Rheumatology and Immunology Practice Questions

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

Reiter's Syndrome. **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].

Q: Which condition is primarily associated with Sicca complex?

Primary Sjögren syndrome.. **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.

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

Temporal arteritis. ### 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.

Q: Which of the following is abdominal angiitis?

Takayasu arteritis. **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.

Q: Ankylosing spondylitis is associated with which of the following?

HLA-B27. **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].

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

Extraocular muscles. 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).

Q: All are features of sarcoidosis except:

Schaumann and asteroid bodies are pathognomic. **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 1

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

Accepted Answer

Reiter's Syndrome

Answer

Osteoarthritis

Answer

Sjogren syndrome

Answer

Behcet disease

Question 2

Which condition is primarily associated with Sicca complex?

Accepted Answer

Primary Sjögren syndrome.

Answer

Secondary Sjögren syndrome.

Answer

Benign lymphoepithelial lesion.

Answer

All of the above.

Question 3

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

Accepted Answer

Temporal arteritis

Answer

Nonspecific arteritis

Answer

Polyarteritis nodosa (PAN)

Answer

Wegener's granulomatosis

Question 4

Which of the following is abdominal angiitis?

Accepted Answer

Takayasu arteritis

Answer

Giant cell arteritis

Answer

Kawasaki disease

Answer

Polyarteritis nodosa

Question 5

Ankylosing spondylitis is associated with which of the following?

Accepted Answer

HLA-B27

Answer

HLA-B8

Answer

HLA-DW4/DR4

Answer

HLA-DR3

Question 6

Involvement of which of the following muscles virtually excludes the diagnosis of polymyositis?

Accepted Answer

Extraocular muscles

Answer

Neck muscles

Answer

Esophageal muscles

Answer

Abdominal muscle

Question 7

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?

Accepted Answer

Subcutaneous nodules

Answer

Chronic arthritis

Answer

Involvement of spinal joints

Answer

Erythema nodosum

Question 8

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?

Accepted Answer

Continue the same dose of steroids

Answer

Raise the dose of steroids

Answer

Start mycophenolate

Answer

Perform muscle biopsy

Question 9

All are features of sarcoidosis except:

Accepted Answer

Schaumann and asteroid bodies are pathognomic

Answer

High CD4:CD8 ratio

Answer

Hypercalciuria and hypercalcemia may be present

Answer

Elevated serum levels of angiotensin-converting enzyme (ACE)

Question 10

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

Accepted Answer

Pain or stiffness in the neck, shoulders, or back for more than six months in a year

Answer

Morning stiffness lasting for one hour or more

Answer

Erythrocyte Sedimentation Rate (ESR) greater than 40 mm/hr

Answer

Rapid and complete response to a low dose of prednisolone (less than 20 mg/day)

Rheumatology and Immunology — MCQs

Rheumatology and Immunology — MCQs

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