Rheumatology and Immunology Practice Questions

Q: In which of the following vasculitides does lung involvement not occur?

Polyarteritis nodosa. Explanation: The key to answering this question lies in understanding the classification of vasculitides based on the size of the vessels involved and their association with Antineutrophil Cytoplasmic Antibodies (ANCA). 1. Why Polyarteritis Nodosa (PAN) is the correct answer: PAN is a medium-vessel vasculitis. A defining clinical characteristic of classic PAN is that it spares the lungs (pulmonary arteries are not involved). It primarily affects the renal and visceral arteries, often leading to hypertension, renal failure (without glomerulonephritis), and abdominal angina. If a patient presents with systemic vasculitis and lung involvement, PAN should be ruled out. 2. Why the other options are incorrect: Options A, C, and D are all ANCA-associated small-vessel vasculitides, which characteristically involve the respiratory tract: * Granulomatosis with polyangiitis (GPA): Classically involves the "triad" of upper respiratory tract (sinusitis, saddle nose), lower respiratory tract (cavitary nodules, hemoptysis), and kidneys. * Eosinophilic granulomatosis with polyangiitis (EGPA/Churg-Strauss): Defined by asthma, peripheral eosinophilia, and pulmonary infiltrates. * Microscopic polyangiitis (MPA): Frequently causes pulmonary capillaritis leading to diffuse alveolar hemorrhage. Unlike GPA, it lacks granulomatous inflammation. Clinical Pearls for NEET-PG: * PAN & Hepatitis B: There is a strong association between PAN and Hepatitis B surface antigenemia (HBsAg). * Microaneurysms: PAN is characterized by "string of beads" appearance on angiography due to microaneurysms. * ANCA Status: PAN is ANCA-negative, whereas GPA (c-ANCA/PR3), MPA (p-ANCA/MPO), and EGPA (p-ANCA/MPO) are typically ANCA-positive. * Renal Involvement: PAN causes renal artery vasculitis (infarcts), while small-vessel vasculitides cause Glomerulonephritis.

Q: Anti-nuclear antibodies (ANA) seen in Systemic Lupus Erythematosus (SLE) are directed against which of the following?

dsDNA. ### Explanation **Correct Option: A (dsDNA)** Systemic Lupus Erythematosus (SLE) is the prototype autoimmune disease characterized by the production of antibodies against self-antigens, primarily those located within the cell nucleus. **Anti-dsDNA (double-stranded DNA)** antibodies are highly specific for SLE (95-100% specificity) and are a hallmark of the disease [1]. They are directed against the deoxyribonucleic acid backbone. Clinically, their titers correlate with disease activity, particularly the development of **Lupus Nephritis** [2]. **Analysis of Incorrect Options:** * **B. mRNA:** While SLE involves antibodies against various ribonucleoproteins (like Anti-Smith or Anti-U1 RNP), they are directed against proteins complexed with RNA, not typically naked messenger RNA (mRNA) itself. * **C. Nucleolus:** Anti-nucleolar antibodies (producing a nucleolar pattern on immunofluorescence) are more characteristic of **Systemic Sclerosis (Scleroderma)**, specifically associated with antigens like PM-Scl or RNA polymerase I [2]. * **D. Ribosomes:** Anti-ribosomal P antibodies are found in SLE but are less common than anti-dsDNA. They are highly specific for **Neuropsychiatric SLE (Lupus Psychosis)** rather than being the primary target of general ANA screening. **High-Yield Clinical Pearls for NEET-PG:** * **Best Screening Test for SLE:** ANA (High sensitivity, >95%) [1]. * **Most Specific Test for SLE:** Anti-Smith (Anti-Sm) and Anti-dsDNA [2]. * **Drug-Induced Lupus:** Anti-Histone antibodies are the marker of choice. * **Neonatal Lupus/Sjogren’s:** Associated with Anti-Ro (SS-A) and Anti-La (SS-B) [2]. * **Mixed Connective Tissue Disease (MCTD):** Characterized by high titers of Anti-U1 RNP [2].

Q: Which of the following is a characteristic finding in Churg-Strauss syndrome?

Allergic rhinitis. **Explanation:** **Churg-Strauss Syndrome**, now formally known as **Eosinophilic Granulomatosis with Polyangiitis (EGPA)**, is a small-to-medium vessel necrotizing vasculitis characterized by three distinct phases: the prodromal (allergic) phase, the eosinophilic phase, and the vasculitic phase [1]. **Why Allergic Rhinitis is Correct:** The hallmark of the prodromal phase is **allergic rhinitis** and **adult-onset asthma**. Nearly all patients (up to 95%) suffer from severe allergic rhinitis, nasal polyposis, and asthma, which often precede the systemic vasculitis by several years. The presence of eosinophilic infiltration in the nasal mucosa makes allergic rhinitis a classic diagnostic feature of EGPA. **Analysis of Incorrect Options:** * **A. Sinusitis:** While sinus involvement occurs in EGPA, it is much more characteristic and destructive in **Granulomatosis with Polyangiitis (GPA/Wegener’s)**. * **B. Raynaud's phenomenon:** This is typically associated with connective tissue diseases like Systemic Sclerosis (Scleroderma) or SLE, rather than primary vasculitides like EGPA. * **D. Epistaxis:** This is a common presenting symptom of **GPA (Wegener’s)** due to friable granulomatous crusting in the nasal passages; it is not a defining feature of EGPA. **Clinical Pearls for NEET-PG:** * **Triad of EGPA:** Asthma + Peripheral Eosinophilia (>1500 cells/µL) + Vasculitis [1]. * **Antibody Marker:** p-ANCA (anti-MPO) is positive in approximately 40-50% of cases (usually those with renal involvement) [1]. * **Organ Involvement:** Most common cause of death is **cardiac involvement** (myocarditis/coronary vasculitis). * **Neurology:** Mononeuritis multiplex (e.g., foot drop) is a frequent vasculitic manifestation [1]. * **Distinction:** Unlike GPA, EGPA is rarely associated with cavitary lung lesions.

Q: Urate crystals are deposited in small joints of the hands and feet in which condition?

Gout. **Explanation:** **Correct Option: A. Gout** Gout is a crystal arthropathy caused by **monosodium urate (MSU)** crystal deposition in joints and soft tissues [1]. This occurs due to chronic hyperuricemia (serum uric acid >6.8 mg/dL) [2]. These crystals are needle-shaped and exhibit **strong negative birefringence** under polarized light [3]. Gout characteristically involves small peripheral joints, most commonly the **first metatarsophalangeal (MTP) joint** (Podagra), but frequently affects the small joints of the hands and feet [4]. The deposition is favored in cooler peripheral areas where urate solubility is lower. **Incorrect Options:** * **B. Still’s Disease:** This is a systemic autoinflammatory disorder (Systemic Onset JIA in children) characterized by high-grade spiking fever, evanescent salmon-pink rash, and arthritis. It does not involve crystal deposition. * **C. Retropharyngeal Abscess:** This is a deep neck space infection. While it can cause "Grisel’s syndrome" (atlantoaxial subluxation), it is an infectious/surgical emergency unrelated to urate metabolism. * **D. Ankylosing Spondylitis:** A seronegative spondyloarthropathy primarily affecting the axial skeleton (sacroiliac joints and spine). It is associated with the **HLA-B27** gene and involves enthesitis and syndesmophyte formation, not urate crystals. **High-Yield Clinical Pearls for NEET-PG:** 1. **Gold Standard Diagnosis:** Identification of needle-shaped, negatively birefringent MSU crystals from joint aspirate [3]. 2. **Radiology:** Look for **"punched-out" erosions** with overhanging edges (Martel’s sign) in chronic tophaceous gout. 3. **Acute Management:** NSAIDs (first-line), Colchicine, or Corticosteroids. 4. **Chronic Management:** Xanthine oxidase inhibitors like **Allopurinol** or Febuxostat [2] (started only after the acute attack has subsided).

Q: Among patients with polymyositis, which one of the following auto-antibodies is associated with an increased risk of interstitial lung disease?

Anti-Jo-1 antibody. Explanation: **Correct Option: D. Anti-Jo-1 antibody** Anti-Jo-1 is the most common **Myositis-Specific Antibody (MSA)**, directed against histidyl-tRNA synthetase. It is the hallmark of **Antisynthetase Syndrome**, a clinical triad characterized by: 1. **Myositis** (Polymyositis or Dermatomyositis) [2] 2. **Interstitial Lung Disease (ILD)**: Present in up to 30% of patients generally and is strongly associated with the presence of antisynthetase (Jo-1) antibodies [2]. 3. **Other features**: Raynaud’s phenomenon, "Mechanic’s hands" (hyperkeratotic skin on fingers), non-erosive arthritis, and fever. The presence of Anti-Jo-1 is a strong predictor of pulmonary involvement, which significantly impacts the prognosis of patients with polymyositis [2]. **Incorrect Options:** * **A. Anti-CCP antibody:** Highly specific for **Rheumatoid Arthritis (RA)**. While RA can cause ILD, this antibody is not a marker for polymyositis. * **B. Anti-dsDNA antibody:** Highly specific for **Systemic Lupus Erythematosus (SLE)** and is associated with lupus nephritis. * **C. Anti-cardiolipin antibody:** Part of the **Antiphospholipid Syndrome (APS)**, associated with arterial/venous thrombosis and recurrent pregnancy loss [3]. **High-Yield Clinical Pearls for NEET-PG:** * **Anti-Mi-2:** Associated with classic Dermatomyositis (good prognosis, "V-sign" and "Shawl sign"). * **Anti-SRP (Signal Recognition Particle):** Associated with severe, necrotizing myopathy and poor response to therapy. * **Anti-MDA5:** Associated with clinically amyopathic dermatomyositis and **rapidly progressive ILD**. * **Initial Screening:** ANA is positive in about 30–80% of patients with dermatomyositis or polymyositis [1], but MSAs (like Jo-1) are required for specific subtyping.

Q: All of the following are true regarding Temporal arteritis except?

Unilateral headache. Temporal Arteritis, also known as **Giant Cell Arteritis (GCA)**, is a large-vessel vasculitis that primarily affects the branches of the external carotid artery. **Why Option B is the Correct Answer (The "Except"):** While headache is the most common symptom of GCA (seen in ~75% of cases), it is typically **bilateral** or generalized. While it can occasionally present unilaterally, the classic description for board exams emphasizes that the pain is often diffuse. More importantly, the other options represent "major" diagnostic criteria or hallmark complications, making "unilateral headache" the least accurate definitive statement among the choices. **Analysis of Other Options:** * **Option A (Age > 50 years):** This is a core diagnostic criterion. GCA almost never occurs in individuals under 50; the incidence peaks between ages 70 and 80. * **Option C (High ESR):** An elevated Erythrocyte Sedimentation Rate (often >50 mm/h, frequently >100 mm/h) is a hallmark laboratory finding. While C-reactive protein (CRP) is more sensitive, a high ESR is a classic diagnostic marker [1]. * **Option D (Ischemic Optic Neuropathy):** This is the most feared complication. Anterior Ischemic Optic Neuropathy (AION) due to occlusion of the posterior ciliary arteries can lead to sudden, irreversible blindness [1]. **NEET-PG High-Yield Pearls:** * **Strong Association:** ~50% of patients have **Polymyalgia Rheumatica (PMR)** (proximal muscle stiffness/pain) [1]. * **Gold Standard Diagnosis:** Temporal artery biopsy (look for "skip lesions"—hence a long segment must be biopsied) [1]. * **Classic Sign:** **Jaw claudication** (highest specificity for GCA). * **Treatment:** Start high-dose corticosteroids **immediately** if suspected to prevent permanent vision loss; do not wait for biopsy results [1].

Q: CREST syndrome is an autoimmune condition associated with atrophy and fibrosis of the oesophageal musculature, resulting in dysphagia and reflux-type symptoms. Which of the following is NOT a feature of CREST syndrome?

Erythematous malar rash. CREST syndrome is a **limited cutaneous form of Systemic Sclerosis (SSc)**. The diagnosis is clinical and defined by its eponymous acronym. [1] **1. Why "Erythematous malar rash" is the correct answer:** An erythematous malar (butterfly) rash is the hallmark clinical feature of **Systemic Lupus Erythematosus (SLE)**, not Systemic Sclerosis. While both are connective tissue diseases, they have distinct cutaneous manifestations. In CREST, skin involvement is typically limited to the face and distal extremities (distal to elbows/knees). **2. Analysis of incorrect options (Features of CREST):** * **C - Calcinosis cutis:** Soft tissue calcifications, often seen as painful nodules on fingertips or pressure points (Option D). * **R - Raynaud’s phenomenon:** Episodic vasospasm of digits in response to cold or stress; usually the earliest manifestation (Option A). * **E - Esophageal dysmotility:** Due to atrophy and fibrosis of the lower two-thirds of the esophageal smooth muscle. * **S - Sclerodactyly:** Thickening and tightening of the skin of the fingers or toes, giving them a "sausage-like" appearance (Option C). [1] * **T - Telangiectasia:** Dilated capillaries on the face, mucous membranes, and palms. **Clinical Pearls for NEET-PG:** * **Serology:** CREST syndrome is highly associated with **Anti-centromere antibodies** (highly specific). In contrast, Diffuse SSc is associated with **Anti-Scl-70 (topoisomerase I)** antibodies. * **Prognosis:** Limited SSc (CREST) generally has a better prognosis than diffuse SSc but carries a significant risk of **Pulmonary Arterial Hypertension (PAH)** later in the disease course. * **Organ Involvement:** Unlike diffuse SSc, CREST rarely involves the kidneys (Scleroderma Renal Crisis) or causes early interstitial lung disease.

Q: A 25-year-old woman complains of low-grade fever, fatigue, and a persistent rash over her nose and upper chest. She also notes pain in her knees and elbows. A skin biopsy shows dermal inflammation and granular deposits of IgG and C3 complement along the basement membrane at the epidermal/dermal junction. Urinalysis reveals microscopic hematuria and proteinuria. The ANA is positive. What is commonly associated with the development of thromboembolic complications in this patient?

Phospholipids. ### Explanation **Correct Answer: D. Phospholipids** **Reasoning:** The clinical presentation—malar rash, photosensitivity, polyarthritis, hematuria/proteinuria (lupus nephritis), and a positive ANA—is classic for **Systemic Lupus Erythematosus (SLE)** [1]. The biopsy finding of granular IgG and C3 at the dermo-epidermal junction is known as the **Lupus Band Test**. In SLE patients, thromboembolic complications (both arterial and venous) are most commonly associated with **Antiphospholipid Syndrome (APS)**. This occurs due to the presence of **antiphospholipid antibodies** (e.g., Lupus Anticoagulant, Anti-cardiolipin, and Anti-̢-glycoprotein I). These antibodies target **phospholipid-binding proteins**, leading to a prothrombotic state, recurrent miscarriages, and thrombocytopenia. **Incorrect Options:** * **A. ABO blood group antigens:** While certain blood groups (like Type A) have a slightly higher risk of general VTE, they are not the specific driver of thrombosis in the context of SLE. * **B. Class II HLA molecules:** HLA-DR2 and DR3 are strongly associated with the *genetic predisposition* to SLE, but they do not directly mediate the coagulation cascade or thromboembolic events. * **C. Clotting factors:** While deficiencies in Protein C or S cause thrombosis, the specific association with SLE-related hypercoagulability is the presence of autoantibodies against phospholipids, not a primary deficiency of clotting factors. **High-Yield Clinical Pearls for NEET-PG:** * **Lupus Band Test:** Positive in both involved and uninvolved skin in SLE, but only in involved skin in Discoid Lupus (DLE). * **APS Paradox:** Patients with Lupus Anticoagulant have a **prolonged aPTT** *in vitro* (due to interference with phospholipids in the assay) but a **prothrombotic state** *in vivo*. * **Libman-Sacks Endocarditis:** Non-bacterial verrucous vegetations on both sides of heart valves, also associated with APS in SLE patients.

Q: Metacarpophalangeal joints are most commonly affected in which condition?

Rheumatoid arthritis. **Explanation:** The **Metacarpophalangeal (MCP) joints** are the hallmark site of involvement in **Rheumatoid Arthritis (RA)**. RA is a chronic, systemic inflammatory disease characterized by symmetric polyarthritis [1]. The underlying pathophysiology involves synovial hypertrophy (pannus formation) that specifically targets the small joints of the hands and feet, particularly the MCP and Proximal Interphalangeal (PIP) joints, while characteristically **sparing the Distal Interphalangeal (DIP) joints** [1]. **Analysis of Options:** * **Osteoarthritis (OA):** Primarily affects weight-bearing joints and the hands at the **DIP joints** (Heberden’s nodes) and **PIP joints** (Bouchard’s nodes). The MCP joints are typically spared in primary OA. * **Psoriatic Arthritis (PsA):** Classically involves the **DIP joints** and can present with "dactylitis" (sausage digits). While it can affect MCPs, it is usually asymmetric and associated with skin/nail changes. * **Rheumatic Fever:** Typically presents with a **migratory large-joint polyarthritis** (knees, ankles, elbows). It rarely involves the small joints of the hands. **High-Yield Clinical Pearls for NEET-PG:** * **RA Hand Deformities:** Ulnar deviation at MCP joints, Swan-neck deformity, and Boutonniere deformity. * **Radiological Signs of RA:** Periarticular osteopenia, uniform joint space narrowing, and marginal erosions. * **Sparing Rule:** If the **DIP joint** is involved, think OA or Psoriatic Arthritis; if the **MCP joint** is involved, think RA or CPPD (Pseudogout) [1]. * **Morning Stiffness:** In RA, stiffness lasts >1 hour and improves with activity; in OA, it lasts <30 minutes and worsens with activity [1].

Q: Which of the following antibodies is most frequently seen in Antiphospholipid Syndrome?

Anti- beta 2 glycoprotein antibody. **Explanation:** Antiphospholipid Syndrome (APS) is an autoimmune prothrombotic state characterized by arterial or venous thrombosis and pregnancy complications. The diagnosis relies on the presence of at least one clinical criterion and one laboratory criterion. **Why Option D is Correct:** The laboratory criteria for APS include three specific antibodies: **Lupus Anticoagulant (LA)**, **Anti-cardiolipin (aCL) antibodies**, and **Anti-beta 2 glycoprotein I (̢2-GPI) antibodies**. Among these, Anti-̢2 glycoprotein I is considered the most specific and is frequently seen in patients with APS. It targets the ̢2-GPI protein, which normally binds to anionic phospholipids, playing a crucial role in the pathogenic clotting cascade of the syndrome. **Why Other Options are Incorrect:** * **Option A:** **Beta 2 microglobulin** is a component of MHC Class I molecules. It is used as a tumor marker (e.g., Multiple Myeloma) or a marker of renal function, but it is not an antibody associated with APS. * **Option B:** **Anti-nuclear antibody (ANA)** is the screening test for Systemic Lupus Erythematosus (SLE) [1]. While APS can occur secondary to SLE, ANA is not specific to APS itself [1], [2]. * **Option C:** **Anti-centromere antibody** is highly specific for **Limited Cutaneous Systemic Sclerosis (CREST syndrome)**, not APS. **High-Yield Clinical Pearls for NEET-PG:** * **The "Lupus Anticoagulant" Paradox:** Despite its name, LA is *prothrombotic* in vivo but causes a *prolonged aPTT* in vitro (which does not correct with a mixing study). * **False Positive VDRL:** Patients with APS often show a false positive syphilis test (VDRL/RPR) because the reagin antibodies cross-react with the cardiolipin used in the test. * **Catastrophic APS (Asherson’s Syndrome):** A rare, life-threatening form involving small-vessel thrombosis in at least three organ systems simultaneously.

Question 1

In which of the following vasculitides does lung involvement not occur?

Accepted Answer

Polyarteritis nodosa

Answer

Eosinophilic granulomatosis with vasculitis

Answer

Microscopic polyangiitis

Answer

Granulomatosis with polyangiitis

Question 2

Anti-nuclear antibodies (ANA) seen in Systemic Lupus Erythematosus (SLE) are directed against which of the following?

Accepted Answer

dsDNA

Answer

mRNA

Answer

Nucleolus

Answer

Ribosomes

Question 3

Which of the following is a characteristic finding in Churg-Strauss syndrome?

Accepted Answer

Allergic rhinitis

Answer

Sinusitis

Answer

Raynaud's phenomenon

Answer

Epistaxis

Question 4

Urate crystals are deposited in small joints of the hands and feet in which condition?

Accepted Answer

Gout

Answer

Still's disease

Answer

Retropharyngeal abscess

Answer

Ankylosing spondylitis

Question 5

Among patients with polymyositis, which one of the following auto-antibodies is associated with an increased risk of interstitial lung disease?

Accepted Answer

Anti-Jo-1 antibody

Answer

Anti-CCP antibody

Answer

Anti ds DNA antibody

Answer

Anti cardiolipin antibody

Question 6

All of the following are true regarding Temporal arteritis except?

Accepted Answer

Unilateral headache

Answer

Age > 50 years

Answer

Diagnosed by measuring high ESR

Answer

Complicated by ischemic optic neuropathy

Question 7

CREST syndrome is an autoimmune condition associated with atrophy and fibrosis of the oesophageal musculature, resulting in dysphagia and reflux-type symptoms. Which of the following is NOT a feature of CREST syndrome?

Accepted Answer

Erythematous malar rash

Answer

Raynaud's phenomenon

Answer

Sclerodactyly

Answer

Soft tissue calcification

Question 8

A 25-year-old woman complains of low-grade fever, fatigue, and a persistent rash over her nose and upper chest. She also notes pain in her knees and elbows. A skin biopsy shows dermal inflammation and granular deposits of IgG and C3 complement along the basement membrane at the epidermal/dermal junction. Urinalysis reveals microscopic hematuria and proteinuria. The ANA is positive. What is commonly associated with the development of thromboembolic complications in this patient?

Accepted Answer

Phospholipids

Answer

ABO blood group antigens

Answer

Class II HLA molecules

Answer

Clotting factors

Question 9

Metacarpophalangeal joints are most commonly affected in which condition?

Accepted Answer

Rheumatoid arthritis

Answer

Osteoarthritis

Answer

Psoriatic arthritis

Answer

Rheumatic fever

Question 10

Which of the following antibodies is most frequently seen in Antiphospholipid Syndrome?

Accepted Answer

Anti- beta 2 glycoprotein antibody

Answer

Beta 2 microglobulin antibody

Answer

Anti-nuclear antibody

Answer

Anti-centromere antibody

Rheumatology and Immunology — MCQs

Rheumatology and Immunology — MCQs

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