Rheumatology and Immunology Practice Questions

Q: A biopsy of the lower lip salivary glands showed replacement of parenchymal tissue by lymphocytes. The patient also had xerostomia and keratoconjunctivitis sicca. These findings are indicative of which of the following?

Sjögren’s syndrome. ### Explanation **Correct Answer: D. Sjögren’s syndrome** **1. Why it is correct:** Sjögren’s syndrome is a chronic autoimmune disorder characterized by lymphocytic infiltration of exocrine glands, primarily the lacrimal and salivary glands. The clinical triad presented—**xerostomia** (dry mouth), **keratoconjunctivitis sicca** (dry eyes), and a **lip biopsy** showing focal lymphocytic sialadenitis—is classic for this condition. The replacement of functional parenchymal tissue by lymphocytes (specifically CD4+ T cells and B cells) leads to glandular dysfunction and the resulting "sicca symptoms." **2. Why the other options are incorrect:** * **A. Lymphoma:** While patients with Sjögren’s have a 40-fold increased risk of developing B-cell MALT lymphoma, the biopsy described (lymphocytic replacement of parenchyma) is the diagnostic hallmark of the underlying autoimmune process, not a malignancy itself. * **B. Crohn’s disease:** This is an inflammatory bowel disease. While it can have extra-intestinal manifestations, it typically presents with granulomatous inflammation of the GI tract, not primary lymphocytic infiltration of salivary glands. * **C. Mumps:** This is an acute viral infection (Paramyxovirus) causing painful parotitis. It does not cause chronic sicca symptoms or the specific histopathological pattern of parenchymal replacement seen in autoimmune diseases. **3. High-Yield Clinical Pearls for NEET-PG:** * **Antibodies:** Highly associated with **Anti-Ro (SS-A)** and **Anti-La (SS-B)**. * **Diagnostic Test:** The **Schirmer’s test** measures tear production (positive if <5 mm in 5 minutes). * **Histology:** The "Focus Score" is used; a score of ≥1 focus (aggregate of 50+ lymphocytes) per 4 $mm^2$ of glandular tissue is diagnostic. * **Complication:** Always monitor for **MALT Lymphoma**, often signaled by persistent parotid swelling or loss of previously positive rheumatoid factor.

Q: Which of the following is NOT a criterion for Juvenile Rheumatoid Arthritis?

All of the above. To understand this question, it is essential to recall the diagnostic criteria for **Juvenile Idiopathic Arthritis (JIA)**, formerly known as Juvenile Rheumatoid Arthritis (JRA) [1]. ### **Explanation of the Correct Answer** The correct answer is **D (All of the above)** because none of the individual options (A, B, or C) represent the actual diagnostic criteria. According to the ILAR (International League of Associations for Rheumatology) classification: 1. **Age of Onset:** Must be **less than 16 years**. Option A says "greater than 16," which is incorrect. 2. **Duration of Arthritis:** Must be **6 weeks or longer** [1]. Option B (3 weeks) and Option C (6 months) are both incorrect timeframes. Since all three statements are factually incorrect regarding the criteria, "All of the above" is the appropriate choice for a "NOT a criterion" question. ### **Analysis of Options** * **Option A:** Incorrect because JIA is defined by onset in childhood/adolescence (<16 years). Onset after 16 is classified as adult-onset inflammatory arthritis. * **Option B:** Incorrect because 3 weeks is too short. This duration often includes transient viral or post-streptococcal arthritides. * **Option C:** Incorrect because while the disease may last 6 months, the diagnostic requirement is only 6 weeks to ensure chronicity. ### **NEET-PG High-Yield Pearls** * **Definition:** Arthritis of unknown etiology in at least one joint for at least 6 weeks in a child <16 years [1]. * **Systemic JIA (Still’s Disease):** Characterized by daily "quotidian" spiking fevers and an evanescent (fleeting) salmon-pink rash [1]. * **Ocular Complication:** Chronic asymptomatic **Anterior Uveitis** is common, especially in Oligoarticular JIA with **ANA positivity**. Regular slit-lamp exams are mandatory. * **RF Factor:** Most JIA patients are RF negative; RF positivity is typically seen only in the polyarticular subtype (older girls).

Q: Sjögren's syndrome consists of all of the following EXCEPT?

Xerophthalmia. ### Explanation Sjögren's syndrome is a chronic systemic autoimmune disease characterized by lymphocytic infiltration of exocrine glands, primarily the lacrimal and salivary glands. **Why "Xerophthalmia" is the correct answer (The Exception):** In the context of this specific question, **Xerophthalmia** is considered the "odd one out" because it is a broad clinical term describing dry eyes often associated with **Vitamin A deficiency**, leading to Bitot's spots and night blindness. While Sjögren's does cause dry eyes, the specific medical term used to describe the inflammatory ocular pathology in this syndrome is **Keratoconjunctivitis sicca (KCS)**, which involves corneal and conjunctival epithelial damage due to aqueous tear deficiency. **Analysis of other options:** * **Keratoconjunctivitis sicca (Option D):** This is the hallmark ocular manifestation of Sjögren's, caused by lymphocytic destruction of the lacrimal glands [1]. * **Chronic arthritis (Option B):** Approximately 50% of patients with Sjögren's develop a symmetric, non-erosive chronic arthritis similar to Rheumatoid Arthritis. * **Chronic dermatitis (Option C):** Cutaneous involvement is common, presenting as xeroderma (dry skin), palpable purpura (vasculitis), or annular erythema. **NEET-PG High-Yield Pearls:** 1. **Primary vs. Secondary:** Primary Sjögren's occurs alone; Secondary Sjögren's is most commonly associated with **Rheumatoid Arthritis** [1]. 2. **Antibodies:** Most specific is **Anti-La (SS-B)**; most sensitive/common are **Anti-Ro (SS-A)** and **ANA**. 3. **Diagnostic Gold Standard:** Minor salivary gland biopsy showing **focal lymphocytic sialadenitis** (Focus score ≥1). 4. **Malignancy Risk:** Patients have a 40-fold increased risk of **B-cell MALT Lymphoma** (watch for persistent parotid swelling). 5. **Schirmer’s Test:** Used to quantify tear production (Positive if <5 mm in 5 minutes).

Q: Which of the following is absent in CREST syndrome?

Endocrine disorders. Explanation: CREST syndrome is a limited form of Systemic Sclerosis (Scleroderma). It is characterized by a specific constellation of clinical features represented by its acronym. 1. Why "Endocrine disorders" is the correct answer: Endocrine dysfunction is not a component of the CREST acronym or the underlying pathophysiology of systemic sclerosis. While patients with autoimmune diseases can occasionally have co-existing conditions (like Hashimoto’s thyroiditis), endocrine failure is not a diagnostic or characteristic feature of this syndrome. 2. Analysis of Incorrect Options (Components of CREST): * C – Calcinosis cutis: Calcium deposits in the skin and soft tissues, often seen on fingertips or over joints. * R – Raynaud's phenomenon: Episodic vasospasm of digits in response to cold or stress; often the earliest manifestation. * E – Esophageal dysmotility: Resulting from neuromuscular dysfunction and fibrosis, leading to dysphagia and GERD. * S – Sclerodactyly: Thickening and tightening of the skin of the fingers and toes, giving them a "tapered" appearance [1]. * T – Telangiectasis: Dilated small blood vessels, typically on the face, mucous membranes, and palms. NEET-PG High-Yield Pearls: * Antibody Marker: The most specific marker for CREST syndrome is Anti-centromere antibody (present in ~90% of cases). * Prognosis: Limited Scleroderma (CREST) generally has a better prognosis than Diffuse Cutaneous Systemic Sclerosis (dcSSc), which is associated with Anti-Scl-70 (Anti-topoisomerase I) antibodies. * Major Complication: The most feared late complication of CREST syndrome is Pulmonary Arterial Hypertension (PAH), whereas diffuse disease is more associated with Interstitial Lung Disease (ILD).

Q: Palpable purpura is caused by?

All of the above. **Explanation:** **Palpable purpura** is the clinical hallmark of **leukocytoclastic vasculitis**. Unlike non-inflammatory purpura (caused by trauma or thrombocytopenia), these lesions are raised because the underlying inflammation leads to vessel wall damage, edema, and the extravasation of red blood cells into the dermis [1]. * **Henoch-Schönlein Purpura (HSP):** This is a small-vessel vasculitis characterized by IgA immune complex deposition [2]. It typically presents with the classic tetrad of palpable purpura (usually on the lower extremities), arthralgia, abdominal pain, and renal involvement. * **Microscopic Polyangiitis (MPA):** This is a pauci-immune small-vessel vasculitis. Because it affects capillaries and post-capillary venules in the skin, palpable purpura is a common cutaneous manifestation [1]. * **Polyarteritis Nodosa (PAN):** While PAN is primarily a medium-vessel vasculitis, it frequently involves the small vessels of the skin. Cutaneous manifestations include livedo reticularis, skin ulcers, and palpable purpura. **Why "All of the above" is correct:** Palpable purpura occurs whenever there is inflammation of the small cutaneous blood vessels. Since all three conditions listed involve small-vessel inflammation (either as the primary pathology or as part of a systemic process), they can all present with this clinical finding. **High-Yield Clinical Pearls for NEET-PG:** 1. **Non-palpable purpura** is usually due to **thrombocytopenia** or vascular fragility (e.g., Senile purpura, Scurvy). 2. **HSP** is the most common vasculitis in children; always look for **IgA deposits** on skin biopsy [2]. 3. **PAN** is strongly associated with **Hepatitis B** infection and characteristically **spares the lungs**. 4. **Microscopic Polyangiitis** is most commonly associated with **p-ANCA** (MPO-antibodies).

Q: A 57-year-old woman presents with frequent headaches, scalp tenderness, arthralgias, fatigue, and jaw discomfort during mastication. Examination reveals a tender right temporal artery. Her erythrocyte sedimentation rate (ESR) is 50 mm/h and hemoglobin is 10.5 g/dL. A temporal artery biopsy has been obtained for diagnostic confirmation. What is the most appropriate next step in management?

Oral prednisone 40 mg/day. ### Explanation **1. Why Option D is Correct:** The patient presents with classic features of **Giant Cell Arteritis (GCA)**, also known as Temporal Arteritis: headache, scalp tenderness, jaw claudication (highly specific), and elevated ESR. In cases of GCA **without visual loss**, the standard of care is immediate initiation of **high-dose oral corticosteroids** (typically Prednisone 40–60 mg/day). Treatment should never be delayed while awaiting biopsy results, as the risk of irreversible blindness (due to anterior ischemic optic neuropathy) is high [1]. **2. Why Other Options are Incorrect:** * **Option A (IV high-dose steroids):** Intravenous "pulse" methylprednisolone is reserved for patients presenting with **acute visual loss** or neurological deficits to prevent further deterioration. This patient has no visual symptoms. * **Option B (Acetylsalicylic acid):** While low-dose aspirin may reduce the risk of stroke in GCA, it is an **adjunctive** therapy and not the primary treatment for the underlying vasculitis. * **Option C (Indomethacin):** This is the treatment of choice for *Hemicrania continua* or *Paroxysmal hemicrania*. It has no role in treating the systemic inflammation of GCA and will not prevent blindness. **3. High-Yield Clinical Pearls for NEET-PG:** * **Association:** ~50% of GCA patients have **Polymyalgia Rheumatica (PMR)** (proximal muscle pain/stiffness). * **Diagnosis:** Temporal artery biopsy is the gold standard [1]. Due to "skip lesions," a long segment (2–3 cm) must be biopsied. * **Histopathology:** Granulomatous inflammation of the media with fragmented internal elastic lamina and multinucleated giant cells [1]. * **Lab Findings:** Characteristically high ESR (>50 mm/h) and Normocytic Normochromic Anemia (Anemia of Chronic Disease). * **Steroid Sparing Agent:** **Tocilizumab** (IL-6 inhibitor) is now FDA-approved for GCA to help taper steroids.

Q: A patient experiencing an acute attack of gout is intolerant to NSAIDs. Which medication should be administered?

Steroid. **Explanation:** The management of an **acute gouty attack** focuses on rapid anti-inflammatory relief. The first-line agents are typically NSAIDs (e.g., Indomethacin, Naproxen), Colchicine, or Corticosteroids. **Why Steroids are correct:** When a patient is intolerant to NSAIDs (due to reasons like peptic ulcer disease, chronic kidney disease, or hypersensitivity) or if NSAIDs are contraindicated, **Corticosteroids** (oral, intravenous, or intra-articular) are the preferred alternative. They effectively suppress the inflammatory response triggered by monosodium urate crystals. **Why the other options are incorrect:** * **A & D (Allopurinol and Febuxostat):** These are **Xanthine Oxidase Inhibitors (XOIs)** used for *chronic* management to lower serum uric acid levels [1]. They should **never** be initiated during an acute attack, as a rapid drop in urate levels can mobilize crystals from tissues, potentially worsening or prolonging the acute inflammation [1]. * **C (Probenecid):** This is a **uricosuric agent** that increases uric acid excretion in the urine. Like XOIs, it is used for chronic prophylaxis and has no role in treating acute inflammation [1]. **Clinical Pearls for NEET-PG:** * **Drug of Choice (DOC):** NSAIDs are generally the first-line treatment for acute gout. * **Colchicine:** Most effective if started within 12–24 hours of symptom onset. Watch for diarrhea as a common side effect. * **The "Golden Rule":** Do not start or stop urate-lowering therapy (Allopurinol) during an acute attack [1]. If the patient is already on it, continue the same dose; if they are not on it, wait 2 weeks after the attack resolves before starting. * **Target Urate Level:** Aim for <6 mg/dL in chronic management.

Q: Libman-Sacks endocarditis is typically seen in which condition?

Systemic lupus erythematosus (SLE). **Explanation:** **Libman-Sacks Endocarditis (LSE)** is a form of **non-bacterial verrucous endocarditis** classically associated with **Systemic Lupus Erythematosus (SLE)**. It is characterized by small, sterile, fibro-fibrinous vegetations that can occur on any part of the heart valve surface (undersurface, chordae tendineae, or endocardial surfaces), though they most commonly affect the mitral and aortic valves. **Why Option B is Correct:** In SLE, immune complex deposition and subsequent complement activation lead to chronic inflammation of the endocardium [1]. Unlike infective endocarditis, these vegetations are **sterile** (non-infectious) and consist of fibrin, inflammatory cells, and hematoxylin bodies (the only pathognomonic finding). **Analysis of Incorrect Options:** * **A. Rheumatic Fever:** Characterized by small, friable vegetations (verrucae) along the **lines of closure** of the valves, leading to commissural fusion and fish-mouth stenosis [2]. * **C. Infective Endocarditis:** Involves **large, friable, destructive vegetations** containing bacteria/fungi, usually associated with fever and systemic emboli [2]. * **D. Hypercoagulable States:** Associated with **Non-Bacterial Thrombotic Endocarditis (NBTE)** or Marantic endocarditis, typically seen in advanced malignancies (e.g., mucinous adenocarcinoma). **High-Yield Clinical Pearls for NEET-PG:** * **Location:** LSE vegetations are unique because they can occur on **both sides** of the valve leaflets (pathognomonic feature). * **Association:** Strongly linked with **Antiphospholipid Antibody Syndrome (APS)** in SLE patients [1]. * **Complication:** While often asymptomatic, it can lead to valve regurgitation or act as a source for systemic embolization (embolic stroke). * **Treatment:** Focuses on managing the underlying SLE and anticoagulation if embolic events occur.

Question 1

A biopsy of the lower lip salivary glands showed replacement of parenchymal tissue by lymphocytes. The patient also had xerostomia and keratoconjunctivitis sicca. These findings are indicative of which of the following?

Accepted Answer

Sjögren’s syndrome

Answer

Lymphoma

Answer

Crohn’s disease

Answer

Mumps

Question 2

Which of the following is NOT a typical feature of Behcet's syndrome?

Accepted Answer

Hepato-splenic involvement

Answer

Arthritis

Answer

Mucocutaneous involvement

Answer

Neuro-ocular involvement

Question 3

Which of the following is NOT a criterion for Juvenile Rheumatoid Arthritis?

Accepted Answer

All of the above

Answer

Age greater than 16 years

Answer

Arthritis duration greater than 3 weeks

Answer

Arthritis duration greater than 6 months

Question 4

Sjögren's syndrome consists of all of the following EXCEPT?

Accepted Answer

Xerophthalmia

Answer

Chronic arthritis

Answer

Chronic dermatitis

Answer

Keratoconjunctivitis sicca

Question 5

Which of the following is absent in CREST syndrome?

Accepted Answer

Endocrine disorders

Answer

Calcinosis cutis

Answer

Raynaud's phenomenon

Answer

Telangiectasis

Question 6

Palpable purpura is caused by?

Accepted Answer

All of the above

Answer

Henoch-Schonlein purpura (HSP)

Answer

Polyarteritis nodosa (PAN)

Answer

Microscopic polyangiitis

Question 7

A 57-year-old woman presents with frequent headaches, scalp tenderness, arthralgias, fatigue, and jaw discomfort during mastication. Examination reveals a tender right temporal artery. Her erythrocyte sedimentation rate (ESR) is 50 mm/h and hemoglobin is 10.5 g/dL. A temporal artery biopsy has been obtained for diagnostic confirmation. What is the most appropriate next step in management?

Accepted Answer

Oral prednisone 40 mg/day

Answer

Intravenous high-dose steroids

Answer

Acetylsalicylic acid

Answer

Indomethacin

Question 8

Oligoarthritis with ascending joint involvement is seen in?

Accepted Answer

Seronegative arthritis

Answer

Juvenile osteoarthritis

Answer

SLE

Answer

Septic arthritis

Question 9

A patient experiencing an acute attack of gout is intolerant to NSAIDs. Which medication should be administered?

Accepted Answer

Steroid

Answer

Allopurinol

Answer

Probenecid

Answer

Febuxostat

Question 10

Libman-Sacks endocarditis is typically seen in which condition?

Accepted Answer

Systemic lupus erythematosus (SLE)

Answer

Rheumatic fever

Answer

Infective endocarditis

Answer

Hypercoagulable states

Rheumatology and Immunology — MCQs

Rheumatology and Immunology — MCQs

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