Rheumatology and Immunology — MCQs

A 33-year-old woman presents with a 2-month history of fever and increasing fatigue. Over the past year, she has experienced muscle and joint soreness and a 4-kg weight loss. On physical examination, her temperature is 37.5°C, pulse is 80/min, respirations are 15/min, and blood pressure is 145/95 mm Hg. She has pleuritic chest pain, and a friction rub is heard on auscultation. Laboratory findings include glucose, 73 mg/dL; total protein, 5.2 g/dL; albumin, 2.9 g/dL; and creatinine, 2.4 mg/dL. Serum complement levels are decreased. CBC shows hemoglobin of 9.7 g/dL, platelet count of 85,000/mm³, and WBC count of 3560/mm³. A renal biopsy specimen shows diffuse proliferative glomerulonephritis with extensive granular immune deposits of IgG and C1q in capillary loops and mesangium. After treatment with immunosuppressive therapy, her condition improves. Which of the following serologic studies is most likely to be positive in this patient?

Q45Easy

Which of the following organs is typically involved in Granulomatosis with Polyangiitis (Wegener's)?

Q46Medium

Which of the following statements about Henoch-Schonlein Purpura is false?

Q47Medium

A young male develops serous otitis media of the left ear with cough, occasional hemoptysis, hematuria, and epistaxis for 2 months. His Hb is 7.5 g% with heavy proteinuria, BP 170/100 mmHg, RA negative, and ANCA positive. What is the most probable diagnosis?

Q48Easy

All of the following are true for Sjogren syndrome except?

Q49Easy

Which disease characteristically involves the distal interphalangeal joint?

Q50Medium

A patient complains of hematuria. Renal biopsy demonstrates a focal necrotizing glomerulonephritis with crescent formation. The patient also complains of decreased sensation in the hands and feet. What is the next best investigation to diagnose the condition?

Rheumatology and Immunology Indian Medical PG Practice Questions and MCQs

Question 41: Which of the following statements is false about erythema marginatum?

A. Begins as a pink papule
B. Clears from the center and leaves a rash behind (Correct Answer)
C. Is the classical rash of acute rheumatic fever
D. Occurs only in patients with carditis

Explanation: **Explanation:** **Erythema Marginatum** is a hallmark cutaneous manifestation of **Acute Rheumatic Fever (ARF)** and serves as one of the Major Criteria in the **Jones Criteria** [1]. 1. **Why Option B is the correct (False) statement:** Erythema marginatum is characterized by its **evanescent (fleeting)** nature. It begins as pinkish-red macules or papules that spread peripherally. As the lesion expands, it **clears centrally**, leaving a sharp, wavy, or "serpiginous" erythematous border. Crucially, it **does not leave a permanent rash or scar behind**; the skin returns to its normal appearance once the lesion fades. 2. **Analysis of Incorrect Options:** * **Option A:** True. The rash typically starts as small, non-pruritic pink papules that extend outward. * **Option C:** True. It is the pathognomonic skin manifestation of ARF, though it occurs in less than 5% of patients. * **Option D:** True. In the context of ARF, erythema marginatum is almost exclusively seen in patients who also have **carditis** [1]. It is rarely, if ever, seen as an isolated finding. **Clinical Pearls for NEET-PG:** * **Morphology:** Non-pruritic, non-tender, blanching, and serpiginous (snake-like) borders. * **Location:** Primarily affects the **trunk and proximal extremities**; it characteristically **spares the face**. * **Triggers:** The rash is transient and can be accentuated by the application of heat (e.g., a hot shower). * **Association:** Highly specific for ARF and strongly associated with a higher risk of underlying rheumatic heart disease [1].

Question 42: All of the following statements regarding systemic sclerosis are TRUE, EXCEPT:

A. Increased serum antibodies to human cytomegalovirus are seen
B. Eosinophilia myalgia syndrome is a subtype of systemic sclerosis (Correct Answer)
C. The incidence of SSc is increased among miners exposed to silica
D. Bleomycin, pentazocine and cocaine may produce systemic sclerosis like illness

Explanation: ### Explanation **Systemic Sclerosis (SSc)** is a complex autoimmune disease characterized by microvascular injury and progressive fibrosis of the skin and internal organs [1]. **Why Option B is the Correct Answer (The False Statement):** Eosinophilia-myalgia syndrome (EMS) is **not** a subtype of systemic sclerosis. Instead, it is a distinct **scleroderma-like disorder** caused by the ingestion of contaminated **L-tryptophan**. While it presents with skin thickening and eosinophilia, it lacks the characteristic Raynaud’s phenomenon and internal organ involvement (like interstitial lung disease) seen in true SSc. **Analysis of Other Options:** * **Option A:** Viral triggers are implicated in SSc pathogenesis. Studies have shown an association between **Human Cytomegalovirus (hCMV)** and SSc, where molecular mimicry between hCMV proteins and endothelial cell antigens may trigger vascular damage. * **Option B:** Environmental exposure to **silica dust** (common in miners and stonecutters) is a well-documented risk factor that increases the incidence of SSc, likely through chronic immune activation. * **Option D:** Several drugs can induce scleroderma-like skin changes. **Bleomycin** (chemotherapy), **Pentazocine** (analgesic), and **Cocaine** are classic examples mentioned in standard textbooks (Harrison’s) as causes of chemically induced fibrosis. **Clinical Pearls for NEET-PG:** * **Limited SSc (CREST):** Anti-Centromere antibodies; involves skin distal to elbows/knees. * **Diffuse SSc:** Anti-Scl-70 (Topoisomerase I) and Anti-RNA Polymerase III antibodies; involves trunk and proximal limbs; higher risk of **Scleroderma Renal Crisis**. * **Early Sign:** Raynaud’s phenomenon is the most common initial presentation [1]. * **Drug of Choice for Renal Crisis:** ACE Inhibitors (even if Creatinine is elevated).

Question 43: Which of the following is used as screening for systemic lupus erythematosus?

A. Anti-nuclear antibody (Correct Answer)
B. Anti-Smith antibody
C. Anti-RNP antibody
D. Anti-phospholipid antibody

Explanation: **Explanation:** The diagnosis of Systemic Lupus Erythematosus (SLE) relies on a combination of clinical features and serological markers. **1. Why Anti-nuclear antibody (ANA) is the correct answer:** ANA is the **best screening test** for SLE because of its exceptionally high **sensitivity (approximately 95–99%)** [1]. In medical screening, a test with high sensitivity is preferred to "rule out" a disease; a negative ANA result makes a diagnosis of SLE highly unlikely [3]. According to the 2019 EULAR/ACR classification criteria, a positive ANA titer (≥1:80 on HEp-2 cells) is now a mandatory **entry criterion** for the diagnosis of SLE. **2. Why the other options are incorrect:** * **Anti-Smith (anti-Sm) antibody:** While it is the **most specific** marker for SLE, its sensitivity is low (only 10–30%) [1]. It is used for confirmation, not screening. * **Anti-RNP antibody:** This is associated with Mixed Connective Tissue Disease (MCTD). While it can be present in SLE, it is not specific or sensitive enough for screening [1]. * **Anti-phospholipid antibody:** These are associated with Antiphospholipid Syndrome (APS) and increased risk of thrombosis/pregnancy loss [2]. They are part of the SLE criteria but are not primary screening tools. **High-Yield Clinical Pearls for NEET-PG:** * **Most Sensitive Test:** ANA (Screening test of choice) [1]. * **Most Specific Test:** Anti-Smith (Anti-Sm) [1]. * **Best Marker for Disease Activity:** Anti-dsDNA (specifically correlates with lupus nephritis and low complement levels C3/C4) [3]. * **Drug-Induced Lupus:** Anti-Histone antibodies (most sensitive). * **Neonatal Lupus/Congenital Heart Block:** Anti-Ro (SS-A) and Anti-La (SS-B) [1].

Question 44: A 33-year-old woman presents with a 2-month history of fever and increasing fatigue. Over the past year, she has experienced muscle and joint soreness and a 4-kg weight loss. On physical examination, her temperature is 37.5°C, pulse is 80/min, respirations are 15/min, and blood pressure is 145/95 mm Hg. She has pleuritic chest pain, and a friction rub is heard on auscultation. Laboratory findings include glucose, 73 mg/dL; total protein, 5.2 g/dL; albumin, 2.9 g/dL; and creatinine, 2.4 mg/dL. Serum complement levels are decreased. CBC shows hemoglobin of 9.7 g/dL, platelet count of 85,000/mm³, and WBC count of 3560/mm³. A renal biopsy specimen shows diffuse proliferative glomerulonephritis with extensive granular immune deposits of IgG and C1q in capillary loops and mesangium. After treatment with immunosuppressive therapy, her condition improves. Which of the following serologic studies is most likely to be positive in this patient?

A. Anti-centromere antibody
B. Anti-DNA topoisomerase I antibody
C. Anti-double-stranded DNA antibody (Correct Answer)
D. Anti-glomerular basement membrane antibody

Explanation: ### Explanation The clinical presentation is classic for **Systemic Lupus Erythematosus (SLE)**. The patient meets multiple ACR/EULAR criteria: constitutional symptoms (fever, weight loss), serositis (pleuritic chest pain, friction rub), hematologic abnormalities (anemia, thrombocytopenia, leukopenia), and renal involvement [1]. **1. Why Option C is Correct:** The renal biopsy reveals **Diffuse Proliferative Glomerulonephritis (DPGN)**, which is Class IV Lupus Nephritis—the most common and severe form of renal involvement in SLE. The presence of "full house" immune deposits (IgG and C1q) is highly characteristic. **Anti-double-stranded DNA (anti-dsDNA) antibodies** are highly specific for SLE and correlate strongly with the presence and activity of lupus nephritis [1], [2]. Low complement levels (C3, C4) further support active SLE, as they are consumed during immune complex deposition [1]. **2. Why Incorrect Options are Wrong:** * **A. Anti-centromere antibody:** Associated with **Limited Cutaneous Systemic Sclerosis (CREST syndrome)**; not linked to glomerulonephritis or pancytopenia [2]. * **B. Anti-DNA topoisomerase I (Anti-Scl-70):** Associated with **Diffuse Cutaneous Systemic Sclerosis**, characterized by pulmonary fibrosis and extensive skin thickening [2]. * **D. Anti-GBM antibody:** Seen in **Goodpasture Syndrome**, which presents with rapidly progressive glomerulonephritis and pulmonary hemorrhage (hemoptysis), but lacks the multisystem involvement (pancytopenia, serositis) seen here. **Clinical Pearls for NEET-PG:** * **Most Specific Antibodies for SLE:** Anti-dsDNA and Anti-Smith (Anti-Sm) [2]. * **Most Sensitive Screening Test:** ANA (Anti-nuclear antibody) [1]. * **Drug-Induced Lupus:** Associated with Anti-Histone antibodies. * **Lupus Nephritis Marker:** Anti-dsDNA titers fluctuate with disease activity, especially renal flares [1]. * **Complement Levels:** Low C3 and C4 indicate active classical pathway activation in SLE [1].

Question 45: Which of the following organs is typically involved in Granulomatosis with Polyangiitis (Wegener's)?

A. Lungs (Correct Answer)
B. Nose
C. Kidney
D. Treatment with cytotoxic agents and steroids

Explanation: **Explanation:** **Granulomatosis with Polyangiitis (GPA)**, formerly known as Wegener's Granulomatosis, is a systemic necrotizing vasculitis that primarily affects small to medium-sized vessels [2]. It is classically characterized by a "triad" of involvement: the upper respiratory tract, the lower respiratory tract (lungs), and the kidneys. **Why Lungs (Option A) is the correct answer:** The lungs are involved in approximately 90% of patients. The hallmark pathological finding is necrotizing granulomatous inflammation [1]. Clinically, this manifests as cough, hemoptysis, and pleuritic chest pain. Radiologically, GPA typically presents with **bilateral, multiple, nodular cavitary lesions** on a chest X-ray or CT scan [1]. **Analysis of Incorrect Options:** * **Option B (Nose):** While the nose (upper respiratory tract) is frequently involved (saddle-nose deformity, chronic sinusitis), the question asks for the "typical organ" involvement in the context of systemic pathology [1]. In many standardized formats, if the lungs are listed, they represent the most common site of major visceral morbidity. However, in this specific question structure, Option D is a treatment modality, making the selection of a primary organ like the lungs the most appropriate clinical focus. * **Option C (Kidney):** Renal involvement (Pauci-immune Crescentic Glomerulonephritis) occurs in about 75-80% of cases [2]. While critical, it often occurs later in the disease course compared to respiratory symptoms. * **Option D (Treatment):** This is a management strategy, not an organ. GPA is treated with induction therapy using Corticosteroids plus either Cyclophosphamide or Rituximab. **NEET-PG High-Yield Pearls:** * **Serology:** Highly specific for **c-ANCA** (anti-PR3 antibodies). * **Classic Triad:** Upper Respiratory Tract + Lungs + Kidneys. * **Key Sign:** Saddle-nose deformity due to septal perforation [1]. * **Biopsy:** Lung biopsy is more definitive than renal biopsy because it shows the characteristic granulomas (renal biopsy only shows vasculitis/GN) [1].

Question 46: Which of the following statements about Henoch-Schonlein Purpura is false?

A. It is a Type 3 Hypersensitivity reaction.
B. Palpable purpura is due to thrombocytopenia. (Correct Answer)
C. Mild glomerulonephritis is a common finding.
D. Polyarthralgia is a frequent symptom.

Explanation: **Explanation:** Henoch-Schönlein Purpura (HSP), now commonly referred to as **IgA Vasculitis**, is a small-vessel vasculitis characterized by the deposition of IgA-dominant immune complexes [1]. **Why Option B is False (The Correct Answer):** In HSP, the palpable purpura is **not** due to a low platelet count (thrombocytopenia). Instead, it is a **non-thrombocytopenic purpura** caused by leukocytoclastic vasculitis. The inflammation and damage to the small blood vessel walls lead to the extravasation of red blood cells into the surrounding tissue. On examination, the platelet count in HSP patients is typically normal or even elevated (as an acute-phase reactant). **Analysis of Other Options:** * **Option A:** HSP is a **Type 3 Hypersensitivity reaction**. It involves the deposition of antigen-antibody (IgA) complexes in vessel walls, which activates the complement system (alternative pathway) and leads to tissue damage [1]. * **Option C:** **Glomerulonephritis** is a common finding (occurring in ~40-50% of cases). It usually presents as microscopic hematuria or mild proteinuria. Histologically, it is identical to IgA Nephropathy (Berger’s disease). * **Option D:** **Polyarthralgia** or arthritis is a frequent symptom (seen in ~75% of cases), typically affecting the large joints of the lower extremities like the knees and ankles. **High-Yield Clinical Pearls for NEET-PG:** * **Classic Tetrad:** Palpable purpura (buttocks/legs), Arthralgia, Abdominal pain (colicky), and Renal disease. * **Diagnosis:** Primarily clinical; however, skin biopsy showing **IgA deposition** via immunofluorescence is definitive. * **Complication:** Intussusception (usually ileo-ileal) is a serious gastrointestinal complication in children. * **Treatment:** Mostly supportive; steroids are used for severe abdominal pain or significant renal involvement.

Question 47: A young male develops serous otitis media of the left ear with cough, occasional hemoptysis, hematuria, and epistaxis for 2 months. His Hb is 7.5 g% with heavy proteinuria, BP 170/100 mmHg, RA negative, and ANCA positive. What is the most probable diagnosis?

A. Wegener's granulomatosis (Correct Answer)
B. Rapidly proliferative glomerulonephritis
C. Rheumatoid arthritis
D. Goodpasture syndrome

Explanation: This question describes a classic multisystem presentation of **Granulomatosis with Polyangiitis (GPA)**, formerly known as **Wegener’s Granulomatosis**. [1] ### **Why Option A is Correct** GPA is a small-vessel vasculitis characterized by a clinical triad of involvement in the **Upper Respiratory Tract (URT), Lower Respiratory Tract (LRT), and Kidneys**. * **URT:** Epistaxis and serous otitis media (due to Eustachian tube dysfunction) are hallmark features. * **LRT:** Cough and hemoptysis indicate pulmonary nodules or alveolar hemorrhage. * **Kidneys:** Hematuria, proteinuria, and hypertension signify **Pauci-immune Glomerulonephritis**. * **Serology:** The presence of **ANCA** (specifically c-ANCA/anti-PR3) is highly specific for GPA. [1] ### **Why Other Options are Incorrect** * **B. Rapidly Progressive Glomerulonephritis (RPGN):** This is a clinical syndrome (pathological finding), not a specific disease. While GPA causes RPGN, it does not explain the URT symptoms (otitis, epistaxis). * **C. Rheumatoid Arthritis:** Though RA can cause lung issues, it typically presents with symmetrical small joint polyarthritis and would be **RA factor positive**. * **D. Goodpasture Syndrome:** This involves a **pulmonary-renal syndrome** (hemoptysis + hematuria) but **never** involves the upper respiratory tract (ears/nose). It is associated with anti-GBM antibodies, not ANCA. ### **High-Yield Clinical Pearls for NEET-PG** * **Classic Triad:** Upper Respiratory + Lower Respiratory + Renal involvement. * **Specific Marker:** **c-ANCA** (Cytoplasmic) directed against **Proteinase-3 (PR3)**. [1] * **Biopsy Findings:** Granulomatous inflammation and necrotizing vasculitis. * **Treatment of Choice:** Cyclophosphamide + Corticosteroids (Rituximab is an alternative). [1] * **Key Radiology:** Cavitary nodules on Chest X-ray.

Question 48: All of the following are true for Sjogren syndrome except?

A. May be associated with other autoimmune diseases
B. Excessive salivation (Correct Answer)
C. Immunologically mediated disease
D. Commonly associated with rheumatoid arthritis

Explanation: **Explanation:** Sjögren syndrome (SS) is a chronic, systemic autoimmune disorder characterized by lymphocytic infiltration of the exocrine glands, primarily the lacrimal and salivary glands. **Why "Excessive salivation" is the correct answer:** The hallmark of Sjögren syndrome is **Xerostomia** (dry mouth) and **Keratoconjunctivitis sicca** (dry eyes). The autoimmune destruction of the salivary glands leads to a significant *decrease* in saliva production, not an increase. Patients often complain of a "cotton-mouth" sensation, difficulty swallowing dry food, and increased dental caries. **Analysis of other options:** * **Option A & D:** Sjögren syndrome is classified as **Primary** (occurring alone) or **Secondary** (associated with another autoimmune disease). **Rheumatoid Arthritis (RA)** is the most common associated condition, followed by SLE and Systemic Sclerosis. * **Option C:** It is an **immunologically mediated disease** involving B-cell hyperactivity and T-cell infiltration. The presence of autoantibodies like **Anti-Ro (SS-A)** and **Anti-La (SS-B)** confirms its immunological basis. **High-Yield Clinical Pearls for NEET-PG:** * **Schirmer’s Test:** Used to quantify tear production (Positive if <5 mm in 5 minutes). * **Diagnostic Gold Standard:** Lip biopsy showing focal lymphocytic sialadenitis. * **Malignancy Risk:** Patients have a 40-fold increased risk of developing **B-cell Non-Hodgkin Lymphoma** (MALToma). * **Extraglandular features:** May include Raynaud’s phenomenon, cutaneous vasculitis, and interstitial lung disease.

Question 49: Which disease characteristically involves the distal interphalangeal joint?

A. Psoriatic arthritis (Correct Answer)
B. Rheumatoid arthritis
C. Systemic lupus erythematosus
D. Gout

Explanation: The involvement of the **Distal Interphalangeal (DIP) joints** is a classic hallmark of **Psoriatic Arthritis (PsA)**, occurring in approximately 40-50% of patients [1]. This is often associated with characteristic psoriatic nail changes (pitting, onycholysis) [1]. In PsA, the inflammation occurs at the **enthesis** (where tendons/ligaments insert into bone), which leads to the characteristic "pencil-in-cup" deformity on X-ray and dactylitis ("sausage digit"). **Analysis of Incorrect Options:** * **B. Rheumatoid Arthritis (RA):** RA characteristically **spares the DIP joints** [2]. It primarily involves the Proximal Interphalangeal (PIP) joints, Metacarpophalangeal (MCP) joints, and wrists. * **C. Systemic Lupus Erythematosus (SLE):** SLE causes a non-erosive arthritis (Jaccoud’s arthropathy) that typically affects the PIPs and MCPs, similar to RA, but without bone destruction. * **D. Gout:** While gout can affect any joint, it most commonly presents as podagra (1st MTP joint). DIP involvement is rare unless the patient has underlying osteoarthritis (nodal gout). **NEET-PG High-Yield Pearls:** 1. **DIP Joint Differential:** The two most common causes of DIP involvement are **Osteoarthritis** (Heberden’s nodes) and **Psoriatic Arthritis** [1]. 2. **Radiology of PsA:** Look for the "Pencil-in-cup" deformity and "Telescoping" of digits (arthritis mutilans) [1]. 3. **CASPAR Criteria:** Used for the diagnosis of PsA; it includes skin psoriasis, nail lesions, dactylitis, and negative Rheumatoid Factor (RF). 4. **Sausage Digit:** Dactylitis is a key feature of Spondyloarthropathies (like PsA) but is absent in RA.

Question 50: A patient complains of hematuria. Renal biopsy demonstrates a focal necrotizing glomerulonephritis with crescent formation. The patient also complains of decreased sensation in the hands and feet. What is the next best investigation to diagnose the condition?

A. MIF (Microscopic Immunofluorescence)
B. Hepatitis B surface antigen (HbsAg)
C. Rheumatoid factor (RA factor)
D. Antineutrophil cytoplasmic antibodies (ANCA) (Correct Answer)

Explanation: **Explanation:** The clinical presentation describes a systemic small-vessel vasculitis. The combination of **focal necrotizing glomerulonephritis with crescent formation** [1] (a hallmark of Rapidly Progressive Glomerulonephritis or RPGN) and **decreased sensation** (suggestive of mononeuritis multiplex or peripheral neuropathy) points toward **ANCA-associated vasculitides (AAV)**, such as Granulomatosis with Polyangiitis (GPA), Microscopic Polyangiitis (MPA), or Eosinophilic Granulomatosis with Polyangiitis (EGPA) [2]. **Why ANCA is the correct answer:** ANCA testing is the primary serological investigation for these conditions. In a patient with "Pauci-immune" glomerulonephritis (necrotizing/crescentic lesions with little to no immune deposition), ANCA (c-ANCA/PR3 or p-ANCA/MPO) confirms the diagnosis [2] and helps differentiate it from other causes of pulmonary-renal syndromes. **Why other options are incorrect:** * **MIF (Microscopic Immunofluorescence):** While immunofluorescence is used on biopsy samples to confirm the "pauci-immune" status [3], it is a technique, not a specific diagnostic investigation for the systemic disease itself in this context. * **HbsAg:** This is strongly associated with **Polyarteritis Nodosa (PAN)**. However, PAN typically affects medium-sized vessels and **does not** cause glomerulonephritis. * **Rheumatoid Factor:** While it can be positive in various autoimmune conditions, it is non-specific and does not help diagnose necrotizing glomerulonephritis. **NEET-PG High-Yield Pearls:** * **Pauci-immune RPGN:** Always think of ANCA-associated vasculitis [1]. * **GPA (Wegener’s):** c-ANCA (PR3) positive; involves upper/lower respiratory tract + kidneys. * **MPA:** p-ANCA (MPO) positive; similar to GPA but lacks granulomatous inflammation and upper airway involvement. * **PAN:** Associated with Hepatitis B; characterized by "string of beads" appearance on angiography; **spares the lungs and glomeruli.**

Practice by Chapter

Rheumatoid Arthritis

Practice Questions

Spondyloarthropathies

Practice Questions

Systemic Lupus Erythematosus

Practice Questions

Vasculitis Syndromes

Practice Questions

Scleroderma and Related Disorders

Practice Questions

Inflammatory Myopathies

Practice Questions

Crystal Arthropathies

Practice Questions

Osteoarthritis

Practice Questions

Primary Immunodeficiency Disorders

Practice Questions

Autoinflammatory Syndromes

Practice Questions

Sjögren's Syndrome

Practice Questions

Antiphospholipid Syndrome

Practice Questions

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