Rheumatology and Immunology Practice Questions

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

Wegener's Granulomatosis. 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.

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

Distal interphalangeal (DIP) joints. 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].

Q: What is not true about angioneurotic edema?

Pitting edema of the face, lips, and mucous membranes.. 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.

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

Mickulicz's disease. 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.

Q: What is true about Henoch-Schönlein purpura?

Palpable purpura. **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.

Q: Which of the following is NOT true about the condition?

Tender nodes. ***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.

Q: Systemic sclerosis shows all except?

Tufting. 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 1

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

Accepted Answer

Wegener's Granulomatosis

Answer

Goodpasture Syndrome

Answer

Tuberculosis

Answer

Sarcoidosis

Question 2

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

Accepted Answer

Distal interphalangeal (DIP) joints

Answer

Proximal interphalangeal (PIP) joints

Answer

Metacarpophalangeal (MCP) joints

Answer

Cervical spine

Question 3

What is not true about angioneurotic edema?

Accepted Answer

Pitting edema of the face, lips, and mucous membranes.

Answer

C1 esterase inhibitor deficiency can cause it.

Answer

Extreme temperature exposure can provoke it.

Answer

It is known to occur with ACE inhibitors.

Question 4

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?

Accepted Answer

Anti-Mi2 antibodies

Answer

Anti-Jo1 antibodies

Answer

Anti-P155 antibody

Answer

Anti-P140 antibody

Question 5

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

Accepted Answer

Mickulicz's disease

Answer

Sicca syndrome

Answer

Sjogren's syndrome

Answer

None of the above

Question 6

What is true about Henoch-Schönlein purpura?

Accepted Answer

Palpable purpura

Answer

Thrombocytopenia

Answer

C-ANCA positive

Answer

All of the above

Question 7

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?

Accepted Answer

Wegener's granulomatosis

Answer

Henoch-Schonlein purpura

Answer

Polyarteritis nodosa

Answer

Disseminated tuberculosis

Question 8

Which of the following is NOT true about the condition?

Accepted Answer

Tender nodes

Answer

Piano key movement of ulnar styloid

Answer

Z line deformity

Answer

Atlantoaxial deformity

Question 9

Systemic sclerosis shows all except?

Accepted Answer

Tufting

Answer

Acroosteolysis

Answer

Calcinosis cutis

Answer

Digital ulcers

Question 10

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?

Accepted Answer

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.

Answer

Initiate cyclophosphamide, 500 mg/m2 body surface area intravenously (IV), and plan to repeat monthly for 3-6 months.

Answer

Initiate hemodialysis.

Answer

Initiate plasmapheresis.

Rheumatology and Immunology — MCQs

Rheumatology and Immunology — MCQs

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