Rheumatology and Immunology — MCQs
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A 37-year-old woman presents with complaints of severe heartburn with or without meals. She has a history of hypertension, which has been treated with captopril. She also has a history of Raynaud disease, multiple facial telangiectasias, and very taut skin on the dorsum of both hands. She has failed to obtain relief for her heartburn with large doses of antacids or omeprazole. Esophageal manometry is ordered. What are the most likely results of this test?
A patient suffering from morning stiffness lasting more than 1 hour presents with the following abnormality in the X-ray. What is the most possible diagnosis?
All of the following is true about Felty syndrome, except?
Felty's syndrome is associated with which of the following conditions?
Which of the following organs is least likely to be involved in Behcet's disease?
Which of the following is FALSE regarding antiphospholipid antibody syndrome (APLA)?
Reactive arthritis is associated with which of the following pathogens?
Which test evaluates lacrimal gland function for the diagnosis of Sjogren syndrome?
A man developed frequent facial swelling with occasional laryngeal edema. What is the most probable diagnosis?
Felty's syndrome is associated with which of the following?
Rheumatology and Immunology Indian Medical PG Practice Questions and MCQs
Question 371: A 37-year-old woman presents with complaints of severe heartburn with or without meals. She has a history of hypertension, which has been treated with captopril. She also has a history of Raynaud disease, multiple facial telangiectasias, and very taut skin on the dorsum of both hands. She has failed to obtain relief for her heartburn with large doses of antacids or omeprazole. Esophageal manometry is ordered. What are the most likely results of this test?
- A. Decreased esophageal peristalsis and decreased LES pressure (Correct Answer)
- B. Decreased esophageal peristalsis and increased LES pressure
- C. Increased esophageal peristalsis and decreased LES pressure
- D. Increased esophageal peristalsis and increased LES pressure
Explanation: ### Explanation **1. Why Option A is Correct:** The clinical presentation—Raynaud phenomenon, telangiectasias, sclerodactyly (taut skin), and hypertension—is diagnostic of **Systemic Sclerosis (Scleroderma)**, specifically the limited cutaneous subtype (CREST syndrome) [1]. In Scleroderma, the underlying pathophysiology involves vascular damage and excessive collagen deposition (fibrosis) [1]. In the esophagus, this leads to: * **Atrophy of the smooth muscle** in the distal two-thirds of the esophagus. * **Fibrosis** of the esophageal wall [3]. This results in **decreased or absent peristalsis** (due to muscle atrophy) and a **decreased Lower Esophageal Sphincter (LES) pressure** (due to the inability of the weakened sphincter to maintain tone) [3]. This combination allows for severe gastroesophageal reflux, often refractory to standard doses of PPIs. **2. Why Other Options are Wrong:** * **Options B & D (Increased LES pressure):** Increased LES pressure is characteristic of **Achalasia Cardia** [2]. In Achalasia, the LES fails to relax, whereas in Scleroderma, the LES is "incompetent" or "patulous" (low pressure). * **Options C & D (Increased peristalsis):** Increased or uncoordinated peristalsis is seen in **Diffuse Esophageal Spasm (DES)** or Nutcracker Esophagus [3]. Scleroderma is characterized by a "hypomotile" or "aperistaltic" esophagus. **3. NEET-PG High-Yield Pearls:** * **Scleroderma Esophagus:** Affects the distal 2/3 (smooth muscle portion). The proximal 1/3 (striated muscle) is usually spared. * **Manometry Gold Standard:** Shows "Low-amplitude" or absent contractions in the distal esophagus + Low LES pressure. * **Barium Swallow:** May show a "glass tube" appearance of the esophagus. * **CREST Syndrome:** **C**alcinosis, **R**aynaud's, **E**sophageal dysmotility, **S**clerodactyly, **T**elangiectasia [3]. * **Renal Crisis:** The patient’s hypertension and use of Captopril (an ACE inhibitor) are classic; ACE inhibitors are the drug of choice for Scleroderma Renal Crisis.
Question 372: A patient suffering from morning stiffness lasting more than 1 hour presents with the following abnormality in the X-ray. What is the most possible diagnosis?
- A. Psoriatic arthritis
- B. Rheumatoid arthritis (Correct Answer)
- C. Osteoarthritis
- D. Relapsing polychondritis
Explanation: ***Rheumatoid arthritis*** - **Morning stiffness lasting more than 1 hour** is a hallmark feature of RA, indicating significant **inflammatory synovitis**. - Classic X-ray findings include **periarticular osteoporosis**, **symmetric joint space narrowing**, and **marginal erosions** at **MCP/PIP joints**. *Psoriatic arthritis* - Typically presents with **asymmetric joint involvement** and characteristic **pencil-in-cup deformity** on X-ray. - Associated with **psoriatic skin lesions** and often affects **DIP joints**, unlike the pattern described. *Osteoarthritis* - Morning stiffness is typically **less than 30 minutes**, indicating mechanical rather than inflammatory pathology. - X-ray shows **osteophytes**, **joint space narrowing**, and commonly affects **DIP joints** with **Heberden's nodes**. *Relapsing polychondritis* - Primarily affects **cartilaginous structures** like ears, nose, and larynx rather than synovial joints. - Does not typically cause the **joint erosions** and **symmetric arthropathy** seen in inflammatory arthritis.
Question 373: All of the following is true about Felty syndrome, except?
- A. Splenomegaly
- B. Increased WBC counts (Correct Answer)
- C. Rheumatoid arthritis
- D. None of the above
Explanation: Felty Syndrome is a rare but serious complication of long-standing, seropositive Rheumatoid Arthritis. It is classically defined by a **clinical triad**: [1] 1. **Rheumatoid Arthritis (RA):** Usually severe, erosive, and chronic. 2. **Splenomegaly:** Enlargement of the spleen. 3. **Neutropenia:** A significant decrease in the absolute neutrophil count (ANC < 2000/mm³), which leads to **decreased total WBC counts (Leukopenia)**. **Why Option B is the correct answer:** The hallmark of Felty syndrome is **neutropenia** (low WBCs), not increased WBC counts. The low neutrophil count is caused by a combination of splenic sequestration and immune-mediated destruction of neutrophils. Therefore, "Increased WBC counts" is the false statement. **Analysis of other options:** * **Option A (Splenomegaly):** This is a core component of the diagnostic triad. While the degree of enlargement varies, it is present in almost all cases. [1] * **Option C (Rheumatoid Arthritis):** Felty syndrome typically occurs in patients who have had RA for over 10 years, often with extra-articular manifestations like nodules or vasculitis. [1] **NEET-PG High-Yield Pearls:** * **Serology:** Almost 100% of patients are positive for **Rheumatoid Factor (RF)** and often have high titers of anti-CCP. [1] * **HLA Association:** Strongly associated with **HLA-DR4**. * **Complications:** Patients are at high risk for recurrent bacterial infections (due to neutropenia) and lower extremity skin ulcers. * **Differential:** Must be distinguished from **Large Granular Lymphocyte (LGL) leukemia**, which presents similarly with RA, splenomegaly, and neutropenia.
Question 374: Felty's syndrome is associated with which of the following conditions?
- A. Thyrotoxicosis
- B. Splenomegaly (Correct Answer)
- C. Hepatomegaly
- D. None of the above
Explanation: **Explanation:** Felty’s syndrome is a rare but severe extra-articular manifestation of long-standing, seropositive **Rheumatoid Arthritis (RA)** [1]. It is classically defined by a clinical triad: 1. **Rheumatoid Arthritis** (usually erosive and deforming) [1] 2. **Splenomegaly** (the correct answer) [1] 3. **Neutropenia** (Absolute Neutrophil Count < 2000/mm³) **Why Splenomegaly is correct:** The pathophysiology involves the sequestration and destruction of neutrophils within the spleen, coupled with the production of autoantibodies against neutrophils. Splenomegaly is a hallmark feature, though the size of the spleen does not always correlate with the severity of the neutropenia [1]. **Analysis of incorrect options:** * **Thyrotoxicosis:** There is no direct association between Felty’s syndrome and hyperthyroidism. While RA is an autoimmune disease and patients may have co-existing autoimmune thyroiditis (Hashimoto’s), thyrotoxicosis is not a component of this syndrome. * **Hepatomegaly:** While some patients may exhibit mild liver enlargement or abnormal liver function tests (e.g., nodular regenerative hyperplasia), it is not a defining characteristic of the syndrome. Splenomegaly is the specific reticuloendothelial finding required for diagnosis. **High-Yield Clinical Pearls for NEET-PG:** * **HLA Association:** Strongly associated with **HLA-DR4**. * **Risk of Infection:** The primary clinical concern is recurrent pyogenic infections (skin and respiratory) due to profound neutropenia. * **SANSET:** A helpful mnemonic for Felty’s is **S**plenomegaly, **A**neamia, **N**eutropenia, **S**eropositive RA, **E**xudative joints, and **T**hrombocytopenia. * **Large Granular Lymphocyte (LGL) Leukemia:** This condition mimics Felty’s syndrome and must be considered in the differential diagnosis.
Question 375: Which of the following organs is least likely to be involved in Behcet's disease?
- A. CNS
- B. Liver (Correct Answer)
- C. Joints
- D. Eyes
Explanation: Behcet’s Disease is a chronic, multisystemic, relapsing inflammatory disorder classified as a **variable vessel vasculitis**. It is unique because it can involve blood vessels of all sizes (small, medium, and large) on both the arterial and venous sides [1]. **Why Liver is the correct answer:** While Behcet’s disease is multisystemic, it characteristically **spares the liver parenchyma**. While rare vascular complications like Budd-Chiari syndrome (hepatic vein thrombosis) can occur due to the disease's prothrombotic nature, the liver is not a primary target organ for the inflammatory process. In contrast, the skin, mucosa, eyes, joints, and CNS are classic sites of involvement. **Analysis of Incorrect Options:** * **CNS (Neuro-Behcet’s):** Involved in about 5–10% of cases. It typically presents as parenchymal disease (brainstem involvement) or non-parenchymal disease (dural sinus thrombosis). * **Joints:** Arthritis or arthralgia occurs in approximately 50% of patients. It is usually a non-erosive, asymmetrical oligoarthritis, most commonly affecting the knees and ankles. * **Eyes:** Ocular involvement is a hallmark of the disease (70% of cases). It typically presents as **bilateral panuveitis** or posterior uveitis, which can lead to blindness if untreated. **High-Yield Clinical Pearls for NEET-PG:** 1. **Classic Triad:** Recurrent oral ulcers (most common), genital ulcers (most specific), and uveitis. 2. **Pathergy Test:** A positive skin hyperreactivity test (formation of a sterile pustule 24–48 hours after a needle prick) is highly specific for Behcet’s. 3. **HLA Association:** Strongly associated with **HLA-B51**. 4. **Vascular:** It is a leading cause of pulmonary artery aneurysms (Hughes-Stovin Syndrome). 5. **Treatment:** Colchicine for mucocutaneous symptoms; Azathioprine or Anti-TNF agents for ocular and CNS involvement.
Question 376: Which of the following is FALSE regarding antiphospholipid antibody syndrome (APLA)?
- A. >=2 unexplained consecutive spontaneous abortions in > 10 weeks of gestation (Correct Answer)
- B. >=3 unexplained consecutive spontaneous abortions in < 10 weeks of gestation
- C. >=1 unexplained death of a morphologically normal fetus >= 10 weeks of gestation
- D. >=1 premature birth of a morphologically normal neonate < 34 weeks of gestation
Explanation: The diagnosis of **Antiphospholipid Antibody Syndrome (APS/APLA)** follows the **Revised Sapporo Criteria (Sydney Criteria)**, which require at least one clinical criterion (vascular thrombosis or pregnancy morbidity) and one laboratory criterion (positive Lupus Anticoagulant, Anticardiolipin, or Anti-̢2-glycoprotein I antibodies). ### **Explanation of Options** * **Option A (Correct/False Statement):** This is the incorrect statement because the criteria specify **three or more** (not two) consecutive spontaneous abortions before the 10th week of gestation. Furthermore, spontaneous abortions occurring **after** 10 weeks are categorized differently (see Option C). * **Option B (True):** Three or more unexplained consecutive spontaneous abortions before the 10th week of gestation, with maternal anatomic/hormonal and paternal/maternal chromosomal causes excluded, is a valid clinical criterion. * **Option C (True):** One or more unexplained deaths of a morphologically normal fetus at or beyond the 10th week of gestation is a valid clinical criterion. * **Option D (True):** One or more premature births of a morphologically normal neonate before the 34th week of gestation due to eclampsia, severe pre-eclampsia, or placental insufficiency is a valid clinical criterion. ### **High-Yield Clinical Pearls for NEET-PG** * **Laboratory Criteria:** Antibodies must be present on two or more occasions, at least **12 weeks apart**. * **Paradoxical Finding:** APLA causes a **prolonged aPTT** (in vitro) despite being a **prothrombotic state** (in vivo). The aPTT does not correct with a 1:1 mixing study. * **False Positive VDRL:** Patients with APLA often show a false positive test for Syphilis due to cross-reactivity with cardiolipin. * **Treatment:** For obstetric APS, the standard of care is **Low Molecular Weight Heparin (LMWH) plus Low-Dose Aspirin**. Warfarin is contraindicated in pregnancy due to teratogenicity.
Question 377: Reactive arthritis is associated with which of the following pathogens?
- A. Chlamydia
- B. Yersinia
- C. Salmonella
- D. All of the above (Correct Answer)
Explanation: **Explanation:** **Reactive Arthritis (ReA)**, formerly known as Reiter’s Syndrome, is a sterile inflammatory synovitis that occurs following a distant infection, typically of the gastrointestinal (GI) or urogenital tract [1]. It is classified as a **Seronegative Spondyloarthropathy**. The correct answer is **D (All of the above)** because ReA is classically triggered by specific Gram-negative intracellular bacteria [2]: * **Chlamydia trachomatis:** The most common cause of urogenital (post-venereal) ReA [1]. * **Enteric Pathogens:** GI-related ReA (post-dysenteric) is commonly caused by **Salmonella**, **Shigella**, **Yersinia**, and **Campylobacter** [2]. **Why the other options are part of the whole:** Options A, B, and C are all classic triggers. Selecting only one would be incomplete, as ReA does not have a single causative agent but rather a spectrum of associated pathogens. **High-Yield Clinical Pearls for NEET-PG:** 1. **Genetic Association:** Strongly associated with **HLA-B27** (present in 30–50% of cases; higher in those with sacroiliitis) [1]. 2. **Clinical Triad:** "Can't see, can't pee, can't climb a tree" (Conjunctivitis/Uveitis, Urethritis, and Arthritis) [2]. 3. **Dermatological Findings:** Look for **Keratoderma blennorrhagicum** (vesicular/pustular lesions on palms/soles) and **Circinate balanitis** [2]. 4. **Pattern of Arthritis:** Typically an asymmetric oligoarthritis affecting the lower extremities (knees, ankles) [2]. 5. **Synovial Fluid:** Culture is characteristically **negative** (sterile), distinguishing it from septic arthritis.
Question 378: Which test evaluates lacrimal gland function for the diagnosis of Sjogren syndrome?
- A. Schirmer test
- B. Rose Bengal test
- C. Slit lamp examination
- D. All of the above (Correct Answer)
Explanation: Sjogren syndrome is a chronic autoimmune disorder [1] characterized by lymphocytic infiltration of exocrine glands, primarily leading to **keratoconjunctivitis sicca** (dry eyes) and xerostomia (dry mouth). Evaluating lacrimal gland dysfunction is a cornerstone of diagnosis. **Explanation of Options:** * **Schirmer test:** This is the most common bedside test. A filter paper strip is placed in the lower eyelid pouch. A result of **≤5 mm of wetting in 5 minutes** is indicative of deficient tear production (aqueous deficiency). * **Rose Bengal test:** This uses a specialized dye to identify areas of devitalized or dead corneal and conjunctival epithelial cells that lack a protective mucin layer. It specifically highlights the damage caused by chronic dryness. * **Slit lamp examination:** This allows for a detailed assessment of the tear film stability (Tear Break-Up Time or TBUT) and can identify corneal ulcerations or filamentary keratitis using **Fluorescein staining**. Since all three methods—measuring tear volume (Schirmer), identifying cell death (Rose Bengal), and visualizing structural damage (Slit lamp)—are used to evaluate ocular involvement in Sjogren syndrome, **Option D** is correct. **High-Yield Clinical Pearls for NEET-PG:** * **Antibodies:** Anti-Ro (SS-A) and Anti-La (SS-B) are the most specific markers [1]. * **Gold Standard Diagnosis:** Minor salivary gland biopsy (showing lymphocytic focus score ≥1). * **Malignancy Risk:** Patients have a 40-fold increased risk of developing **B-cell MALT Lymphoma**. * **Extraglandular manifestation:** The most common is arthralgia/arthritis; others include Raynaud’s phenomenon and cutaneous vasculitis [1].
Question 379: A man developed frequent facial swelling with occasional laryngeal edema. What is the most probable diagnosis?
- A. C1 esterase deficiency (Correct Answer)
- B. Allergy
- C. Anaphylaxis
- D. Not esterase deficiency
Explanation: The clinical presentation of recurrent facial swelling and life-threatening laryngeal edema without associated urticaria (hives) or pruritus is the classic hallmark of **Hereditary Angioedema (HAE)**, which is caused by **C1 esterase inhibitor deficiency**. [3] ### **Why C1 Esterase Deficiency is Correct** C1 esterase inhibitor (C1-INH) normally regulates the classical complement pathway and the kinin-generating cascade. A deficiency leads to the unregulated activation of kallikrein, resulting in excessive production of **bradykinin**. Bradykinin increases vascular permeability, leading to episodic, non-pitting edema of the face, extremities, and, most critically, the larynx. ### **Why Other Options are Incorrect** * **Allergy & Anaphylaxis:** These are Type I hypersensitivity reactions mediated by IgE and mast cell degranulation (histamine release). [1] They are almost always accompanied by **urticaria (wheals)**, intense itching, and hypotension. [3] The absence of "itchy rashes" in the history strongly points away from an allergic etiology. * **Not esterase deficiency:** This is a distractor that contradicts the clinical findings. ### **NEET-PG High-Yield Pearls** * **Inheritance:** Autosomal Dominant. * **Key Mediator:** Bradykinin (NOT histamine). * **Clinical Trigger:** Often precipitated by minor trauma, dental procedures, or stress. * **Diagnostic Screening:** Low **C4 levels** (best initial screening test, even between attacks). * **Management:** * *Acute attack:* Purified C1-INH concentrate or **Icatibant** (bradykinin B2 receptor antagonist). * *Prophylaxis:* **Danazol** (stanozolol) or Tranexamic acid. * **Note:** ACE inhibitors are contraindicated in these patients as they prevent bradykinin breakdown, worsening the edema. [2]
Question 380: Felty's syndrome is associated with which of the following?
- A. Osteoarthritis
- B. Rheumatoid arthritis (Correct Answer)
- C. Ankylosing spondylitis
- D. Psoriatic arthritis
Explanation: **Explanation:** **Felty’s Syndrome** is a rare but serious extra-articular manifestation of long-standing, seropositive **Rheumatoid Arthritis (RA)** [1]. It is classically defined by a clinical triad: 1. **Rheumatoid Arthritis** (usually severe, erosive, and chronic). 2. **Splenomegaly.** 3. **Neutropenia** (Absolute Neutrophil Count <2000/mm³). **Why Rheumatoid Arthritis is correct:** Felty’s syndrome typically occurs in patients who have had RA for over 10 years [1]. These patients often have high titers of Rheumatoid Factor (RF) and anti-CCP antibodies, along with extra-articular features like rheumatoid nodules and vasculitis. The pathophysiology involves autoimmune-mediated destruction of neutrophils and splenic sequestration. **Why the other options are incorrect:** * **Osteoarthritis:** This is a degenerative joint disease, not an autoimmune systemic inflammatory disorder, and is not associated with splenomegaly or cytopenias. * **Ankylosing Spondylitis:** This is a seronegative spondyloarthropathy primarily affecting the axial skeleton (HLA-B27 associated); it does not manifest as Felty’s syndrome. * **Psoriatic Arthritis:** While an inflammatory arthritis, it belongs to the seronegative group and is characterized by skin lesions and dactylitis rather than the Felty’s triad [2]. **High-Yield Clinical Pearls for NEET-PG:** * **HLA Association:** Strongly associated with **HLA-DR4**. * **Risk of Infection:** The primary clinical concern is recurrent bacterial infections (skin and respiratory) due to profound neutropenia. * **Large Granular Lymphocyte (LGL) Leukemia:** There is a significant phenotypic overlap between Felty’s syndrome and T-cell LGL leukemia; both present with RA and neutropenia. * **Treatment:** Management focuses on controlling the underlying RA, typically using **Methotrexate**. Granulocyte colony-stimulating factor (G-CSF) may be used in severe cases.
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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