Rheumatology and Immunology — MCQs

A patient presents with progressive muscle weakness, difficulty rising from a chair, and climbing stairs. Physical examination reveals lilac-colored discoloration of the upper eyelids and periorbital edema. Laboratory tests show elevated creatinine phosphokinase and a positive antinuclear antibody. Which of the following antibodies is most likely to be present in this patient?

Q455Medium

Xerostomia and enlargement of salivary and lacrimal glands are seen in which condition?

Q456Easy

What is true about Henoch-Schönlein purpura?

Q457Medium

A 20-year-old woman presents with bilateral conductive deafness, palpable purpura on the legs, and hemoptysis. Radiograph of the chest shows a thin-walled cavity in the left lower zone. Investigations reveal a total leukocyte count of 12,000/mm³, red cell casts in the urine, and serum creatinine of 3 mg/dL. What is the most probable diagnosis?

Q458Medium

Which of the following is NOT true about the condition?

Q459Easy

Systemic sclerosis shows all except?

Q460Medium

A 50-year-old woman with SLE presents with headache and fatigue. Her past manifestations of SLE have included arthralgias, hemolytic anemia, malar rash, and mouth ulcers. She has high titres of antibodies to dsDNA. She is currently taking prednisone, 5 mg daily, and hydroxychloroquine, 200 mg daily. On examination, her BP is 190/110 mmHg with a HR of 98 bpm. Urinalysis shows 25 RBCs per HPF with 2+ proteinuria. No RBC casts are identified. Her BUN is 90 mg/dL, and creatinine is 2.8 mg/dL (baseline 0.8 mg/dL). She has no prior renal disease related to SLE and is not taking NSAIDs. She denies recent illness, decreased oral intake, or diarrhea. What is the most appropriate next step in the management of this patient?

Rheumatology and Immunology Indian Medical PG Practice Questions and MCQs

Question 451: A person with involvement of the upper respiratory tract, lungs, and kidneys shows evidence of granulomas on histopathology. What is the most likely diagnosis?

A. Wegener's Granulomatosis (Correct Answer)
B. Goodpasture Syndrome
C. Tuberculosis
D. Sarcoidosis

Explanation: The clinical triad of **upper respiratory tract** (sinusitis, otitis media, saddle-nose deformity), **lower respiratory tract** (nodules, cavitary lesions), and **kidneys** (pauci-immune glomerulonephritis) is the hallmark of **Granulomatosis with Polyangiitis (GPA)**, formerly known as **Wegener’s Granulomatosis** [1]. The presence of **granulomas** on histopathology is the defining feature that distinguishes it from other small-vessel vasculitides like Microscopic Polyangiitis (MPA) [1]. **Why other options are incorrect:** * **Goodpasture Syndrome:** Characterized by the "pulmonary-renal syndrome" (hemoptysis and hematuria) due to anti-GBM antibodies [1]. However, it **does not involve the upper respiratory tract** and does not show granulomas on biopsy. * **Tuberculosis:** While TB causes granulomas and can affect lungs and kidneys, it is an infectious process. The specific "triad" involvement, especially the destructive upper airway disease, is more characteristic of GPA in a vasculitis context. * **Sarcoidosis:** A multisystem granulomatous disease that commonly affects the lungs and hilar lymph nodes [1]. However, it rarely causes the aggressive necrotizing glomerulonephritis or the destructive upper airway lesions seen in GPA. **NEET-PG High-Yield Pearls:** * **Serology:** GPA is strongly associated with **c-ANCA (anti-PR3)**. * **Histopathology:** Look for the triad of vasculitis, mucosal inflammation, and **necrotizing granulomas** [1]. * **Renal Biopsy:** Shows "Pauci-immune" crescentic glomerulonephritis (minimal Ig/complement deposition). * **Treatment:** Induction with Cyclophosphamide or Rituximab plus Corticosteroids.

Question 452: Which of the following joints is NOT typically involved in rheumatoid arthritis?

A. Proximal interphalangeal (PIP) joints
B. Distal interphalangeal (DIP) joints (Correct Answer)
C. Metacarpophalangeal (MCP) joints
D. Cervical spine

Explanation: Rheumatoid Arthritis (RA) is a chronic, systemic inflammatory disorder characterized by symmetrical polyarthritis that primarily targets the **synovial lining** of joints [1]. **1. Why DIP joints are the correct answer:** The **Distal Interphalangeal (DIP) joints** are characteristically **spared** in Rheumatoid Arthritis [2]. Involvement of the DIP joints is a hallmark of other conditions, most notably **Osteoarthritis (Heberden’s nodes)** and **Psoriatic Arthritis** [3]. If a patient presents with DIP involvement, clinicians should look for alternative diagnoses rather than RA. **2. Why the other options are incorrect:** * **PIP and MCP joints (Options A & C):** These are the "classic" sites of involvement in RA. Symmetrical swelling and tenderness of the Proximal Interphalangeal (PIP) and Metacarpophalangeal (MCP) joints are early diagnostic features [2]. * **Cervical Spine (Option D):** While RA typically spares the thoracolumbar spine, it frequently involves the **cervical spine**, particularly the **atlanto-axial (C1-C2) joint**. This can lead to atlanto-axial subluxation, a serious complication that can cause spinal cord compression [4]. **Clinical Pearls for NEET-PG:** * **Symmetry:** RA is typically a symmetrical polyarthritis. * **Morning Stiffness:** Lasts >1 hour and improves with activity (unlike Osteoarthritis) [2]. * **Spared Joints:** RA spares the DIP joints and the entire thoracolumbar spine. * **Deformities:** Chronic RA leads to Ulnar deviation, Swan-neck deformity, and Boutonniere deformity. * **Serology:** Anti-CCP (Cyclic Citrullinated Peptide) is more specific than Rheumatoid Factor (RF) for diagnosis [2].

Question 453: What is not true about angioneurotic edema?

A. Pitting edema of the face, lips, and mucous membranes. (Correct Answer)
B. C1 esterase inhibitor deficiency can cause it.
C. Extreme temperature exposure can provoke it.
D. It is known to occur with ACE inhibitors.

Explanation: Angioedema is characterized by **non-pitting edema**. Unlike peripheral edema caused by heart failure or renal issues (which involves fluid accumulation in the interstitium), angioedema involves deep dermal, subcutaneous, or submucosal swelling caused by increased vascular permeability [2]. Because this swelling is often tense and involves protein-rich fluid or bradykinin-mediated pathways, it does not leave an indentation when pressed. **2. Analysis of other options:** * **Option B:** C1 esterase inhibitor deficiency is the hallmark of **Hereditary Angioedema (HAE)**. Lack of this enzyme leads to the overproduction of bradykinin, a potent vasodilator [3]. * **Option C:** Physical triggers, including **extreme temperature changes** (cold or heat), trauma, or emotional stress, are well-documented triggers for acute attacks in susceptible individuals. * **Option D:** **ACE inhibitors** are a common cause of acquired angioedema [1]. They prevent the breakdown of bradykinin, leading to its accumulation [3]. This is a "class effect" and can occur even after years of uneventful therapy. **Clinical Pearls for NEET-PG:** * **Key Mediator:** Bradykinin (in HAE and ACE-I induced) [3] or Histamine (in allergic types). * **Life-threatening Complication:** Laryngeal edema (airway obstruction) [2]. * **Treatment:** For HAE, antihistamines and steroids are **ineffective** [4]. Use C1 inhibitor concentrate, Ecallantide, or Icatibant (bradykinin B2 receptor antagonist). Fresh Frozen Plasma (FFP) can be used in emergencies if specific concentrates are unavailable. * **Diagnostic Marker:** Low **C4 levels** are the most sensitive screening tool for HAE, even between attacks.

Question 454: A patient presents with progressive muscle weakness, difficulty rising from a chair, and climbing stairs. Physical examination reveals lilac-colored discoloration of the upper eyelids and periorbital edema. Laboratory tests show elevated creatinine phosphokinase and a positive antinuclear antibody. Which of the following antibodies is most likely to be present in this patient?

A. Anti-Jo1 antibodies
B. Anti-P155 antibody
C. Anti-P140 antibody
D. Anti-Mi2 antibodies (Correct Answer)

Explanation: ### Explanation **1. Why Anti-Mi2 is the Correct Answer:** The patient presents with classic features of **Dermatomyositis (DM)**: proximal muscle weakness (difficulty rising from a chair/climbing stairs) and pathognomonic cutaneous findings, specifically the **Heliotrope rash** (lilac-colored discoloration of the upper eyelids with periorbital edema) [1]. **Anti-Mi2 antibodies** are highly specific for Dermatomyositis. They are associated with the classic skin manifestations (Heliotrope rash, Gottron papules), a relatively acute onset, and a **good prognosis** with a favorable response to corticosteroid therapy. **2. Analysis of Incorrect Options:** * **Anti-Jo1 (Option A):** These are the most common Myositis-Specific Antibodies (MSAs) but are primarily associated with **Antisynthetase Syndrome**, characterized by interstitial lung disease (ILD), "mechanic's hands," Raynaud’s phenomenon, and inflammatory arthritis [1]. * **Anti-P155/140 (Options B & C):** Also known as Anti-TIF1-$\gamma$ (P155) and Anti-NXP2 (P140). While these are found in DM, they are strongly associated with **cancer-associated (malignancy-associated) dermatomyositis** in adults and juvenile dermatomyositis. The classic presentation described here points more typically toward the Mi2 subtype in a standard exam setting. **3. NEET-PG High-Yield Clinical Pearls:** * **Pathognomonic Skin Signs:** Heliotrope rash (eyelids), Gottron papules (violaceous papules over MCP/PIP joints), and Shawl sign (erythema over back/shoulders) [1]. * **Muscle Enzymes:** Creatinine Phosphokinase (CPK) is the most sensitive enzyme for monitoring disease activity. * **Gold Standard Diagnosis:** Muscle biopsy showing **perifascicular atrophy** and perivascular infiltrates (CD4+ T cells). * **Anti-SRP antibodies:** Associated with severe, treatment-resistant necrotizing myopathy. * **Anti-MDA5:** Associated with "amyopathic" dermatomyositis and rapidly progressive ILD. * **ANA Association:** Antinuclear antibodies are present in 30–80% of patients with dermatomyositis or polymyositis [2].

Question 455: Xerostomia and enlargement of salivary and lacrimal glands are seen in which condition?

A. Sicca syndrome
B. Sjogren's syndrome
C. Mickulicz's disease (Correct Answer)
D. None of the above

Explanation: The correct answer is **Mickulicz’s disease**. This condition is characterized by the triad of **painless, symmetrical enlargement** of the lacrimal and salivary glands (parotid and submandibular) accompanied by **xerostomia** (dry mouth) and **xerophthalmia** (dry eyes). Historically, Mikulicz’s disease was considered a subset of Sjogren’s syndrome; however, modern pathology classifies it as a manifestation of **IgG4-related disease (IgG4-RD)**. It is distinguished by elevated serum IgG4 levels and prominent infiltration of IgG4-positive plasma cells in the affected glands, leading to hypertrophy rather than the atrophy typically seen in primary Sjogren’s. **Analysis of Options:** * **A. Sicca syndrome:** This refers specifically to the clinical symptoms of dryness (dry eyes and dry mouth) without systemic involvement. While it describes the symptoms, it does not inherently imply the glandular enlargement characteristic of Mikulicz’s. * **B. Sjogren’s syndrome:** While Sjogren’s presents with xerostomia and xerophthalmia, the glands are often **atrophic** or only mildly enlarged. Mikulicz’s disease is the specific historical term used when massive, persistent glandular hypertrophy is the dominant feature. * **D. None of the above:** Incorrect, as Mikulicz’s disease is the classic description for this presentation. **High-Yield Clinical Pearls for NEET-PG:** * **Mikulicz’s Disease vs. Sjogren’s:** Mikulicz’s involves IgG4+ plasma cells and responds well to glucocorticoids; Sjogren’s involves anti-Ro (SS-A) and anti-La (SS-B) antibodies. * **Mikulicz’s Syndrome:** This is a separate term used when glandular enlargement is secondary to other systemic diseases like Sarcoidosis, Leukemia, or Lymphoma. * **Histology:** Look for "storiform fibrosis" and "obliterative phlebitis" in IgG4-related diseases.

Question 456: What is true about Henoch-Schönlein purpura?

A. Palpable purpura (Correct Answer)
B. Thrombocytopenia
C. C-ANCA positive
D. All of the above

Explanation: **Henoch-Schönlein Purpura (HSP)**, now officially known as **IgA Vasculitis**, is a small-vessel leukocytoclastic vasculitis characterized by the deposition of IgA-dominant immune complexes [1]. ### **Explanation of Options:** * **A. Palpable purpura (Correct):** This is the clinical hallmark of HSP [1]. Unlike simple purpura (which is flat), the purpura in HSP is **palpable** because the underlying pathology is an inflammatory vasculitis. This inflammation causes vessel wall damage and localized edema, making the lesions raised [2]. They typically appear in a symmetrical distribution on the lower extremities and buttocks. * **B. Thrombocytopenia (Incorrect):** HSP is a **non-thrombocytopenic** purpura. The platelet count is characteristically **normal** or even elevated (as an acute phase reactant). This is a crucial diagnostic differentiator from other causes of purpura like ITP or TTP. * **C. C-ANCA positive (Incorrect):** HSP is a **pauci-immune/immune-complex mediated** vasculitis, not an ANCA-associated vasculitis. C-ANCA is specifically associated with Granulomatosis with Polyangiitis (Wegener's). ### **High-Yield Clinical Pearls for NEET-PG:** 1. **Classic Tetrad:** * **Palpable Purpura** (without thrombocytopenia). * **Arthralgia/Arthritis** (usually knees and ankles). * **Abdominal Pain** (may lead to Intussusception—specifically ileo-ileal). * **Renal Involvement** (HSP Nephritis, which is histologically identical to IgA Nephropathy/Berger’s disease). 2. **Trigger:** Often follows an **Upper Respiratory Tract Infection** (Group A Streptococcus is common) [1]. 3. **Diagnosis:** Primarily clinical. If a biopsy is done (skin or kidney), **Immunofluorescence** shows **IgA and C3 deposition**. 4. **Prognosis:** Generally excellent and self-limiting; the long-term prognosis depends entirely on the severity of renal involvement.

Question 457: A 20-year-old woman presents with bilateral conductive deafness, palpable purpura on the legs, and hemoptysis. Radiograph of the chest shows a thin-walled cavity in the left lower zone. Investigations reveal a total leukocyte count of 12,000/mm³, red cell casts in the urine, and serum creatinine of 3 mg/dL. What is the most probable diagnosis?

A. Henoch-Schonlein purpura
B. Polyarteritis nodosa
C. Wegener's granulomatosis (Correct Answer)
D. Disseminated tuberculosis

Explanation: The clinical presentation of Wegener’s Granulomatosis (now known as Granulomatosis with Polyangiitis or GPA) is characterized by the classic triad of upper respiratory tract, lower respiratory tract, and renal involvement [1]. 1. **Upper Respiratory/Ear:** Conductive deafness (due to otitis media or Eustachian tube obstruction) and nasal crusting are hallmark features. 2. **Lower Respiratory:** Hemoptysis and **cavitary lesions** on chest X-ray (classically thin-walled) indicate necrotizing granulomatous inflammation [1]. In some patients, pulmonary haemorrhage may occur, which can be life-threatening [1]. 3. **Renal:** Red cell casts and elevated creatinine (3 mg/dL) signify **Pauci-immune Glomerulonephritis** [1]. Small-vessel vasculitis commonly affects the kidneys, with rapid and profound impairment of glomerular function [1]. 4. **Skin:** Palpable purpura reflects underlying small-vessel vasculitis [2]. **Why other options are incorrect:** * **Henoch-Schonlein Purpura (IgA Vasculitis):** While it presents with palpable purpura and hematuria, it typically affects children, involves the GI tract (abdominal pain), and **rarely** causes cavitary lung lesions or deafness. * **Polyarteritis Nodosa (PAN):** PAN is a medium-vessel vasculitis that **spares the lungs**. It does not cause pulmonary cavitation or glomerulonephritis (it causes renal artery microaneurysms instead). * **Disseminated Tuberculosis:** While it causes cavities and hemoptysis, it would not explain the acute glomerulonephritis (red cell casts) or the specific multisystem vasculitic pattern seen here. **NEET-PG High-Yield Pearls:** * **Marker:** GPA is strongly associated with **c-ANCA (anti-PR3)**. * **Biopsy:** Shows necrotizing granulomas and vasculitis. * **Treatment:** Induction with Corticosteroids + Cyclophosphamide (or Rituximab) [1]. * **Limited GPA:** Term used when the disease is restricted to the respiratory tract without renal involvement.

Question 458: Which of the following is NOT true about the condition?

A. Piano key movement of ulnar styloid
B. Z line deformity
C. Atlantoaxial deformity
D. Tender nodes (Correct Answer)

Explanation: ***Tender nodes*** - **Rheumatoid nodules** are characteristically **non-tender** firm subcutaneous masses that occur in approximately 20-30% of RA patients. - These nodules are typically found over **pressure points** like elbows and are associated with **positive rheumatoid factor** and more severe disease. *Piano key movement of ulnar styloid* - This is a classic sign of **distal radioulnar joint subluxation** commonly seen in advanced rheumatoid arthritis. - The **ulnar styloid** can be depressed like a piano key due to **synovial destruction** and ligamentous laxity at the wrist. *Z line deformity* - Also known as **Z-thumb deformity**, this involves **hyperextension** at the metacarpophalangeal joint and **flexion** at the interphalangeal joint. - Results from **synovial inflammation** destroying joint capsules and tendons, leading to characteristic hand deformities in RA. *Atlantoaxial deformity* - **Atlantoaxial subluxation** occurs in up to 25% of RA patients due to **synovial proliferation** affecting the C1-C2 joint. - Can lead to **cervical myelopathy** and requires careful monitoring, especially before intubation or surgery.

Question 459: Systemic sclerosis shows all except?

A. Acroosteolysis
B. Tufting (Correct Answer)
C. Calcinosis cutis
D. Digital ulcers

Explanation: Systemic Sclerosis (Scleroderma) is a multisystem connective tissue disorder characterized by vascular dysfunction and progressive fibrosis [1]. To answer this question, one must distinguish between the destruction of bone and the preservation of the distal tufts. **Why "Tufting" is the correct answer:** In Systemic Sclerosis, the hallmark radiological finding in the digits is **Acroosteolysis** (resorption of the terminal phalanges). This leads to the **loss or sharpening of the distal tufts**, resulting in a "pencil-tip" appearance. Therefore, "Tufting" (the presence of normal bulbous distal phalangeal bone) is NOT a feature of the disease; rather, its destruction is. **Analysis of Incorrect Options:** * **Acroosteolysis (A):** This is a classic feature. Ischemic changes due to Raynaud’s phenomenon and pressure from tight, fibrotic skin lead to the resorption of the distal phalangeal bone. * **Calcinosis cutis (C):** This refers to dystrophic calcification in the skin and soft tissues. It is a core component of the **CREST syndrome** (Calcinosis, Raynaud’s, Esophageal dysmotility, Sclerodactyly, Telangiectasia). * **Digital ulcers (D):** These occur due to severe digital ischemia and vasculopathy [1]. They are painful, difficult to heal, and often located at the fingertips or over bony prominences. **Clinical Pearls for NEET-PG:** * **Radiology:** Look for "tapering of soft tissues" and "subcutaneous calcification" on X-rays of the hands. * **Antibody Associations:** * **Anti-Scl-70 (Anti-topoisomerase I):** Associated with Diffuse Cutaneous Systemic Sclerosis and interstitial lung disease. * **Anti-Centromere:** Associated with Limited Cutaneous Systemic Sclerosis (CREST) and pulmonary hypertension. * **Earliest Sign:** Raynaud’s phenomenon is often the first clinical manifestation [1].

Question 460: A 50-year-old woman with SLE presents with headache and fatigue. Her past manifestations of SLE have included arthralgias, hemolytic anemia, malar rash, and mouth ulcers. She has high titres of antibodies to dsDNA. She is currently taking prednisone, 5 mg daily, and hydroxychloroquine, 200 mg daily. On examination, her BP is 190/110 mmHg with a HR of 98 bpm. Urinalysis shows 25 RBCs per HPF with 2+ proteinuria. No RBC casts are identified. Her BUN is 90 mg/dL, and creatinine is 2.8 mg/dL (baseline 0.8 mg/dL). She has no prior renal disease related to SLE and is not taking NSAIDs. She denies recent illness, decreased oral intake, or diarrhea. What is the most appropriate next step in the management of this patient?

A. Initiate cyclophosphamide, 500 mg/m2 body surface area intravenously (IV), and plan to repeat monthly for 3-6 months.
B. Initiate hemodialysis.
C. Initiate high-dose steroid therapy (IV methylprednisolone, 1000 mg daily for 3 doses, followed by oral prednisone, 1 mg/kg daily) and mycophenolate mofetil, 2 g daily. (Correct Answer)
D. Initiate plasmapheresis.

Explanation: ### Explanation The patient presents with **Lupus Nephritis (LN)**, likely Class III or IV (Proliferative), characterized by sudden-onset hypertension, hematuria, proteinuria, and a significant rise in serum creatinine (Rapidly Progressive Glomerulonephritis pattern). In a patient with known SLE and high anti-dsDNA titers, new-onset renal impairment is a medical emergency [1]. **1. Why Option C is Correct:** The standard of care for induction therapy in severe or proliferative LN (Class III/IV) involves **high-dose "pulse" corticosteroids** (IV Methylprednisolone) to rapidly suppress inflammation, followed by an immunosuppressive agent. Both **Mycophenolate Mofetil (MMF)** and **Cyclophosphamide** are first-line options for induction. MMF is often preferred in women of childbearing age due to the lower risk of infertility compared to Cyclophosphamide and has shown equal efficacy in achieving remission. **2. Why Other Options are Incorrect:** * **Option A:** While Cyclophosphamide is a valid induction agent, it is typically used alongside pulse steroids, not as monotherapy. Furthermore, MMF (Option C) is increasingly favored as the initial choice in many clinical scenarios. * **Option B:** Hemodialysis is indicated for refractory uremia, hyperkalemia, or fluid overload. While her BUN/Creatinine are elevated, there is no immediate indication for dialysis before attempting to reverse the underlying inflammatory process. * **Option C:** Plasmapheresis is not a standard treatment for LN. It is reserved for specific complications like Thrombotic Thrombocytopenic Purpura (TTP) or Catastrophic Antiphospholipid Syndrome (CAPS). **Clinical Pearls for NEET-PG:** * **Gold Standard Diagnosis:** Renal biopsy is the most definitive test to classify LN and guide therapy. * **Monitoring:** Low C3/C4 levels and rising anti-dsDNA titers are highly predictive of a lupus flare, especially renal involvement [1]. * **Drug of Choice:** MMF is superior to Cyclophosphamide for induction in African American and Hispanic populations and is preferred for maintaining fertility. * **Target:** The goal of therapy is to reduce proteinuria and stabilize/improve serum creatinine.

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

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Sjögren's Syndrome

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Antiphospholipid Syndrome

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