* - **Anti-Golgi antibodies** are less commonly tested and are not specifically associated with systemic sclerosis or CREST syndrome. - They are sometimes found in various **autoimmune liver diseases** or in patients with certain neurological conditions.

Q: Takayasu arteritis mainly affects?

Subclavian artery. ***Subclavian artery*** - **Takayasu arteritis** is a chronic inflammatory disorder that primarily affects the **aorta** and its **major branches**, including the subclavian arteries [1]. - Inflammation of the subclavian arteries can lead to **stenosis** or **occlusion**, resulting in symptoms such as **arm claudication**, **pulse deficit**, and blood pressure discrepancies between arms. *Pulmonary artery* - While Takayasu arteritis can, in rare cases, affect the pulmonary arteries, it is not their primary or most common target. - **Pulmonary artery involvement** is less frequent and generally seen in more widespread or severe disease. *Celiac artery* - The celiac artery, a branch of the aorta supplying the foregut, can be affected by **Takayasu arteritis**, but it is less commonly involved than the great vessels emanating from the aortic arch. - Involvement here can lead to symptoms like **abdominal pain** or **mesenteric ischemia**. *Aorta* - Takayasu arteritis primarily targets the **aorta** and its **proximal branches**, but "subclavian artery" is a more specific and common site of severe clinical manifestation often associated with the disease [1]. - The aorta itself is frequently affected, leading to **aneurysms**, **stenosis**, or **dilatation** throughout its course.

Q: Which of the following is NOT a major criterion in the Modified Jones Criteria for diagnosing Rheumatic Fever?

Polyarthalgia. ***Polyarthralgia*** - **Polyarthralgia** is a **minor criterion** in the Modified Jones Criteria, indicating joint pain without objective signs of inflammation. - For a criterion to be considered major, it must involve objective signs of arthritis, which differentiate it from mere pain. [1] *Carditis* - **Carditis** is a **major criterion** and refers to inflammation of the heart, which can manifest as pericarditis, myocarditis, or endocarditis, often leading to valvular damage. [1] - It is one of the most serious manifestations of rheumatic fever and can lead to long-term cardiac complications. *Chorea* - **Sydenham chorea** (also known as St. Vitus' dance) is a **major criterion** characterized by involuntary, purposeless movements, primarily affecting the face, hands, and feet. [1] - It is a neurological manifestation reflecting central nervous system involvement and can appear late in the course of the disease. [1] *Erythema marginatum* - **Erythema marginatum** is a **major criterion** presenting as a distinctive, transient, non-pruritic rash with pink or red rings and clear centers, typically appearing on the trunk and proximal extremities. - This rash is a specific skin manifestation of acute rheumatic fever, though it is relatively uncommon.

Q: Arthritis involving DIP, PIP, 1st carpometacarpal with sparing of MCP and wrist joints is typical of ?

Osteoarthritis. ***Osteoarthritis*** - **Osteoarthritis** characteristically affects the **DIP (Heberden's nodes)**, **PIP (Bouchard's nodes)**, and the **first carpometacarpal joint** at the base of the thumb [1]. - It typically spares the **MCP and wrist joints**, which helps differentiate it from inflammatory arthropathies like rheumatoid arthritis [1], [2]. *Rheumatoid arthritis* - **Rheumatoid arthritis** characteristically affects the **MCP and wrist joints**, sparing the DIP joints [2]. - It presents with **symmetrical polyarthritis** and often includes systemic symptoms [2]. *Ankylosing spondylitis* - **Ankylosing spondylitis** primarily affects the **axial skeleton**, particularly the sacroiliac joints and spine. - While peripheral joint involvement can occur, it's typically in larger joints and not the characteristic DIP/PIP pattern of this case. *Psoriatic arthritis* - **Psoriatic arthritis** can affect the **DIP joints** and can present with patterns similar to osteoarthritis or rheumatoid arthritis [3]. - However, it's strongly associated with **psoriasis** (skin and nail changes) and can involve various other patterns, including **dactylitis** (sausage digits) and enthesitis, which makes it less typical for the described sparing pattern alone [3].

Q: What is the most common variant of Takayasu Disease in India?

Type V. ***Type V*** - **Type V Takayasu arteritis** involves both the **aortic arch and its branches** (similar to Type I or II) AND the **abdominal aorta** and/or **renal arteries** (similar to Type III or IV) [1]. - This extensive involvement is the **most common presentation** of Takayasu arteritis in India, characterized by widespread vasculopathy affecting multiple arterial segments. *Type I* - **Type I Takayasu arteritis** primarily affects the **aortic arch** and its main branches, such as the subclavian, carotid, and vertebral arteries [1]. - While present in India, it is **less common** than Type V, which also includes abdominal aortic involvement. *Type III* - **Type III Takayasu arteritis** involves the **thoracic descending aorta, abdominal aorta**, and/or **renal arteries**, in addition to the **aortic arch branches**. - Although it includes abdominal involvement, it does not encompass the full range of involvement seen in the most prevalent Indian variant, Type V. *Type IV* - **Type IV Takayasu arteritis** is characterized by involvement of the **abdominal aorta** and/or **renal arteries** [1]. - This type is less common as a sole presentation in India; typically, renal artery involvement is part of a more generalized arterial inflammation, as seen in Type V.

Q: Which joint is most commonly affected in pseudogout?

Knee. ***Knee*** - The **knee joint** is the most frequent site of acute attacks and chronic arthropathy in pseudogout due to the propensity for **calcium pyrophosphate dihydrate (CPPD)** crystal deposition in larger joints [1]. - Patients often present with acute, painful, and swollen knees, sometimes mimicking septic arthritis or gout [1]. *Hip* - While hip involvement can occur in pseudogout, it is **less common** than knee involvement and often manifests as chronic degenerative changes. - Acute hip flares are rarer compared to the knee. *MP joint great toe* - The **metatarsaophalangeal (MTP) joint of the great toe** is the classic site for **gout**, caused by monosodium urate crystal deposition. - Although pseudogout can affect various joints, this specific location is strongly indicative of gout, not pseudogout. *MP joint thumb* - The **metacarpophalangeal (MCP) joint of the thumb** can be affected in various arthropathies, but it is not the most commonly affected joint in pseudogout. - Involvement here is less specific for pseudogout compared to the knee.

Q: Tophi in gout are typically found in all regions except which of the following?

Muscle. ***Muscle*** - **Tophi** are crystalline deposits of **monosodium urate** that form in chronic gout [1]. They are typically found in cooler, less vascularized tissues, or tissues subjected to repetitive trauma. - While tophi can occur in many soft tissues, their presence **within muscle tissue is rare**, as muscle is well-vascularized and not a primary site for urate deposition [2]. *Prepatellar bursae* - The **prepatellar bursa** is a common site for tophi due to its superficial location and exposure to trauma. - Inflammation of the prepatellar bursa due to tophi accumulation is known as **"housemaid's knee"**. *Helix of ear* - The **helix of the ear** is a classic location for tophi due to its cooler temperature and poor vascularity, making it an ideal site for urate crystal deposition [1]. - These are often painless, subcutaneous nodules. *Synovial membrane* - The **synovial membrane** lining joints is a very common site for tophi formation, leading to destructive arthritis in chronic gout [2]. - Urate crystals in the synovial fluid provoke acute inflammatory attacks and can accumulate in the membrane over time.

Q: The most likely diagnosis in the case of a patient with multiple pulmonary cavities, hematuria, and red cell casts is?

Wegener's granulomatosis. ***Wegener's granulomatosis (Granulomatosis with Polyangiitis)*** - This condition classically presents with a **triad of upper airway, lower airway, and renal involvement** [1]. **Pulmonary cavities** represent severe lung involvement, and **hematuria with red cell casts** indicate rapidly progressive glomerulonephritis [3]. - It is an **ANCA-associated vasculitis**, specifically associated with **c-ANCA (anti-PR3 antibodies)**, and often leads to **granulomatous inflammation** [1], [3]. *Anti-GBM disease* - Characterized by **glomerulonephritis** and **pulmonary hemorrhage** (Goodpasture's syndrome) [1], but does not typically cause multiple **pulmonary cavities**. - Diagnosed by the presence of **anti-glomerular basement membrane antibodies** and linear IgG deposition on renal biopsy [1]. *Churg-Strauss* - This condition, now known as **Eosinophilic Granulomatosis with Polyangiitis (EGPA)**, is characterized by **asthma, eosinophilia, and vasculitis** [1]. - While it can affect the lungs and kidneys, **pulmonary cavities** are less common, and severe eosinophilia is a hallmark, which is not mentioned here [1]. *Systemic lupus erythematosus* - A multisystem autoimmune disease that can cause **glomerulonephritis (lupus nephritis)** and pleuropulmonary involvement [2]. - However, **multiple pulmonary cavities** are not a typical manifestation, and other classic lupus features (e.g., malar rash, arthralgias) are absent in the description [2].

Question 1

Which condition is characterized by the triad of skin lesions, mononeuritis multiplex, and elevated eosinophils?

Accepted Answer

Churg - Strauss syndrome

Answer

Alports syndrome

Answer

Wegeners granulomatosus

Answer

Cryoglobulinemia

Question 2

Anti Jo-1 antibodies are a feature associated with?

Accepted Answer

Polymyositis

Answer

Systemic sclerosis (scleroderma)

Answer

Rheumatoid arthritis (RA)

Answer

Systemic lupus erythematosus (SLE)

Question 3

In patients with the limited cutaneous form of CREST syndrome, which autoantibody is most commonly found?

Accepted Answer

Anti-centromere antibodies

Answer

Anti-DNA topoisomerase I

Answer

Anti-double-stranded DNA antibodies

Answer

Anti-Golgi antibodies

Question 4

Takayasu arteritis mainly affects?

Accepted Answer

Subclavian artery

Answer

Pulmonary artery

Answer

Celiac artery

Answer

Aorta

Question 5

Which of the following is NOT a major criterion in the Modified Jones Criteria for diagnosing Rheumatic Fever?

Accepted Answer

Polyarthalgia

Answer

Erythema marginatum

Answer

Carditis

Answer

Chorea

Question 6

Arthritis involving DIP, PIP, 1st carpometacarpal with sparing of MCP and wrist joints is typical of ?

Accepted Answer

Osteoarthritis

Answer

Rheumatoid arthritis

Answer

Ankylosing spondylitis

Answer

Psoriatic arthritis

Question 7

What is the most common variant of Takayasu Disease in India?

Accepted Answer

Type V

Answer

Type III

Answer

Type IV

Answer

Type I

Question 8

Which joint is most commonly affected in pseudogout?

Accepted Answer

Knee

Answer

Hip

Answer

MP joint great toe

Answer

MP joint thumb

Question 9

Tophi in gout are typically found in all regions except which of the following?

Accepted Answer

Muscle

Answer

Helix of ear

Answer

Prepatellar bursae

Answer

Synovial membrane

Question 10

The most likely diagnosis in the case of a patient with multiple pulmonary cavities, hematuria, and red cell casts is?

Accepted Answer

Wegener's granulomatosis

Answer

Anti-GBM disease

Answer

Churg-Strauss

Answer

Systemic lupus erythematousus

Rheumatology and Immunology — MCQs

Rheumatology and Immunology — MCQs

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