Rheumatology and Immunology Practice Questions

Q: Abatacept is used in the treatment of which of the following conditions?

Rheumatoid Arthritis. **Explanation:** **Abatacept** is a biological disease-modifying antirheumatic drug (bDMARD) used primarily in the treatment of **Rheumatoid Arthritis (RA)**, particularly in patients who have had an inadequate response to methotrexate or TNF inhibitors [1]. **Mechanism of Action:** Abatacept is a soluble fusion protein consisting of the extracellular domain of human **CTLA-4** linked to a modified Fc portion of human IgG1. It acts as a **T-cell costimulation modulator**. To activate a T-cell, two signals are required: 1. The TCR binding to the MHC-peptide complex. 2. The **CD28** on the T-cell binding to **CD80/86** on the Antigen Presenting Cell (APC). Abatacept binds to CD80/86 with high affinity, preventing it from binding to CD28, thereby inhibiting T-cell activation and the subsequent inflammatory cascade in RA [1]. **Analysis of Incorrect Options:** * **B. Asthma:** Managed with inhaled corticosteroids, beta-agonists, or biologics like Omalizumab (anti-IgE) or Mepolizumab (anti-IL-5). * **C. Paroxysmal Nocturnal Hemoglobinuria (PNH):** The drug of choice is **Eculizumab**, a monoclonal antibody against the complement protein C5. * **D. Autoimmune Hemolytic Anemia (AIHA):** Primarily treated with corticosteroids, immunosuppressants (Azathioprine/Mycophenolate), or Rituximab (anti-CD20). **High-Yield Clinical Pearls for NEET-PG:** * **Belatacept:** A related drug (higher affinity for CD80/86) used in **renal transplantation** to prevent graft rejection. * **Screening:** Always screen for **Latent TB** and Hepatitis B before starting Abatacept. * **Contraindication:** Do not combine Abatacept with TNF-alpha inhibitors (e.g., Etanercept, Infliximab) due to the significantly increased risk of serious infections [1]. **Note on Management:** Early use of DMARDs such as methotrexate is the mainstay of RA treatment, with progression to biological drugs if necessary [2].

Q: Which joint is typically spared in Rheumatoid arthritis?

Distal interphalangeal joints of the finger. Rheumatoid Arthritis (RA) is a chronic, systemic inflammatory disease characterized by symmetric polyarthritis [1]. The hallmark of RA is the involvement of small joints of the hands and feet, specifically targeting the synovium [1]. **1. Why the Distal Interphalangeal (DIP) joints are spared:** The DIP joints are typically spared in RA because they have minimal synovial tissue compared to other joints. Inflammation of the DIP joints is instead a classic feature of **Osteoarthritis (Heberden’s nodes)** and **Psoriatic Arthritis** [3]. If a patient presents with DIP involvement, clinicians should look for alternative diagnoses. **2. Analysis of Incorrect Options:** * **Metacarpophalangeal (MCP) joints:** These are the most commonly involved joints in the hand in RA, leading to characteristic deformities like ulnar deviation. * **Proximal Interphalangeal (PIP) joints:** These are frequently involved early in the disease, often resulting in "fusiform swelling" or "spindle-shaped" fingers [1]. * **Atlanto-axial joint (C1-C2):** While RA generally spares the lumbosacral spine, it characteristically involves the cervical spine. Synovitis of the transverse ligament can lead to **atlanto-axial subluxation**, a potentially life-threatening complication. **3. High-Yield Clinical Pearls for NEET-PG:** * **Joints Spared in RA:** DIP joints, Thoracolumbar spine, and Sacroiliac joints. * **Early Sign:** Symmetrical involvement of the MTP (Metatarsophalangeal) joints of the feet is often the earliest manifestation. * **Deformities:** Boutonnière deformity (PIP flexion, DIP hyperextension) and Swan-neck deformity (PIP hyperextension, DIP flexion). * **Serology:** Anti-CCP (Cyclic Citrullinated Peptide) is more specific than Rheumatoid Factor (RF) for diagnosis [2].

Q: A young male presents with leg swelling and recurrent aphthous ulcers of his lips and tongue. He has also recently noted painful genital ulcers. There is no urethritis or conjunctivitis. On exam, he has evidence of deep vein thrombophlebitis. What is the most likely diagnosis?

Behcet syndrome. **Explanation:** The clinical presentation of **recurrent oral aphthous ulcers**, **genital ulcers**, and **vascular involvement** (deep vein thrombophlebitis) in a young male is classic for **Behcet Syndrome**. Behcet syndrome is a chronic, multisystemic, relapsing inflammatory perivasculitis. The hallmark is painful, recurrent oral ulcers (required for diagnosis) plus at least two of the following: recurrent genital ulcers, eye lesions (uveitis), skin lesions (erythema nodosum), or a positive pathergy test. A unique feature of Behcet is its **vasculitis of all vessel sizes** (small, medium, and large) on both the arterial and venous sides. Venous thrombosis (DVT) and pulmonary artery aneurysms are high-yield systemic manifestations. **Why other options are incorrect:** * **Ankylosing Spondylitis:** Primarily involves the axial skeleton (sacroiliitis). While it can cause uveitis, it does not present with recurrent aphthous or genital ulcers. * **Polymyalgia Rheumatica:** Typically affects patients over age 50, presenting with proximal muscle pain and stiffness (shoulders/hips) and elevated ESR, not mucosal ulcers or DVT. * **Polyarteritis Nodosa (PAN):** A medium-vessel vasculitis often associated with Hepatitis B. While it causes skin ulcers and livedo reticularis, it characteristically **spares the lungs** and does not typically cause recurrent aphthous/genital ulcers. **NEET-PG High-Yield Pearls:** * **Pathergy Test:** Development of a papule/pustule 24–48 hours after a sterile needle prick; highly specific for Behcet. * **HLA Association:** Strongly linked with **HLA-B51**. * **Vascular:** It is one of the few causes of **pulmonary artery aneurysms** (Hughes-Stovin Syndrome is considered a variant). * **Treatment:** Steroids for acute flares; Colchicine for mucosal lesions; Azathioprine or Anti-TNF agents for systemic/ocular involvement.

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

Ankylosing spondylitis. **Explanation:** **Ankylosing Spondylitis (AS)** is the correct answer because it has the strongest known association with **HLA-B27** among all human diseases [1]. Over **90-95%** of patients with AS are HLA-B27 positive, and the marker is also strongly associated with reactive arthritis [1]. HLA-B27 is a Class I surface antigen encoded by the B locus in the Major Histocompatibility Complex (MHC) on chromosome 6. It plays a critical role in the pathogenesis of Seronegative Spondyloarthritides (SpA) by presenting arthritogenic peptides to T-cells. **Analysis of Incorrect Options:** * **B. Rheumatoid Arthritis:** Associated with **HLA-DR4** (specifically the "shared epitope" on the DRB1 allele), not HLA-B27. * **C. Chronic Active Hepatitis:** Autoimmune hepatitis is more commonly associated with **HLA-DR3** and **HLA-DR4**. * **D. Diabetes Mellitus (Type 1):** Strongly linked to MHC Class II alleles, specifically **HLA-DR3** and **HLA-DR4** (DQ2 and DQ8). **High-Yield Clinical Pearls for NEET-PG:** * **Mnemonic for HLA-B27 (PAIR):** **P**soriatic arthritis, **A**nkylosing spondylitis, **I**nflammatory bowel disease-associated arthritis, and **R**eactive arthritis [1]. * **Relative Risk:** A person with HLA-B27 is approximately 90 times more likely to develop AS than someone without the marker. * **Other HLA Associations to Remember:** * **HLA-B51:** Behcet’s disease. * **HLA-DR2:** SLE, Multiple Sclerosis, Goodpasture syndrome. * **HLA-DQ2/DQ8:** Celiac disease. * **HLA-B*5701:** Abacavir hypersensitivity.

Q: Which of the following organs can be involved in Wegener's granulomatosis?

All of the above. Wegener’s Granulomatosis, now more commonly known as **Granulomatosis with Polyangiitis (GPA)**, is a systemic necrotizing vasculitis that primarily affects small to medium-sized vessels [1]. It is characterized by a classic "triad" of involvement: the upper respiratory tract, the lower respiratory tract, and the kidneys [2]. * **Nose (Upper Respiratory Tract):** This is often the earliest site of involvement (90% of cases). Clinical features include chronic sinusitis, epistaxis, and the classic **saddle-nose deformity** due to destruction of the nasal septal cartilage [2]. * **Lung (Lower Respiratory Tract):** Pulmonary involvement is a hallmark of the disease, presenting as cough, hemoptysis, and pleuritic chest pain. Radiologically, it often shows **bilateral nodular infiltrates** which frequently undergo cavitation [2]. * **Kidney:** Renal involvement occurs in approximately 75-80% of patients, manifesting as **Pauci-immune Crescentic Glomerulonephritis**. This can lead to rapidly progressive renal failure if left untreated. Since GPA is a multisystemic disease that frequently involves all three mentioned organs, **Option D** is the correct answer. **High-Yield Clinical Pearls for NEET-PG:** * **Serology:** The most specific marker is **c-ANCA (anti-PR3)**. Levels often correlate with disease activity. * **Histopathology:** Look for the "triad" of vasculitis, mucosal granulomatous inflammation, and tissue necrosis [2]. * **Treatment:** The standard induction therapy includes a combination of **Corticosteroids** and **Cyclophosphamide** (or Rituximab). * **Limited GPA:** A variant where the disease is restricted to the respiratory tract without renal involvement.

Q: A 25-year-old female presents with serous otitis media of the left ear, along with cough, occasional hemoptysis, hematuria, and epistaxis for one and a half months. Her hemoglobin is 7 gm. Blood pressure is > 170/100, proteinuria is +++, RA is positive, and ANCA is positive. What is the likely diagnosis?

Wegener's granulomatosis. The clinical presentation follows the classic triad of **Granulomatosis with Polyangiitis (GPA)**, formerly known as Wegener’s Granulomatosis: **Upper Respiratory Tract (URT), Lower Respiratory Tract (LRT), and Renal involvement.** 1. **Why Wegener’s is correct:** * **URT:** Epistaxis and serous otitis media (due to Eustachian tube dysfunction) are hallmark early signs. * **LRT:** Cough and hemoptysis indicate pulmonary nodules or alveolar hemorrhage. * **Renal:** Hematuria, proteinuria, and hypertension signify necrotizing glomerulonephritis [1]. * **Serology:** GPA is strongly associated with **c-ANCA (PR3-ANCA)**. While RA factor can be positive in 50% of GPA cases, it is a non-specific finding here. 2. **Why other options are incorrect:** * **Rheumatoid Arthritis:** While RA factor is positive, RA typically presents with symmetrical small joint polyarthritis, not life-threatening pulmonary-renal syndromes or chronic sinusitis. * **Rapidly Progressive Glomerulonephritis (RPGN):** This is a clinical syndrome (pathological finding), not a specific disease. While GPA causes RPGN, the multisystem involvement (ear, lung, nose) makes GPA the more complete diagnosis. * **Goodpasture’s Syndrome:** This involves a pulmonary-renal syndrome (anti-GBM antibodies) but **never** involves the upper respiratory tract (ears/sinuses). **Clinical Pearls for NEET-PG:** * **Classic Triad:** Sinusitis/Otitis + Pulmonary nodules + Glomerulonephritis. * **Biopsy Gold Standard:** Shows necrotizing granulomatous inflammation. * **Treatment:** Induction with Corticosteroids + Cyclophosphamide (or Rituximab). * **High-Yield Sign:** "Saddle nose deformity" due to nasal cartilage destruction.

Q: What is the most common cause of death in rheumatoid arthritis?

Cardiovascular complications. **Explanation:** **Rheumatoid Arthritis (RA)** is a chronic systemic inflammatory disorder. While joint destruction is the hallmark, the systemic inflammatory milieu significantly impacts long-term survival. **1. Why Cardiovascular Complications are Correct:** Cardiovascular disease (CVD) is the leading cause of mortality in RA patients, accounting for approximately **40-50% of deaths**. Chronic systemic inflammation leads to accelerated **atherosclerosis**, endothelial dysfunction, and an increased risk of myocardial infarction and stroke [1]. Pro-inflammatory cytokines (TNF-α, IL-6) promote a pro-thrombotic state and alter lipid metabolism, making RA an independent risk factor for CVD, similar to Diabetes Mellitus [2]. **2. Why Other Options are Incorrect:** * **Neurological complications:** While RA can cause atlantoaxial subluxation or peripheral neuropathies (e.g., Carpal Tunnel Syndrome), these are rarely fatal [3]. * **Lung disease complications:** Interstitial Lung Disease (ILD) is the second most common cause of death in RA [4]. While significant, its prevalence and mortality rate remain lower than CVD. * **Haematological complications:** RA is associated with anemia of chronic disease and Felty’s syndrome, but these are typically managed clinically and are not primary causes of death [3]. **Clinical Pearls for NEET-PG:** * **Most common cause of death:** Cardiovascular disease (Accelerated Atherosclerosis). * **Most common pulmonary manifestation:** Pleurisy/Pleural effusion (Exudative, characteristically very low glucose) [4]. * **Most common valvular lesion:** Mitral Regurgitation (though clinical heart failure is more often due to ischemic heart disease). * **Key marker for prognosis:** Anti-CCP (ACPA) antibodies are more specific than RF and correlate with aggressive joint destruction.

Q: The Kveim test is used for the diagnosis of which condition?

Sarcoidosis. The **Kveim test** (or Kveim-Siltzbach test) is a historical skin test used for the diagnosis of **Sarcoidosis**. It involves the intradermal injection of a suspension derived from the spleen or lymph node of a patient with known sarcoidosis [1]. If the patient has active sarcoidosis, a nodule develops at the injection site within 4–6 weeks. A biopsy of this nodule revealing **non-caseating granulomas** confirms a positive result. While highly specific, it is rarely used in modern practice due to the risk of transmitting infections (like BSE/vCJD) and the availability of more reliable diagnostic tools like EBUS-guided biopsy. **Analysis of Incorrect Options:** * **Tuberculosis:** Diagnosed via the **Mantoux (Tuberculin) test**, which uses Purified Protein Derivative (PPD) [2]. Unlike the Kveim test, the Mantoux test is read within 48–72 hours and shows caseating granulomas on biopsy. * **Systemic Lupus Erythematosus (SLE):** Diagnosis relies on clinical criteria (SLICC/ACR) and serology (ANA, Anti-dsDNA, Anti-Smith). The **Lupus Band Test** (direct immunofluorescence of skin biopsy) is the relevant skin-related investigation. * **Cystic Fibrosis:** The gold standard for diagnosis is the **Pilocarpine Iontophoresis (Sweat Chloride Test)**, which measures chloride concentration in sweat. **High-Yield Clinical Pearls for NEET-PG:** * **Sarcoidosis Hallmark:** Non-caseating granulomas and bilateral hilar lymphadenopathy [1]. * **Biochemical marker:** Elevated **Serum ACE levels** (reflects total granuloma burden). * **Calcium metabolism:** Hypercalcemia and hypercalciuria occur due to 1-alpha-hydroxylase activity in macrophages (increased Vitamin D3) [1]. * **Scadding’s Stages:** Stage I (Hilar adenopathy only) to Stage IV (Pulmonary fibrosis) [1].

Question 1

Abatacept is used in the treatment of which of the following conditions?

Accepted Answer

Rheumatoid Arthritis

Answer

Asthma

Answer

Paroxysmal nocturnal hemoglobinuria

Answer

Autoimmune Hemolytic Anemia

Question 2

Which joint is typically spared in Rheumatoid arthritis?

Accepted Answer

Distal interphalangeal joints of the finger

Answer

Metacarpophalangeal joints of the hand

Answer

Proximal interphalangeal joints of the finger

Answer

Atlanto-axial joint

Question 3

A young male presents with leg swelling and recurrent aphthous ulcers of his lips and tongue. He has also recently noted painful genital ulcers. There is no urethritis or conjunctivitis. On exam, he has evidence of deep vein thrombophlebitis. What is the most likely diagnosis?

Accepted Answer

Behcet syndrome

Answer

Ankylosing spondylitis

Answer

Polymyalgia rheumatica

Answer

Polyarteritis nodosa

Question 4

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

Accepted Answer

Ankylosing spondylitis

Answer

Rheumatoid arthritis

Answer

Chronic active hepatitis

Answer

Diabetes

Question 5

Which of the following organs can be involved in Wegener's granulomatosis?

Accepted Answer

All of the above

Answer

Lung

Answer

Nose

Answer

Kidney

Question 6

A 25-year-old female presents with serous otitis media of the left ear, along with cough, occasional hemoptysis, hematuria, and epistaxis for one and a half months. Her hemoglobin is 7 gm. Blood pressure is > 170/100, proteinuria is +++, RA is positive, and ANCA is positive. What is the likely diagnosis?

Accepted Answer

Wegener's granulomatosis

Answer

Rheumatoid arthritis

Answer

Rapidly proliferative glomerulonephritis

Answer

Goodpasture's syndrome

Question 7

What is the most common cause of death in rheumatoid arthritis?

Accepted Answer

Cardiovascular complications

Answer

Neurological complications

Answer

Lung disease complications

Answer

Haematological complications

Question 8

Which of the following are classified as small vessel vasculitis?

Accepted Answer

Classical Polyarteritis Nodosa, HSP, Churg-Strauss syndrome

Answer

Classical Polyarteritis Nodosa, HSP, Wegener's granulomatosis

Answer

Classical Polyarteritis Nodosa, Churg-Strauss syndrome, Giant cell arteritis

Answer

Wegener's granulomatosis, Giant cell arteritis, Churg-Strauss syndrome

Question 9

The Kveim test is used for the diagnosis of which condition?

Accepted Answer

Sarcoidosis

Answer

Tuberculosis

Answer

Systemic lupus erythematosus

Answer

Cystic fibrosis

Question 10

A patient with limited systemic sclerosis for the past 10 years complains of shortness of breath for the past one year. His pulmonary function tests are as follows: FVC 2.63 (predicted 2.82), FEV1 88% of predicted, and DLCO 5.2 (predicted 16.3). Which among the following is the probable diagnosis?

Accepted Answer

Pulmonary hypertension

Answer

Interstitial lung disease

Answer

Pneumothorax

Answer

Diaphragmatic weakness

Rheumatology and Immunology — MCQs

Rheumatology and Immunology — MCQs

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