Rheumatology and Immunology Practice Questions

Q: Which of the following is NOT seen in Behçet's syndrome?

Pyoderma gangrenosum. Explanation: Behçet’s syndrome is a chronic, multisystemic, relapsing inflammatory perivasculitis. The hallmark of the disease is the presence of painful **oral and genital ulcers** along with ocular and cutaneous involvement. **Why Pyoderma Gangrenosum (D) is the correct answer:** While Behçet’s syndrome presents with various skin lesions, **Pyoderma Gangrenosum (PG)** is classically associated with **Inflammatory Bowel Disease (IBD)**, particularly Ulcerative Colitis, and certain hematologic malignancies. In Behçet’s, the characteristic skin findings are **Erythema Nodosum**, pseudofolliculitis, and acneiform eruptions. Although Behçet’s patients exhibit **Pathergy** (an exaggerated skin injury response), the specific deep, necrotic ulceration of PG is not a diagnostic or common feature of the syndrome. **Analysis of Incorrect Options:** * **A & C (Genital and Oral Ulcers):** These are the most common clinical features. Oral aphthous ulcers are usually the presenting symptom (99% of cases). Genital ulcers are similar in appearance but do not occur as frequently as oral ulcers; however, they are more specific to Behçet’s. * **B (Uveitis):** Ocular involvement occurs in about 70% of patients. **Panuveitis** or posterior uveitis is a serious complication that can lead to blindness and is a key component of the clinical spectrum. **High-Yield Clinical Pearls for NEET-PG:** * **Pathergy Test:** Development of a sterile pustule 24–48 hours after a skin prick with a 20-gauge needle. It is highly specific for Behçet’s. * **HLA Association:** Strongly associated with **HLA-B51**. * **Vascular Involvement:** Unlike other vasculitides, Behçet’s can involve **both arteries and veins** of all sizes (e.g., Budd-Chiari syndrome, pulmonary artery aneurysms). * **Magic Syndrome:** A variant involving features of both Behçet’s and Relapsing Polychondritis (Mouth And Genital ulcers with Inflamed Cartilage).

Q: Under the Jones criteria, which one of the following is a major criterion for the diagnosis of acute rheumatic fever?

Carditis. **Explanation:** Acute Rheumatic Fever (ARF) is an autoimmune multi-systemic response following a Group A Streptococcal (GAS) pharyngeal infection. Diagnosis is based on the **Revised Jones Criteria (2015)**, which categorizes findings into Major and Minor criteria based on the patient's risk population (Low vs. Moderate/High risk). **Why Carditis is correct:** **Carditis** is a **Major criterion** across all risk groups. It typically presents as a pancarditis (endocarditis, myocarditis, and pericarditis). In the revised criteria, carditis can be either **clinical** (murmurs of mitral or aortic regurgitation) or **subclinical** (detected via echocardiographic evidence of valvulitis), even if a murmur is absent. **Analysis of Incorrect Options:** * **A. Elevated acute-phase reactants (ESR/CRP):** These are **Minor criteria**. They indicate systemic inflammation but are non-specific to ARF. * **B. Elevated ASO titer:** This is not a Jones criterion itself; rather, it is the **required evidence of a preceding GAS infection**. Diagnosis of ARF requires meeting Jones criteria *plus* evidence of infection (except in cases of chorea or indolent carditis). * **C. Increased PR interval:** This is a **Minor criterion**. It represents a first-degree AV block and must be interpreted carefully if carditis is already used as a major criterion. **High-Yield Clinical Pearls for NEET-PG:** 1. **Mnemonic for Major Criteria (JONES):** **J**oints (Migratory Polyarthritis), **O** (Carditis - looks like a heart), **N**odules (Subcutaneous), **E**rythema marginatum, **S**ydenham chorea. 2. **Joints:** In high-risk populations, *Monoarthritis* or *Polyarthralgia* can now be considered Major criteria. 3. **Most common valve affected:** Mitral valve (Mitral Regurgitation is the most common early finding). 4. **Pathognomonic finding:** **Aschoff bodies** (granulomas with Anitschkow "caterpillar" cells) seen on myocardial histology.

Q: Libman-Sachs endocarditis is classically found in which condition?

Systemic lupus erythematosus. **Explanation:** **Libman-Sachs Endocarditis (LSE)**, also known as verrucous or non-bacterial thrombotic endocarditis (NBTE), is a classic cardiac manifestation of **Systemic Lupus Erythematosus (SLE)**. 1. **Why SLE is correct:** LSE is characterized by small, sterile, fibro-fibrinous vegetations. Unlike infective endocarditis, these vegetations can occur on **both sides** (superior and inferior surfaces) of the valve leaflets and the chordae tendineae. They are most commonly found on the **mitral valve**. The underlying mechanism involves immune complex deposition and subsequent chronic inflammation, leading to tissue repair and scarring. 2. **Why other options are incorrect:** * **Rheumatoid Arthritis:** Cardiac involvement typically manifests as pericarditis or rheumatoid nodules in the myocardium/valves, but not LSE. * **Syphilis:** Classically associated with **syphilitic aortitis** (involving the ascending aorta) and aortic regurgitation due to "tree-barking" of the intima, not sterile valvular vegetations. * **Systemic Sclerosis:** Primarily affects the myocardium (focal patches of fibrosis) and can cause pulmonary hypertension, but is not typically associated with endocardial vegetations. **High-Yield Clinical Pearls for NEET-PG:** * **Location:** Mitral valve is most common, followed by the aortic valve. * **Pathology:** Vegetations are **sterile** (no organisms) and consist of fibrin, inflammatory cells, and hematoxylin bodies (characteristic of SLE). * **Association:** Strongly associated with **Antiphospholipid Antibody Syndrome (APS)**. * **Complication:** While often asymptomatic, LSE can lead to valvular regurgitation or systemic embolization (stroke).

Q: Anti U1-RNP antibody is seen in which condition?

Mixed Connective Tissue Disease (MCTD). ### Explanation **Correct Answer: C. Mixed Connective Tissue Disease (MCTD)** **1. Why MCTD is the correct answer:** Mixed Connective Tissue Disease (MCTD) is a distinct overlap syndrome characterized by clinical features of SLE, Systemic Sclerosis, and Polymyositis [2]. The **hallmark serological marker** for MCTD is the presence of high titers of **Anti-U1 ribonucleoprotein (U1-RNP) antibodies** [1], [2]. In fact, according to the Kasukawa or Alarcón-Segovia diagnostic criteria, a high titer of Anti-U1 RNP is a **mandatory requirement** for the diagnosis of MCTD. **2. Why other options are incorrect:** * **A. SLE:** While Anti-U1 RNP can be seen in about 30-40% of SLE patients, it is not specific. The highly specific markers for SLE are **Anti-dsDNA** and **Anti-Smith (Sm)** antibodies [1], [3]. * **B. Scleroderma:** The characteristic antibodies are **Anti-Scl-70 (Anti-topoisomerase I)** for diffuse cutaneous systemic sclerosis and **Anti-centromere** for limited cutaneous systemic sclerosis (CREST syndrome). * **C. Dermatomyositis:** The most specific marker is **Anti-Mi-2** antibody. Other myositis-specific antibodies include **Anti-Jo-1** (associated with Antisynthetase syndrome). **3. High-Yield Clinical Pearls for NEET-PG:** * **MCTD Clinical Triad:** Raynaud’s phenomenon, "Puffy hands" (swollen fingers), and Myositis/Arthralgia [2]. * **Prognosis:** The most common cause of death in MCTD is **Pulmonary Hypertension** [2]. * **Serology Tip:** If a question mentions "Anti-RNP" without the "U1" prefix, it still refers to MCTD. However, if **Anti-Smith** is present, the diagnosis shifts toward SLE, as Anti-Smith is rarely positive in pure MCTD [1]. * **ANA Pattern:** Anti-U1 RNP typically produces a **speckled pattern** on Immunofluorescence.

Q: What crystals are aspirated from the bursa of an elbow in a patient with rheumatoid arthritis?

Cholesterol. ### Explanation **Correct Option: A. Cholesterol** In patients with chronic inflammatory conditions like **Rheumatoid Arthritis (RA)**, cholesterol crystals can occasionally be found in the synovial fluid or bursal aspirates (especially the olecranon bursa) [1]. These crystals form due to the chronic breakdown of cell membranes and local tissue destruction within the joint or bursa, leading to an accumulation of lipids that crystallize over time. Under polarized light, they typically appear as **large, notched, square or rectangular plates** with "broken corners." **Why other options are incorrect:** * **B. Calcium apatite:** These are associated with calcific tendonitis or Milwaukee shoulder. They are extremely small and usually require electron microscopy or Alizarin Red staining for visualization. * **C. Calcium oxalate:** These are typically seen in the joints of patients with **End-Stage Renal Disease (ESRD)** undergoing long-term hemodialysis [1]. They appear as envelope-shaped crystals. * **D. Calcium pyrophosphate dihydrate (CPPD):** These are the hallmark of **Pseudogout** [1]. They are rhomboid-shaped and show weak positive birefringence. While RA patients can have co-existing CPPD, cholesterol crystals are the classic association for chronic rheumatoid bursal effusions. **High-Yield Clinical Pearls for NEET-PG:** * **Cholesterol Crystals:** Associated with "chronic" effusions (RA, Osteoarthritis) [1]. They have a characteristic **"notched corner"** appearance. * **Monosodium Urate (MSU):** Needle-shaped, strongly negative birefringent (Gout) [1]. * **CPPD:** Rhomboid-shaped, weakly positive birefringent (Pseudogout). * **Rheumatoid Factor (RF):** Though common in RA, it is not specific; however, high titers often correlate with extra-articular manifestations like rheumatoid nodules and bursitis.

Q: Primary idiopathic polymyositis does not involve which of the following muscle groups?

Ocular muscles. Primary idiopathic polymyositis (PM) is an inflammatory myopathy characterized by symmetric, subacute, and progressive weakness of the **proximal skeletal muscles** [1]. **Why Ocular Muscles are the Correct Answer:** A hallmark diagnostic feature of Polymyositis (and Dermatomyositis) is the **sparing of the ocular and facial muscles**. If a patient presents with proximal muscle weakness along with ptosis or diplopia (extraocular muscle involvement), clinicians should instead suspect conditions like **Myasthenia Gravis** or **Mitochondrial Myopathies** (e.g., CPEO). **Analysis of Incorrect Options:** * **A. Pelvic girdle muscles:** These are the classic site of involvement. Patients typically complain of difficulty rising from a chair or climbing stairs [1]. * **B. Neck muscles:** Weakness of the neck flexors is common, often leading to "dropped head syndrome" or difficulty lifting the head from a pillow. * **C. Pharyngeal muscles:** Involvement of the striated muscles of the upper esophagus and pharynx is common in advanced or severe cases, leading to dysphagia and an increased risk of aspiration pneumonia [1]. **High-Yield Clinical Pearls for NEET-PG:** * **Diagnostic Marker:** Elevated Serum Creatine Kinase (CK) is the most sensitive enzyme marker. * **Autoantibodies:** **Anti-Jo-1** (Histidyl-tRNA synthetase) is the most common myositis-specific antibody and is associated with "Antisynthetase Syndrome" (interstitial lung disease, Raynaud’s, and mechanic's hands) [1]. * **Gold Standard Diagnosis:** Muscle biopsy showing endomysial inflammation with CD8+ T-cell infiltration. * **Malignancy:** Unlike Dermatomyositis, Polymyositis has a lower (though still present) association with underlying occult malignancies.

Question 1

Which of the following statements are true about Dermatomyositis?

Accepted Answer

All cases are associated with internal malignancy

Answer

Gottron sign

Answer

ANA is positive in all cases

Answer

Proximal muscle wasting

Question 2

A 48-year-old man presents with macules, papules, ulcers, and eschars on his extremities, hands, genitals, and face, as well as nasal and oral mucosae. The rash is accompanied by severe malaise, fever, arthralgia, paresthesia, and microhematuria. Cryoglobulins and high titers of circulating immune complexes were noted. Steroid treatment and plasmapheresis were instituted with good results. This clinical presentation is most likely secondary to which of the following conditions?

Accepted Answer

Vasculitic ulcer

Answer

Aerial ulcer

Answer

Venous ulcer

Answer

Neurotrophic ulcer

Question 3

Which of the following is NOT seen in Behçet's syndrome?

Accepted Answer

Pyoderma gangrenosum

Answer

Genital ulcers

Answer

Uveitis

Answer

Oral ulcers

Question 4

Tophi in gout are found in all regions except which of the following?

Accepted Answer

Joint capsule

Answer

Skin

Answer

Muscle

Answer

Articular cartilage

Question 5

Under the Jones criteria, which one of the following is a major criterion for the diagnosis of acute rheumatic fever?

Accepted Answer

Carditis

Answer

Elevated acute-phase reactants

Answer

Elevated ASO titer

Answer

Increased PR interval

Question 6

Libman-Sachs endocarditis is classically found in which condition?

Accepted Answer

Systemic lupus erythematosus

Answer

Rheumatoid arthritis

Answer

Syphilis

Answer

Systemic sclerosis

Question 7

Gold salts can be used in which of the following conditions?

Accepted Answer

Rheumatoid arthritis

Answer

Ankylosing spondylitis

Answer

Osteoarthritis

Answer

Behcet's syndrome

Question 8

Anti U1-RNP antibody is seen in which condition?

Accepted Answer

Mixed Connective Tissue Disease (MCTD)

Answer

Systemic Lupus Erythematosus (SLE)

Answer

Scleroderma

Answer

Dermatomyositis

Question 9

What crystals are aspirated from the bursa of an elbow in a patient with rheumatoid arthritis?

Accepted Answer

Cholesterol

Answer

Calcium apatite

Answer

Calcium oxalate

Answer

Calcium pyrophosphate dihydrate

Question 10

Primary idiopathic polymyositis does not involve which of the following muscle groups?

Accepted Answer

Ocular muscles

Answer

Pelvic girdle muscles

Answer

Neck muscles

Answer

Pharyngeal muscle

Rheumatology and Immunology — MCQs

Rheumatology and Immunology — MCQs

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