Rheumatology and Immunology Practice Questions

Q: Wegener's granulomatosis is diagnosed by:

c-ANCA. **Explanation:** **Wegener’s Granulomatosis** (now officially termed **Granulomatosis with Polyangiitis or GPA**) is a small-vessel vasculitis characterized by a triad of necrotizing granulomas of the respiratory tract, vasculitis, and glomerulonephritis. **Why c-ANCA is correct:** The hallmark laboratory finding in GPA is the presence of **c-ANCA** (cytoplasmic Antineutrophil Cytoplasmic Antibodies). These antibodies are directed against the enzyme **Proteinase-3 (PR3)**. c-ANCA is highly specific (>95%) and sensitive for GPA, especially when the disease is active and systemic. Monitoring c-ANCA titers can also help in assessing disease activity and predicting relapses. **Why other options are incorrect:** * **P-ANCA (Perinuclear-ANCA):** These antibodies are directed against **Myeloperoxidase (MPO)**. They are typically associated with **Microscopic Polyangiitis (MPA)** and **Churg-Strauss Syndrome** (Eosinophilic Granulomatosis with Polyangiitis) [2]. * **m-ANCA:** This is not a standard clinical term used in vasculitis diagnostics; it is likely a distractor for MPO-ANCA. * **A.N.A (Antinuclear Antibody):** This is the screening test for **Systemic Lupus Erythematosus (SLE)** and other connective tissue diseases, but it is not specific for Wegener’s [1]. **High-Yield Clinical Pearls for NEET-PG:** 1. **The Triad:** Upper respiratory tract (sinusitis/saddle nose deformity), Lower respiratory tract (hemoptysis/cavitation), and Kidneys (Crescentic GN). 2. **Biopsy Gold Standard:** While c-ANCA is the best initial diagnostic test, the definitive diagnosis is made via biopsy (showing necrotizing granulomas). 3. **Treatment:** Induction with Corticosteroids + Cyclophosphamide (or Rituximab). 4. **ELISA Correlation:** c-ANCA correlates with anti-PR3; p-ANCA correlates with anti-MPO [2].

Q: Which of the following is not a feature of granulomatosis with polyangiitis (Wegener's syndrome)?

Cardiomegaly. **Explanation:** **Granulomatosis with Polyangiitis (GPA)**, formerly known as Wegener’s granulomatosis, is a small-vessel vasculitis characterized by a triad of necrotising granulomas of the upper and lower respiratory tracts, systemic vasculitis, and glomerulonephritis [1]. **Why Cardiomegaly is the correct answer:** While GPA can occasionally involve the heart (pericarditis or coronary vasculitis) [2], **cardiomegaly** is not a characteristic or diagnostic feature of the syndrome. GPA primarily targets the respiratory system and kidneys. Cardiomegaly is more typically associated with conditions causing congestive heart failure or valvular diseases, rather than primary small-vessel vasculitis. **Analysis of other options:** * **Chronic Sinusitis:** This is the most common presenting feature of GPA (found in >90% of cases). Patients often present with nasal crusting, epistaxis, and saddle-nose deformity due to septal perforation [1]. * **Bronchiectasis:** GPA causes chronic inflammation and scarring of the airways. Necrotising granulomas and recurrent infections secondary to airway stenosis can lead to permanent dilation of the bronchi (bronchiectasis). * **Situs Inversus:** While not a primary feature of GPA itself, this option is often used in exams to differentiate GPA from **Kartagener’s Syndrome** (Primary Ciliary Dyskinesia), which presents with the triad of situs inversus, bronchiectasis, and sinusitis. In the context of this specific question, situs inversus is considered a "distractor" or a feature *not* associated with GPA. **High-Yield Clinical Pearls for NEET-PG:** * **Serology:** Highly specific for **c-ANCA** (anti-proteinase 3 antibodies). * **Classic Triad:** Upper respiratory tract + Lower respiratory tract + Kidneys (Pauci-immune glomerulonephritis) [1]. * **Radiology:** Lung nodules with **cavitation** are a hallmark finding [1]. * **Treatment:** Induction with Corticosteroids + Cyclophosphamide (or Rituximab).

Q: A 20-year-old girl presented with low-grade fever, malaise, night sweats, weight loss, arthralgia, and fatigue for 1 month. On examination, peripheral pulses were weak in the upper extremities and a bruit was present over the carotid and subclavian arteries. Investigations revealed dilatation of the proximal aorta. What is the most likely diagnosis?

Takayasu's arteritis. The clinical presentation is classic for **Takayasu’s Arteritis (TA)**, also known as "Pulseless Disease." It is a chronic, large-vessel vasculitis that primarily involves the **aorta and its major branches** [1]. 1. **Why it is correct:** The patient is a young female (typical demographic) presenting with systemic symptoms (fever, weight loss) followed by features of vascular inflammation. The **weak upper extremity pulses** and **bruit** over the carotids/subclavian arteries indicate arterial stenosis. The **dilatation of the proximal aorta** (aortitis) is a hallmark finding. According to ACR criteria, age <40, claudication, and decreased brachial artery pulses are key diagnostic features. 2. **Why the other options are wrong:** * **Polyarteritis nodosa (PAN):** A medium-vessel vasculitis that typically involves the renal and visceral arteries (sparing the lungs). It presents with hypertension, skin nodules, and mononeuritis multiplex, not absent pulses in the upper limbs. * **Temporal arteritis (Giant Cell Arteritis):** Also a large-vessel vasculitis, but it almost exclusively affects patients **over the age of 50**. It typically presents with headache, jaw claudication, and visual loss. * **Kawasaki's disease:** Primarily affects **children (<5 years)** [1]. It involves medium-sized vessels, most notably the **coronary arteries** (aneurysms), and presents with "strawberry tongue," conjunctivitis, and rash [1]. **High-Yield Clinical Pearls for NEET-PG:** * **Demographic:** Most common in Asian females 10 mmHg) between the two arms.

Q: All of the following conditions are associated with palpable purpura, EXCEPT?

Giant cell arteritis. **Explanation:** The hallmark of **palpable purpura** is cutaneous small-vessel vasculitis [1]. It occurs when inflammation of the post-capillary venules leads to vessel wall necrosis and the extravasation of red blood cells into the dermis, creating lesions that are both visible and elevated [1]. **1. Why Giant Cell Arteritis (GCA) is the correct answer:** GCA is a **large-vessel vasculitis** that primarily affects the aorta and its major branches (especially the extracranial branches of the carotid artery). Because it involves large muscular arteries rather than superficial small vessels or capillaries, it does **not** present with palpable purpura. Typical clinical features include temporal headache, jaw claudication, and visual loss. **2. Analysis of incorrect options:** * **Henoch-Schönlein Purpura (IgA Vasculitis):** This is a small-vessel vasculitis [1]. Palpable purpura (typically on the lower extremities and buttocks) is a mandatory diagnostic criterion. * **Wegener Granulomatosis (GPA):** This is an ANCA-associated small-vessel vasculitis. While it primarily affects the respiratory tract and kidneys, cutaneous involvement with palpable purpura occurs in approximately 15-45% of cases. * **Polyarteritis Nodosa (PAN):** PAN is a medium-vessel vasculitis; however, it frequently involves smaller vessels at the dermal-subcutaneous junction, leading to skin manifestations like palpable purpura, livedo reticularis, and skin ulcers. **High-Yield Clinical Pearls for NEET-PG:** * **Vessel Size Rule:** Palpable purpura = Small-vessel involvement [1]. * **Non-palpable purpura:** Usually suggests non-inflammatory causes (e.g., thrombocytopenia, senile purpura, or scurvy). * **GCA Association:** Strongly associated with **Polymyalgia Rheumatica (PMR)** in 50% of patients. * **Diagnostic Gold Standard:** Temporal artery biopsy for GCA; however, "skip lesions" can occur.

Q: Which condition is specifically associated with anti-ds DNA antibodies?

Systemic Lupus Erythematosus (SLE). **Explanation:** **Systemic Lupus Erythematosus (SLE)** is the correct answer because anti-double-stranded DNA (anti-dsDNA) antibodies are highly specific (approx. 95–97%) for this condition. While Antinuclear Antibody (ANA) is the best screening test due to its high sensitivity, anti-dsDNA is a **confirmatory marker** [1]. Clinically, anti-dsDNA levels correlate with **disease activity**, particularly the development and severity of **Lupus Nephritis**. **Analysis of Incorrect Options:** * **Systemic Sclerosis (Diffuse):** Characteristically associated with **anti-Scl-70 (anti-topoisomerase I)** antibodies. * **CREST Syndrome (Limited Scleroderma):** Strongly associated with **anti-centromere** antibodies. * **Sjogren’s Syndrome:** Characterized by **anti-Ro (SS-A)** and **anti-La (SS-B)** antibodies [1]. **High-Yield Clinical Pearls for NEET-PG:** * **Most Specific Antibody for SLE:** Anti-Smith (anti-Sm) antibody (does not fluctuate with disease activity) [1]. * **Most Sensitive Antibody for SLE:** ANA (best initial screening test) [1]. * **Drug-Induced Lupus:** Associated with **anti-histone** antibodies; anti-dsDNA is typically absent. * **Neonatal Lupus/Congenital Heart Block:** Associated with maternal **anti-Ro/SSA** antibodies. * **Monitoring:** Anti-dsDNA and **complement levels (C3, C4)** are the two primary laboratory markers used to monitor SLE flares and renal involvement [1].

Q: A 34-year-old woman presents with a red rash over her cheeks, frequent oral ulcers, and pain and swelling in both wrists and small joints of her hands. Laboratory investigations reveal a positive ANA and 3+ proteinuria. Which of the following organ involvement will cause the most symptoms during the course of this disease?

Musculoskeletal pathology. ### Explanation The clinical presentation (malar rash, oral ulcers, inflammatory arthritis, and proteinuria) in a young female is diagnostic of **Systemic Lupus Erythematosus (SLE)**. [1] **1. Why Musculoskeletal pathology is correct:** While renal involvement is the most significant predictor of mortality and morbidity, **musculoskeletal involvement** is the **most common clinical manifestation** of SLE, affecting over 90% of patients. [2] It is typically the earliest symptom and causes the most frequent symptoms (pain, stiffness, and swelling) throughout the disease course. The arthritis in SLE is characteristically inflammatory, symmetrical, and non-erosive (unlike Rheumatoid Arthritis). [4] **2. Why other options are incorrect:** * **Renal pathology:** Although 3+ proteinuria indicates Lupus Nephritis (a major cause of morbidity), it is often asymptomatic in early stages. Only about 50% of patients develop clinically significant renal disease. * **Cardiopulmonary pathology:** While pleuritis and pericarditis are common, they occur less frequently than joint involvement. [3] * **Thrombotic events:** These are associated with secondary Antiphospholipid Syndrome (APS). While life-threatening, they occur in a minority of SLE patients (approx. 30% have APL antibodies, but fewer have clinical events). **Clinical Pearls for NEET-PG:** * **Most common symptom/initial presentation:** Arthritis/Arthralgia (>90%). * **Most common cardiac manifestation:** Pericarditis. * **Most common pulmonary manifestation:** Pleuritis. * **Jaccoud’s Arthropathy:** A reversible, non-erosive deformity seen in SLE due to ligamentous laxity. * **Best Screening Test:** ANA (High sensitivity). [3] * **Most Specific Test:** Anti-dsDNA and Anti-Smith (Anti-dsDNA levels also correlate with disease activity/nephritis). [3]

Q: Anti-nuclear antibodies are not found in which of the following conditions?

Sarcoidosis. The presence of **Anti-nuclear antibodies (ANA)** is a hallmark of systemic autoimmune rheumatic diseases (SARDs). ANA serves as a screening tool for conditions where the immune system loses tolerance to self-antigens within the cell nucleus [4]. **Why Sarcoidosis is the Correct Answer:** Sarcoidosis is a multisystem inflammatory disease characterized by the formation of **non-caseating granulomas**. Its pathogenesis is primarily driven by a **Type IV (cell-mediated) hypersensitivity** reaction involving T-lymphocytes and macrophages [2], rather than a B-cell mediated autoantibody response. Therefore, ANA is typically negative in Sarcoidosis. **Analysis of Incorrect Options:** * **Systemic Lupus Erythematosus (SLE):** ANA is the best initial screening test for SLE, with a sensitivity of **>95-99%** [1]. A negative ANA virtually rules out SLE (high negative predictive value). * **Drug-induced Lupus (DILE):** ANA is positive in nearly 100% of cases. The classic marker is **Anti-histone antibodies** (found in >90% of DILE cases). * **Diffuse Scleroderma (Systemic Sclerosis):** ANA is positive in approximately 95% of patients [1]. Specific markers include **Anti-Scl-70 (anti-topoisomerase I)** for diffuse disease and **Anti-centromere** for limited disease (CREST syndrome). **High-Yield Clinical Pearls for NEET-PG:** 1. **ANA Pattern:** A "Homogeneous" pattern is common in SLE, while a "Nucleolar" pattern is highly suggestive of Systemic Sclerosis. 2. **Most Specific Test for SLE:** Anti-dsDNA and Anti-Smith (Anti-Sm) antibodies [1]. 3. **Sarcoidosis Markers:** Elevated **Serum ACE levels**, hypercalciuria/hypercalcemia, and bilateral hilar lymphadenopathy on CXR (Stage I) [3]. 4. **ANA-Negative SLE:** Extremely rare; historically associated with Anti-Ro (SS-A) antibodies [1].

Q: C-ANCA is associated with which of the following conditions?

Wegener's granulomatosis. Explanation: **C-ANCA (Cytoplasmic Antineutrophil Cytoplasmic Antibody)** is highly specific for **Wegener’s Granulomatosis** (now known as Granulomatosis with Polyangiitis or GPA). The target antigen for C-ANCA is **Proteinase-3 (PR3)**. In GPA, the classic clinical triad involves the upper respiratory tract (sinusitis, saddle nose deformity), lower respiratory tract (hemoptysis, cavitary nodules), and kidneys (pauci-immune glomerulonephritis). **Analysis of Options:** * **Microscopic Polyangiitis (MPA):** This is primarily associated with **P-ANCA** (Perinuclear-ANCA), which targets the enzyme **Myeloperoxidase (MPO)**. Unlike GPA, MPA lacks granulomatous inflammation. * **Churg-Strauss Syndrome (EGPA):** This condition is also associated with **P-ANCA** (MPO-ANCA) in about 40-50% of cases [1]. It is characterized by asthma, peripheral eosinophilia, and extravascular granulomas [1]. * **Polyarteritis Nodosa (PAN):** PAN is a medium-vessel vasculitis that is characteristically **ANCA-negative**. It is strongly associated with Hepatitis B infection and involves microaneurysms (seen on angiography). **High-Yield Clinical Pearls for NEET-PG:** 1. **C-ANCA = PR3-ANCA** (Wegener’s/GPA). 2. **P-ANCA = MPO-ANCA** (Microscopic Polyangiitis, Churg-Strauss, Primary Sclerosing Cholangitis, and Ulcerative Colitis) [1]. 3. **Wegener’s Triad:** Sinus + Lung + Kidney. 4. **Drug-induced Lupus** is associated with anti-histone antibodies, while **Drug-induced ANCA vasculitis** (e.g., by Hydralazine or Propylthiouracil) is usually P-ANCA positive. 5. **Negative ANCA** in a systemic vasculitis should immediately make you think of **PAN** or **Kawasaki disease**.

Question 1

Wegener's granulomatosis is diagnosed by:

Accepted Answer

c-ANCA

Answer

P-ANCA

Answer

m-ANCA

Answer

A.N.A

Question 2

In giant cell arteritis, which of the following statements is false?

Accepted Answer

Giant cells are typically seen on biopsy but are not strictly required for diagnosis.

Answer

Histological diagnosis is based on fragmentation of the internal elastic lamina.

Answer

C-reactive protein is usually elevated.

Answer

Anemia of chronic disease is a common feature.

Question 3

Which of the following is not a feature of granulomatosis with polyangiitis (Wegener's syndrome)?

Accepted Answer

Cardiomegaly

Answer

Situs inversus

Answer

Chronic sinusitis

Answer

Bronchiectasis

Question 4

A 20-year-old girl presented with low-grade fever, malaise, night sweats, weight loss, arthralgia, and fatigue for 1 month. On examination, peripheral pulses were weak in the upper extremities and a bruit was present over the carotid and subclavian arteries. Investigations revealed dilatation of the proximal aorta. What is the most likely diagnosis?

Accepted Answer

Takayasu's arteritis

Answer

Polyarteritis nodosa

Answer

Temporal arteritis

Answer

Kawasaki's disease

Question 5

All of the following conditions are associated with palpable purpura, EXCEPT?

Accepted Answer

Giant cell arteritis

Answer

Henoch-Schönlein purpura

Answer

Wegener granulomatosis

Answer

Polyarteritis nodosa

Question 6

A 45-year-old female, known case of Irritable Bowel Syndrome, presented with poorly localized pain, especially involving the axial skeleton. The pain was severe, associated with tenderness, and had been occurring for the past 4 months. She also reported fatigue, stiffness, sleep disturbance, cognitive dysfunction, anxiety, and depression. Lab and radiographic studies were normal. Which of the following drugs is NOT approved for the management of this patient's condition?

Accepted Answer

Tetrabenazine

Answer

Cyclobenzaprine

Answer

Duloxetine

Answer

Pregabalin

Question 7

Which condition is specifically associated with anti-ds DNA antibodies?

Accepted Answer

Systemic Lupus Erythematosus (SLE)

Answer

Systemic sclerosis

Answer

CREST syndrome

Answer

Sjogren's syndrome

Question 8

A 34-year-old woman presents with a red rash over her cheeks, frequent oral ulcers, and pain and swelling in both wrists and small joints of her hands. Laboratory investigations reveal a positive ANA and 3+ proteinuria. Which of the following organ involvement will cause the most symptoms during the course of this disease?

Accepted Answer

Musculoskeletal pathology

Answer

Renal pathology

Answer

Cardiopulmonary pathology

Answer

Thrombotic events

Question 9

Anti-nuclear antibodies are not found in which of the following conditions?

Accepted Answer

Sarcoidosis

Answer

Systemic Lupus Erythematosus (SLE)

Answer

Diffuse Scleroderma

Answer

Drug-induced lupus

Question 10

C-ANCA is associated with which of the following conditions?

Accepted Answer

Wegener's granulomatosis

Answer

Microscopic polyangitis

Answer

Churg Strauss syndrome

Answer

Polyarteritis nodosa (PAN)

Rheumatology and Immunology — MCQs

Rheumatology and Immunology — MCQs

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