Rheumatology and Immunology — MCQs

Rheumatology and Immunology Indian Medical PG Practice Questions and MCQs

Question 231: Which of the following conditions is associated with HLA-B27?

A. Osteoarthritis
B. Sjogren syndrome
C. Behcet disease
D. Reiter's Syndrome (Correct Answer)

Explanation: **Explanation:** **Reiter’s Syndrome (Reactive Arthritis)** is the correct answer because it belongs to the group of **Seronegative Spondyloarthropathies (SpA)**. These conditions are characterized by the absence of Rheumatoid Factor (RF) and a strong genetic association with the **HLA-B27** allele [1]. Reiter’s Syndrome typically presents as a triad of urethritis, conjunctivitis, and arthritis ("Can't see, can't pee, can't climb a tree") following a gastrointestinal or urogenital infection [1][2]. **Analysis of Incorrect Options:** * **A. Osteoarthritis:** This is a degenerative joint disease caused by "wear and tear" of articular cartilage. It is not an immune-mediated inflammatory condition and has no association with HLA-B27. * **B. Sjogren Syndrome:** This is an autoimmune destruction of exocrine glands [1]. It is primarily associated with **HLA-DR3** and **HLA-DQ2**, as well as antibodies like Anti-Ro (SS-A) and Anti-La (SS-B). * **C. Behcet Disease:** While this is a multi-system inflammatory vasculitis, its strongest genetic association is with **HLA-B51**, not HLA-B27. **High-Yield Clinical Pearls for NEET-PG:** * **Mnemonic for HLA-B27 (PEAR):** **P**soriatic arthritis, **E**nteropathic arthritis (IBD), **A**nkylosing spondylitis (strongest association >90%), and **R**eactive arthritis [1]. * **Reactive Arthritis** is most commonly triggered by *Chlamydia trachomatis* (urogenital) or *Shigella, Salmonella, Campylobacter* (enteric) [1][2]. * **Extra-articular features** of HLA-B27 conditions often include **Acute Anterior Uveitis** and **Aortitis** [1]. * **Keratoderma blennorrhagica** (skin lesions on palms/soles) is a pathognomonic finding in Reiter’s Syndrome [2].

Question 232: Which condition is primarily associated with Sicca complex?

A. Primary Sjögren syndrome. (Correct Answer)
B. Secondary Sjögren syndrome.
C. Benign lymphoepithelial lesion.
D. All of the above.

Explanation: **Explanation:** **Sicca complex** refers to the combination of dry eyes (**xerophthalmia**) and dry mouth (**xerostomia**) resulting from lymphocytic infiltration of the lacrimal and salivary glands. 1. **Why Option A is correct:** **Primary Sjögren syndrome (pSS)** is a systemic autoimmune disease characterized by the isolated presence of the Sicca complex without an associated connective tissue disease. It is the condition most "primarily" and classically defined by these symptoms. In pSS, the exocrine gland dysfunction is the central feature of the pathology. 2. **Why other options are incorrect:** * **Secondary Sjögren syndrome:** While this also involves Sicca symptoms, it occurs in the presence of another established autoimmune disease (most commonly Rheumatoid Arthritis, SLE, or Scleroderma) [1]. The term "Sicca complex" is more fundamentally linked to the primary form in clinical nomenclature. * **Benign lymphoepithelial lesion (Mikulicz disease):** This refers to the histological finding of glandular enlargement (usually parotid) with lymphocytic infiltration. * **All of the above:** While all involve glandular infiltration, "Sicca complex" is the hallmark clinical definition specifically used to identify Primary Sjögren syndrome. **NEET-PG High-Yield Pearls:** * **Schirmer’s Test:** Used to quantify tear production (Positive if <5 mm in 5 minutes). * **Autoantibodies:** Anti-Ro (SS-A) and Anti-La (SS-B) are highly specific markers [1]. * **Gold Standard Diagnosis:** Minor salivary gland biopsy showing focal lymphocytic sialadenitis (Focus score ≥1). * **Malignancy Risk:** Patients with Sjögren syndrome have a 40-fold increased risk of developing **B-cell MALT Lymphoma**. * **Extraglandular manifestation:** The most common is arthralgia/arthritis; the most serious is vasculitis.

Question 233: An elderly female presents with fever, headache, and diplopia. Biopsy of the artery revealed panarteritis. What is the most probable diagnosis?

A. Nonspecific arteritis
B. Polyarteritis nodosa (PAN)
C. Wegener's granulomatosis
D. Temporal arteritis (Correct Answer)

Explanation: ### Explanation **Correct Answer: D. Temporal arteritis (Giant Cell Arteritis)** **Why it is correct:** Temporal arteritis (GCA) is a large-vessel vasculitis that characteristically affects elderly individuals (usually >50 years) [1]. The classic triad includes **fever, headache, and visual disturbances** (like diplopia or amaurosis fugax). The pathology reveals **panarteritis**—inflammation involving all layers of the arterial wall—often with giant cell formation and internal elastic lamina fragmentation [2]. Diplopia in GCA is a medical emergency, as it signifies ischemia to the extraocular muscles or cranial nerves, risking permanent blindness. **Why the other options are incorrect:** * **A. Nonspecific arteritis:** This is a vague term and not a recognized clinical entity for this specific presentation. * **B. Polyarteritis nodosa (PAN):** This is a medium-vessel vasculitis that typically spares the lungs and rarely presents with isolated headache/diplopia. It is strongly associated with Hepatitis B and presents with "rosary sign" (aneurysms) on angiography [1]. * **C. Wegener's granulomatosis (GPA):** This small-vessel vasculitis involves the triad of upper respiratory tract (sinusitis), lower respiratory tract (hemoptysis), and kidneys (GN). It is c-ANCA positive. **High-Yield Clinical Pearls for NEET-PG:** * **Gold Standard Diagnosis:** Temporal artery biopsy (look for "skip lesions") [2]. * **Lab Marker:** Markedly elevated **ESR** (often >100 mm/hr) and CRP [2]. * **Associated Condition:** Strongly linked with **Polymyalgia Rheumatica (PMR)**, characterized by proximal muscle stiffness. * **Treatment:** Immediate high-dose **Corticosteroids** (do not wait for biopsy results if vision is threatened) to prevent permanent vision loss [2]. * **Most common vessel involved:** Superficial temporal artery.

Question 234: Which of the following is abdominal angiitis?

A. Giant cell arteritis
B. Takayasu arteritis (Correct Answer)
C. Kawasaki disease
D. Polyarteritis nodosa

Explanation: **Explanation:** **Takayasu Arteritis (Correct Answer):** Takayasu arteritis is a chronic, large-vessel vasculitis that primarily involves the aorta and its major branches. It is often referred to as "abdominal angiitis" or "aortic arch syndrome" because of its predilection for the abdominal aorta and renal arteries. In approximately 50% of cases, the abdominal aorta is involved, leading to symptoms like mesenteric ischemia (abdominal angina) and renovascular hypertension. It is most commonly seen in young females (under 40 years). **Incorrect Options:** * **Giant Cell Arteritis (GCA):** While also a large-vessel vasculitis, it primarily affects the extracranial branches of the carotid artery (e.g., temporal artery). It typically occurs in patients over 50 and is associated with Polymyalgia Rheumatica. * **Kawasaki Disease:** This is a medium-vessel vasculitis seen in children. Its hallmark is the involvement of **coronary arteries** (aneurysms), not the abdominal aorta. * **Polyarteritis Nodosa (PAN):** This is a medium-vessel vasculitis that affects the renal and visceral arteries. While it causes "microaneurysms" in the mesenteric vessels (rosary sign), the specific term "abdominal angiitis" is classically associated with the large-vessel involvement seen in Takayasu. **High-Yield Clinical Pearls for NEET-PG:** * **Classification:** Takayasu is Type IV when it involves the abdominal aorta and/or renal arteries. * **Clinical Sign:** "Pulseless disease" – absent or weak peripheral pulses with a blood pressure discrepancy between arms (>10 mmHg). * **Diagnosis:** Gold standard is **CT/MR Angiography** showing "skip lesions" or tapered narrowing of the aorta. * **Bruit:** Presence of an abdominal or carotid bruit in a young female is a classic exam trigger for Takayasu.

Question 235: Ankylosing spondylitis is associated with which of the following?

A. HLA-B27 (Correct Answer)
B. HLA-B8
C. HLA-DW4/DR4
D. HLA-DR3

Explanation: **Explanation:** **Ankylosing Spondylitis (AS)** is the prototype of **Seronegative Spondyloarthritides (SpA)** [1]. The strongest genetic association in rheumatology exists between AS and **HLA-B27** (a Class I MHC molecule). 1. **Why HLA-B27 is Correct:** Approximately **90-95%** of patients with AS carry the HLA-B27 allele [1]. While the exact pathogenesis is debated, theories like the "Misfolded Protein Response" and "Arthritogenic Peptide Hypothesis" suggest that HLA-B27 leads to aberrant immune activation and chronic inflammation of the sacroiliac joints and spine. 2. **Analysis of Incorrect Options:** * **HLA-B8:** Associated with Myasthenia Gravis and Graves' disease. * **HLA-DR4 (DW4):** This is the primary genetic association for **Rheumatoid Arthritis (RA)**. Remember: RA is "Seropositive," whereas AS is "Seronegative." * **HLA-DR3:** Associated with Systemic Lupus Erythematosus (SLE), Dermatitis Herpetiformis, and Type 1 Diabetes Mellitus. **High-Yield Clinical Pearls for NEET-PG:** * **The "M" Rule:** HLA-B27 is also associated with other SpAs: **M**ultiple (Psoriatic arthritis), **M**alignant (Reactive arthritis/Reiter’s), and **M**edical (IBD-associated arthritis) [1]. * **Radiology:** Look for "Bamboo Spine" (syndesmophytes) and "Dagger Sign" on X-ray [1]. * **Clinical Sign:** Positive **Schober’s Test** (measures restricted lumbar flexion). * **Extra-articular manifestation:** The most common is **Acute Anterior Uveitis** (unilateral) [1]. * **Treatment:** NSAIDs are first-line; TNF-alpha inhibitors (e.g., Etanercept, Infliximab) are used for refractory cases [1].

Question 236: Involvement of which of the following muscles virtually excludes the diagnosis of polymyositis?

A. Neck muscles
B. Extraocular muscles (Correct Answer)
C. Esophageal muscles
D. Abdominal muscle

Explanation: The hallmark of **Polymyositis (PM)** and **Dermatomyositis (DM)** is symmetric, subacute, progressive weakness of the **proximal limb muscles** (shoulders and hips) [1]. A crucial diagnostic feature of these inflammatory myopathies is the **sparing of the extraocular and facial muscles.** **Why Extraocular Muscles are the Correct Answer:** Polymyositis specifically targets skeletal muscles of the trunk and limbs but characteristically spares the extraocular muscles. If a patient presents with proximal muscle weakness accompanied by ptosis or diplopia (extraocular involvement), clinicians must look for alternative diagnoses such as **Myasthenia Gravis** or **Oculopharyngeal Muscular Dystrophy** [2]. **Analysis of Incorrect Options:** * **Neck Muscles:** Weakness of the neck flexors is very common in PM, often leading to "dropped head syndrome" or difficulty lifting the head from a pillow. * **Esophageal Muscles:** PM frequently involves the striated muscles of the upper third of the esophagus and the pharynx [1]. This leads to **dysphagia** and an increased risk of aspiration pneumonia. * **Abdominal Muscles:** As part of the core/trunk musculature, the abdominal muscles can be involved, contributing to difficulty rising from a supine position [1]. **NEET-PG High-Yield Pearls:** * **Sparing Rule:** PM/DM spares extraocular muscles (unlike Myasthenia Gravis) and distal muscles (unlike Inclusion Body Myositis). * **Enzymes:** Elevated **Creatine Kinase (CK)** is the most sensitive initial lab marker. * **Antibodies:** **Anti-Jo-1** (Histidyl-tRNA synthetase) is the most common myositis-specific antibody and is associated with "Mechanic’s hands," interstitial lung disease, and Raynaud's phenomenon (Antisynthetase Syndrome) [1]. * **Malignancy:** Dermatomyositis (more than PM) has a strong association with underlying occult malignancies (e.g., ovarian, lung, breast).

Question 237: Following a severe sore throat, a 15-year-old boy feels unwell. He has had pain and swelling in his elbows and knees, and cardiac examination reveals new mitral regurgitation and tachycardia. Which of the following might also be seen on further examination?

A. Chronic arthritis
B. Involvement of spinal joints
C. Subcutaneous nodules (Correct Answer)
D. Erythema nodosum

Explanation: This clinical scenario describes a classic presentation of **Acute Rheumatic Fever (ARF)**, a non-suppurative sequela of Group A Streptococcal (GAS) pharyngitis. The diagnosis is based on the **Jones Criteria**, which require evidence of a preceding GAS infection plus two major criteria (or one major and two minor) [1]. ### Why the Correct Answer is Right **Subcutaneous nodules** are one of the five **Major Jones Criteria**. They are firm, painless, mobile nodules typically found over bony prominences or extensor tendons (like the elbows, wrists, or knees). They usually appear weeks after the onset of ARF and are strongly associated with severe carditis, which this patient exhibits (new mitral regurgitation and tachycardia) [1]. ### Why the Other Options are Wrong * **A & B: Chronic arthritis / Spinal involvement:** The arthritis in ARF is characteristically a **migratory polyarthritis** that affects large joints (knees, ankles, elbows). It is exquisitely responsive to NSAIDs and, crucially, **never causes chronic joint damage** or spinal involvement (unlike Rheumatoid Arthritis or Ankylosing Spondylitis). * **D: Erythema nodosum:** While this is a skin manifestation of various inflammatory conditions (including post-streptococcal states), it is **not** a Jones criterion for ARF. The specific dermatological major criterion for ARF is **Erythema marginatum** (evanescent, pink, non-pruritic rings). ### NEET-PG High-Yield Pearls * **Jones Criteria (Major):** **J**oints (Migratory Polyarthritis), **O** (Carditis - "O" looks like a heart), **N**odules (Subcutaneous), **E**rythema marginatum, **S**ydenham chorea [1]. * **Most common valve affected:** Mitral valve (Regurgitation in acute phase; Stenosis in chronic phase) [1]. * **Pathognomonic Histology:** **Aschoff bodies** (granulomatous lesions) containing **Anitschkow cells** ("caterpillar cells"). * **Treatment:** Penicillin (for GAS eradication) + Aspirin (for arthritis) + Steroids (if severe carditis) [1].

Question 238: A patient with dermatomyositis shows improvement of symptoms on steroids for the last 6 months. Labs today show a Creatine kinase of 1300 Units/L. What is the next step in the management?

A. Continue the same dose of steroids (Correct Answer)
B. Raise the dose of steroids
C. Start mycophenolate
D. Perform muscle biopsy

Explanation: ### Explanation The management of inflammatory myopathies like dermatomyositis relies on monitoring **clinical symptoms** (muscle strength and functional status) rather than just laboratory markers [1]. **1. Why Option A is Correct:** In this patient, the clinical status is improving ("improvement of symptoms"). In dermatomyositis, **serum Creatine Kinase (CK) levels do not always correlate with disease activity.** [1] CK levels can remain elevated for weeks or months even after clinical remission has begun, or they may fluctuate due to physical activity. Since the patient is clinically responding to the current steroid regimen, there is no indication to escalate therapy. The goal of treatment is functional recovery, not "normalizing the lab report." **2. Why the other options are incorrect:** * **Option B:** Raising the steroid dose is unnecessary and increases the risk of side effects (e.g., steroid-induced myopathy, osteoporosis). Dose escalation is only indicated if there is clinical worsening or a plateau in strength improvement. * **Option C:** Mycophenolate (or Azathioprine/Methotrexate) is a steroid-sparing agent. It is indicated if the patient is steroid-resistant, has frequent relapses, or to reduce long-term steroid toxicity. * **Option D:** Muscle biopsy is a diagnostic tool used at the initial presentation [1]. It has no role in monitoring the treatment response of a patient already diagnosed and improving. **Clinical Pearls for NEET-PG:** * **Most sensitive enzyme** for monitoring myositis: Creatine Kinase (CK) [1]. * **Most specific enzyme** for muscle: CK-MM. * **Steroid-induced myopathy:** A common confusion in exams. If a patient on steroids develops *new* weakness despite *normal* CK levels, suspect steroid-induced myopathy. If weakness worsens and CK is *rising*, suspect a disease flare. * **Dermatomyositis Association:** Always screen for underlying **malignancy** (Ovarian, Lung, Gastric) in elderly patients with dermatomyositis [1].

Question 239: All are features of sarcoidosis except:

A. High CD4:CD8 ratio
B. Hypercalciuria and hypercalcemia may be present
C. Elevated serum levels of angiotensin-converting enzyme (ACE)
D. Schaumann and asteroid bodies are pathognomic (Correct Answer)

Explanation: **Explanation:** **Why Option D is the correct answer:** While **Schaumann bodies** (laminated calcium-protein concretions) and **asteroid bodies** (stellate inclusions within giant cells) are classic histological findings in sarcoidosis, they are **not pathognomonic**. These inclusions can also be seen in other granulomatous diseases, such as tuberculosis, berylliosis, and foreign body granulomas. The diagnosis of sarcoidosis remains one of exclusion, requiring a compatible clinical/radiological picture and histological evidence of non-caseating granulomas. **Analysis of Incorrect Options:** * **Option A:** Sarcoidosis is characterized by an exaggerated T-helper cell response. Bronchoalveolar lavage (BAL) typically shows a **high CD4:CD8 ratio** (often >3.5:1), which is a high-yield diagnostic marker. * **Option B:** Macrophages within the sarcoid granulomas possess **1-alpha-hydroxylase** activity, which converts Vitamin D to its active form (1,25-dihydroxyvitamin D). This leads to increased intestinal calcium absorption, resulting in **hypercalciuria** (more common) and **hypercalcemia** [1]. * **Option C:** **ACE levels** are elevated in approximately 60-80% of patients with active sarcoidosis, as ACE is produced by the epithelioid cells of the granuloma. While useful for monitoring disease activity, it lacks specificity. **NEET-PG Clinical Pearls:** * **Lofgren’s Syndrome:** A classic triad of Erythema nodosum, bilateral hilar lymphadenopathy (BHL), and arthralgia (excellent prognosis) [1]. * **Heerfordt’s Syndrome (Uveoparotid fever):** Parotid enlargement, facial nerve palsy, and anterior uveitis [1]. * **Radiology:** Stage I is characterized by Bilateral Hilar Lymphadenopathy (BHL) alone [1]. * **Histology:** The hallmark is the **non-caseating granuloma**.

Question 240: Which of the following is NOT included in the diagnostic criteria for Polymyalgia Rheumatica?

A. Morning stiffness lasting for one hour or more
B. Pain or stiffness in the neck, shoulders, or back for more than six months in a year (Correct Answer)
C. Erythrocyte Sedimentation Rate (ESR) greater than 40 mm/hr
D. Rapid and complete response to a low dose of prednisolone (less than 20 mg/day)

Explanation: Polymyalgia Rheumatica (PMR) is a clinical diagnosis characterized by inflammatory pain and stiffness in the proximal joints. The correct answer is **B** because the duration requirement in the diagnostic criteria (such as the Bird criteria or the 2012 ACR/EULAR criteria) is typically **at least 2 weeks**, not six months. PMR is an acute to subacute condition; requiring symptoms for six months would delay necessary treatment and is not part of any standard diagnostic framework. **Analysis of Options:** * **Option A:** Morning stiffness lasting **>45–60 minutes** is a hallmark feature of PMR, reflecting the underlying systemic inflammatory nature of the disease. * **Option C:** An elevated inflammatory marker is essential. An **ESR >40 mm/hr** (or elevated CRP) is a classic diagnostic criterion, though it is not 100% specific. * **Option D:** A "dramatic" response to **low-dose corticosteroids** (10–20 mg/day of Prednisolone) within 48–72 hours is so characteristic of PMR that it is often used as a retrospective diagnostic tool. **High-Yield Clinical Pearls for NEET-PG:** * **Demographics:** Almost exclusively affects patients **>50 years** of age. * **Association:** Strongly associated with **Giant Cell Arteritis (GCA)**; approximately 15-20% of PMR patients develop GCA, and 50% of GCA patients have PMR. * **Key Negative:** Despite severe pain, there is usually **no true muscle weakness** (unlike polymyositis) and **normal Creatine Kinase (CK)** levels. * **Imaging:** Ultrasound may show bilateral subacromial bursitis or tenosynovitis of the long head of the biceps.

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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