Rheumatology and Immunology Practice Questions

Q: A 52-year-old female presents with dysphagia, heartburn, and joint pain in her fingers. She reports her fingers and toes turn blue upon exposure to cold. Physical examination reveals thickened skin of her fingers and tender, painful small lumps under the skin of the flexor surface of her hands, some of which drain a white, chalky substance. What is the clinical diagnosis?

CREST syndrome. **Explanation:** The patient presents with the classic pentad of **CREST syndrome**, a limited form of systemic sclerosis. The diagnosis is established by identifying the following clinical features: * **C**alcinosis cutis: The "white, chalky substance" draining from lumps on the flexor surfaces is calcium hydroxyapatite [2]. * **R**aynaud’s phenomenon: Fingers turning blue upon cold exposure. * **E**sophageal dysmotility: Manifesting as dysphagia and heartburn (due to lower esophageal sphincter incompetence). * **S**clerodactyly: Thickened skin of the fingers [1]. * **T**elangiectasia: (Though not explicitly mentioned, the other four features confirm the diagnosis). **Why other options are incorrect:** * **Systemic Lupus Erythematosus (SLE):** While SLE presents with joint pain and Raynaud’s, it typically features a malar rash, photosensitivity, and renal involvement, rather than calcinosis or esophageal dysmotility. * **Sjogren Syndrome:** Characterized primarily by sicca symptoms (dry eyes/mouth). While it can coexist with scleroderma, it does not explain the chalky skin deposits or skin thickening. * **Mikulicz Syndrome:** This is a historical term for bilateral enlargement of the lacrimal and salivary glands (often associated with IgG4-related disease or Sjogren’s), which does not match this patient's systemic presentation. **High-Yield Clinical Pearls for NEET-PG:** * **Antibody Association:** CREST syndrome is strongly associated with **Anti-centromere antibodies** (highly specific). Diffuse systemic sclerosis is associated with **Anti-Scl-70 (topoisomerase I)**. * **Pulmonary Complication:** The most common cause of death in CREST is **Pulmonary Arterial Hypertension (PAH)**, whereas in diffuse scleroderma, it is Interstitial Lung Disease (ILD) [2]. * **Treatment:** Raynaud’s is managed with Calcium Channel Blockers (e.g., Nifedipine). ACE inhibitors are life-saving for Scleroderma Renal Crisis [2].

Q: A 35-year-old female presented with pain in the metacarpophalangeal (MCP) and proximal interphalangeal (PIP) joints. What is the most likely diagnosis?

Rheumatoid Arthritis. ### Explanation **Correct Option: A. Rheumatoid Arthritis (RA)** The clinical presentation of a young to middle-aged female with symmetrical involvement of the small joints of the hands, specifically the **Metacarpophalangeal (MCP)** and **Proximal Interphalangeal (PIP)** joints, is the classic hallmark of Rheumatoid Arthritis [1]. RA typically spares the Distal Interphalangeal (DIP) joints [1]. The inflammatory nature of the disease often presents with morning stiffness lasting >1 hour and improves with activity [1]. **Why the other options are incorrect:** * **B. Rheumatic Fever:** Typically presents as a **migratory polyarthritis** affecting large joints (knees, ankles, elbows). It follows a streptococcal throat infection and rarely involves the small joints of the hand in a chronic pattern. * **C. Gouty Arthritis:** Characteristically presents as an acute, exquisite pain in a single joint (**monoarthritis**), most commonly the first metatarsophalangeal joint (Podagra). Chronic tophaceous gout can involve hands, but it is usually asymmetrical and associated with hyperuricemia. * **D. Psoriatic Arthritis:** While it involves small joints, it classically affects the **Distal Interphalangeal (DIP)** joints and is often associated with skin psoriasis, nail pitting, and "sausage digits" (dactylitis) [1]. **High-Yield Clinical Pearls for NEET-PG:** * **Joint Sparing:** RA characteristically **spares the DIP joints** and the axial skeleton (except the C1-C2 cervical spine) [1]. * **Deformities:** Late-stage RA features include **Swan-neck deformity** (PIP hyperextension, DIP flexion) and **Boutonniere deformity** (PIP flexion, DIP hyperextension). * **Serology:** **Anti-CCP (ACPA)** is more specific for RA than Rheumatoid Factor (RF) [1]. * **Radiology:** Earliest sign is juxta-articular osteopenia and soft tissue swelling; later signs include marginal erosions and joint space narrowing [1].

Q: Which of the following is NOT a common manifestation of Henoch-Schönlein purpura (HSP)?

Hemoptysis. **Explanation:** Henoch-Schönlein Purpura (HSP), now officially known as **IgA Vasculitis**, is a small-vessel systemic vasculitis characterized by the deposition of IgA-dominant immune complexes [1]. It typically presents with a classic clinical tetrad affecting the skin, joints, gastrointestinal tract, and kidneys. **Why Hemoptysis is the correct answer:** While HSP is a systemic vasculitis, it primarily involves the small vessels of the skin, gut, and glomeruli [2]. **Hemoptysis** (diffuse alveolar hemorrhage) is an **extremely rare** and life-threatening complication, rather than a common manifestation. Hemoptysis is more characteristically associated with ANCA-associated vasculitides (like Granulomatosis with Polyangiitis) or Anti-GBM disease (Goodpasture syndrome). **Analysis of incorrect options:** * **Purpura:** This is the hallmark of the disease. It typically presents as **palpable purpura** (without thrombocytopenia) distributed symmetrically over the buttocks and lower extremities [1]. * **Arthritis:** Migratory arthralgia or arthritis (usually involving the knees and ankles) occurs in about 75% of patients. It is typically non-deforming. * **Abdominal pain:** Colicky abdominal pain is common due to mucosal edema and submucosal hemorrhage [1]. It can lead to complications like **intussusception** (most common in children). **NEET-PG High-Yield Pearls:** * **Pathogenesis:** IgA1-dominant immune complex deposition [1]. * **Renal involvement:** Presents as microscopic hematuria; histologically identical to **IgA Nephropathy (Berger’s disease)** [3]. * **Platelet Count:** Always **normal** (distinguishes it from ITP). * **Trigger:** Often follows an Upper Respiratory Tract Infection (URTI) [1]. * **Diagnosis:** Primarily clinical; biopsy shows **leukocytoclastic vasculitis** with IgA deposits on immunofluorescence.

Q: Which of the following is the most frequent presenting symptom in patients with giant cell arteritis?

Headache. **Explanation:** **Giant Cell Arteritis (GCA)**, also known as temporal arteritis, is a large-vessel vasculitis that primarily affects individuals over the age of 50 [3]. **1. Why Headache is the correct answer:** **Headache** is the most common presenting symptom, occurring in approximately **two-thirds (60–90%)** of patients. It is typically new-onset, localized to the temporal or occipital regions, and may be associated with scalp tenderness (e.g., pain while combing hair) [1]. The underlying mechanism is the granulomatous inflammation of the branches of the external carotid artery. **2. Analysis of Incorrect Options:** * **B. Jaw claudication:** While this is the **most specific** symptom for GCA (highest positive likelihood ratio), it occurs in only about 50% of patients, making it less frequent than headache. * **C. Polymyalgia rheumatica (PMR):** PMR (proximal muscle pain and stiffness) is closely associated with GCA; about 40–50% of GCA patients have concurrent PMR [2]. However, it is a co-morbidity or associated syndrome rather than the primary presenting symptom of the vasculitis itself. * **D. Blindness:** Permanent visual loss (due to anterior ischemic optic neuropathy) is the most dreaded complication, but with modern diagnostic awareness, it occurs in only about 15–20% of patients [2]. **Clinical Pearls for NEET-PG:** * **Gold Standard Diagnosis:** Temporal artery biopsy (look for "skip lesions") [2]. * **Best Initial Test:** ESR (usually >50 mm/hr, often >100 mm/hr) and CRP [2]. * **Treatment:** Immediate high-dose corticosteroids (Prednisone) to prevent blindness; do not wait for biopsy results if clinical suspicion is high [2]. * **Key Association:** Strongly linked with the **HLA-DR4** haplotype.

Q: A 55-year-old male presented with history suggestive of Fever of Unknown Origin (FUO). He had multiple firm, discrete cervical lymphadenopathy. Serum angiotensin-converting enzyme was elevated in this patient. What is the most likely diagnosis?

Sarcoidosis. ### Explanation **Correct Answer: C. Sarcoidosis** The clinical presentation of persistent fever (FUO), cervical lymphadenopathy, and an **elevated Serum Angiotensin-Converting Enzyme (SACE)** level is a classic triad pointing towards **Sarcoidosis** [1]. * **Pathophysiology:** Sarcoidosis is a multisystem granulomatous disease characterized by non-caseating granulomas. The epithelioid cells within these granulomas produce ACE; hence, elevated SACE levels serve as a surrogate marker for the total body granuloma burden (though it lacks high specificity). * **Lymphadenopathy:** While bilateral hilar lymphadenopathy is most common, peripheral involvement (cervical, axillary) occurs in up to 30% of cases [1]. **Why other options are incorrect:** * **Non-Hodgkin’s Lymphoma (A) & CLL (D):** While both present with lymphadenopathy and fever (B-symptoms), they are typically associated with elevated LDH or specific peripheral smear/flow cytometry findings. They do not characteristically cause elevated SACE levels. * **Kikuchi Disease (B):** Also known as histiocytic necrotizing lymphadenitis, it presents with fever and cervical lymphadenopathy in younger patients. However, it is a self-limiting condition and is not associated with elevated SACE. **NEET-PG High-Yield Pearls:** 1. **SACE Levels:** Elevated in Sarcoidosis, but also seen in Gaucher’s disease, Leprosy, Hyperthyroidism, and Silicosis. 2. **Kveim-Siltzbach Test:** A historical skin test for Sarcoidosis (now rarely used). 3. **Biopsy Gold Standard:** Shows **non-caseating granulomas** with Asteroid bodies or Schaumann bodies. 4. **Lofgren’s Syndrome:** A specific acute presentation of Sarcoidosis consisting of the triad: Erythema nodosum, Bilateral hilar lymphadenopathy, and Arthralgia [1]. 5. **Hypercalcemia:** Occurs in Sarcoidosis due to 1-alpha-hydroxylase activity in macrophages, which increases Vitamin D activation [1].

Q: Henoch-Schonlein Purpura presents with deposition of which immunoglobulin?

IgA. **Explanation:** **Henoch-Schönlein Purpura (HSP)**, now formally known as **IgA Vasculitis**, is a small-vessel leukocytoclastic vasculitis [1]. The hallmark of its pathogenesis is the deposition of **immune complexes containing IgA1** (specifically galactose-deficient IgA1) within the walls of small vessels (capillaries, venules, and arterioles). This triggers an inflammatory response, leading to the classic clinical tetrad of palpable purpura, arthralgia, abdominal pain, and renal involvement (IgA nephropathy) [1]. **Analysis of Options:** * **Option C (IgA): Correct.** Immunofluorescence microscopy of skin or renal biopsy specimens characteristically shows granular IgA deposits [1]. This is the defining immunological feature of the disease. * **Option A (IgG) & B (IgM):** While IgG and IgM can sometimes be found co-deposited in HSP, they are not the primary or diagnostic immunoglobulins. IgG is more typically associated with Type II and III hypersensitivity reactions like SLE or Polyarteritis Nodosa. * **Option D (IgE):** IgE is associated with Type I hypersensitivity (atopy, asthma, anaphylaxis) and parasitic infections, not the systemic vasculitis seen in HSP. **High-Yield Clinical Pearls for NEET-PG:** 1. **Most Common:** HSP is the most common vasculitis in children, often following an **Upper Respiratory Tract Infection (URTI)**. 2. **Clinical Tetrad:** 1. Palpable Purpura (usually on buttocks/lower limbs); 2. Arthritis/Arthralgia; 3. Abdominal Pain (may lead to intussusception); 4. Renal involvement (hematuria). 3. **Diagnosis:** Primarily clinical; however, biopsy shows **Leukocytoclastic vasculitis** with IgA deposition [1]. 4. **Platelet Count:** Unlike ITP, the platelet count in HSP is **normal** (it is a non-thrombocytopenic purpura). 5. **Prognosis:** Generally excellent, but long-term morbidity is determined by the severity of renal involvement.

Q: A 20-year-old male presents with a 2-week history of knee joint pain. He also reports mild backache and stiffness. Four weeks prior to these complaints, he experienced fever and diarrhea. The patient is currently afebrile and has no diarrhea. His laboratory investigations reveal normal ESR and elevated CRP. What is the most probable diagnosis?

Reactive arthritis. The clinical presentation is classic for **Reactive Arthritis (ReA)**, a member of the Spondyloarthritides (SpA) group. [3] **Why Reactive Arthritis is correct:** Reactive arthritis is an asymmetric inflammatory oligoarthritis that typically follows 1–4 weeks after a gastrointestinal (e.g., *Salmonella, Shigella, Campylobacter*) or urogenital (e.g., *Chlamydia*) infection. [2] This patient’s timeline (diarrhea 4 weeks prior followed by knee pain and back stiffness) fits perfectly. [3] It typically affects young adults and involves large weight-bearing joints (knees/ankles) and the axial skeleton (backache). [3] Elevated CRP is a common marker of acute inflammation, even if ESR is normal. **Why other options are incorrect:** * **Ankylosing Spondylitis:** While it causes backache, it is a chronic, progressive disease characterized by sacroiliitis. [1] It does not typically follow an acute diarrheal episode. * **Rheumatoid Arthritis:** Usually presents as a symmetric, small-joint polyarthritis (PIP, MCP) and is not preceded by an enteric infection. * **Enteropathic Arthritis:** This is arthritis associated with active Inflammatory Bowel Disease (Crohn’s or Ulcerative Colitis). This patient had a transient episode of fever and diarrhea (suggestive of infectious enteritis), not chronic IBD. **NEET-PG High-Yield Pearls:** * **Reiter’s Syndrome Triad:** Arthritis, Urethritis, and Conjunctivitis ("Can't see, can't pee, can't climb a tree"). [3] * **HLA-B27:** Strongly associated (up to 80% of cases). [2] * **Extra-articular features:** Keratoderma blennorrhagicum (skin lesions on palms/soles) and Circinate balanitis. [3] * **Treatment:** NSAIDs are the first-line therapy; antibiotics do not treat the arthritis once it has started.

Q: A 35-year-old woman presents with a red rash over her cheeks and pain and swelling in both knees and several small joints in her hands. She has a history of hypertension and was started on a new medication for blood pressure control two months ago. Her ANA and anti-histone antibodies are positive. Which of the following medications is most likely to have caused her condition?

hydralazine. ### Explanation **Correct Option: A. Hydralazine** The clinical presentation describes **Drug-Induced Lupus Erythematosus (DILE)**. The patient exhibits classic symptoms of systemic lupus (malar rash, polyarthritis) following the initiation of a new antihypertensive [1]. The presence of **anti-histone antibodies** is the hallmark serological marker for DILE, occurring in >95% of cases. Hydralazine is one of the most common drugs associated with this condition, especially in "slow acetylators" (individuals who metabolize the drug slowly via the N-acetyltransferase enzyme). **Incorrect Options:** * **B. Hydrochlorothiazide:** While thiazides can cause photosensitivity or trigger subacute cutaneous lupus erythematosus (SCLE), they are not typically associated with the classic anti-histone positive DILE syndrome [2]. * **C. Ramipril:** ACE inhibitors are not recognized causes of drug-induced lupus. Their common side effects include cough and angioedema [2]. * **D. Nifedipine:** Calcium channel blockers are not associated with DILE. **High-Yield Clinical Pearls for NEET-PG:** * **Common Culprits (SHIPP):** **S**ulfonamides, **H**ydralazine, **I**soniazid, **P**henytoin, **P**rocainamide (Procainamide has the highest risk; Hydralazine is the most common in clinical practice). * **Serology:** Anti-histone antibodies are positive in DILE, but **Anti-dsDNA is usually negative** (unlike idiopathic SLE) [3]. Complement levels (C3, C4) are typically normal [3]. * **Clinical Features:** DILE usually presents with pleuropericarditis, fever, and arthralgia. Notably, **renal and CNS involvement are rare** compared to idiopathic SLE. * **Management:** Symptoms typically resolve within weeks after discontinuing the offending drug.

Question 1

A 52-year-old female presents with dysphagia, heartburn, and joint pain in her fingers. She reports her fingers and toes turn blue upon exposure to cold. Physical examination reveals thickened skin of her fingers and tender, painful small lumps under the skin of the flexor surface of her hands, some of which drain a white, chalky substance. What is the clinical diagnosis?

Accepted Answer

CREST syndrome

Answer

Systemic lupus erythematosus

Answer

Sjogren Syndrome

Answer

Mikulicz syndrome

Question 2

A 35-year-old female presented with pain in the metacarpophalangeal (MCP) and proximal interphalangeal (PIP) joints. What is the most likely diagnosis?

Accepted Answer

Rheumatoid Arthritis

Answer

Rheumatic Fever

Answer

Gouty Arthritis

Answer

Psoriatic Arthritis

Question 3

Which of the following is NOT a common manifestation of Henoch-Schönlein purpura (HSP)?

Accepted Answer

Hemoptysis

Answer

Purpura

Answer

Arthritis

Answer

Abdominal pain

Question 4

Which of the following is more frequently seen in Churg Strauss Syndrome compared to Wegener's Granulomatosis?

Accepted Answer

Lower Respiratory Tract involvement

Answer

Renal involvement

Answer

Eye involvement

Answer

Upper Respiratory Tract involvement

Question 5

Which of the following is the most frequent presenting symptom in patients with giant cell arteritis?

Accepted Answer

Headache

Answer

Jaw claudication

Answer

Polymyalgia rheumatica

Answer

Blindness

Question 6

A 55-year-old male presented with history suggestive of Fever of Unknown Origin (FUO). He had multiple firm, discrete cervical lymphadenopathy. Serum angiotensin-converting enzyme was elevated in this patient. What is the most likely diagnosis?

Accepted Answer

Sarcoidosis

Answer

Non-Hodgkin's lymphoma

Answer

Kikuchi disease

Answer

Chronic lymphocytic leukemia

Question 7

Henoch-Schonlein Purpura presents with deposition of which immunoglobulin?

Accepted Answer

IgA

Answer

IgG

Answer

IgM

Answer

IgE

Question 8

A 20-year-old male presents with a 2-week history of knee joint pain. He also reports mild backache and stiffness. Four weeks prior to these complaints, he experienced fever and diarrhea. The patient is currently afebrile and has no diarrhea. His laboratory investigations reveal normal ESR and elevated CRP. What is the most probable diagnosis?

Accepted Answer

Reactive arthritis

Answer

Ankylosing spondylitis

Answer

Rheumatoid arthritis

Answer

Enteropathic arthritis

Question 9

Which of the following is NOT an indication for steroid use in Systemic Lupus Erythematosus?

Accepted Answer

Endocarditis

Answer

Myocarditis

Answer

Thrombocytopenia

Answer

Neuropsychiatric symptoms

Question 10

A 35-year-old woman presents with a red rash over her cheeks and pain and swelling in both knees and several small joints in her hands. She has a history of hypertension and was started on a new medication for blood pressure control two months ago. Her ANA and anti-histone antibodies are positive. Which of the following medications is most likely to have caused her condition?

Accepted Answer

hydralazine

Answer

hydrochlorothiazide

Answer

ramipril

Answer

nifedipine

Rheumatology and Immunology — MCQs

Rheumatology and Immunology — MCQs

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