Rheumatology and Immunology Practice Questions

Q: A 13-year-old female child presented with recurrent sinusitis, fever, arthralgia, respiratory distress, hematuria, and hypertension. Renal biopsy showed necrotizing granuloma. The anti-proteinase-3 ANCA was positive. What is the most likely diagnosis?

Wegener's granulomatosis. ### Explanation The clinical presentation of a triad involving the **upper respiratory tract** (sinusitis), **lower respiratory tract** (respiratory distress), and **kidneys** (hematuria, hypertension) is classic for **Granulomatosis with Polyangiitis (GPA)**, formerly known as **Wegener’s granulomatosis** [1]. **Why Option B is Correct:** * **Clinical Triad:** GPA typically affects the upper airway (90%), lungs, and kidneys (Pauci-immune glomerulonephritis). * **Pathology:** The presence of **necrotizing granulomas** on renal biopsy is a hallmark feature that distinguishes it from other small-vessel vasculitides. * **Serology:** **c-ANCA (anti-PR3)** is highly specific (approx. 90%) for GPA [2]. **Why Other Options are Incorrect:** * **A. Polyarteritis Nodosa (PAN):** A medium-vessel vasculitis associated with Hepatitis B. It typically **spares the lungs** and does not involve granuloma formation or ANCA. * **C. Microscopic Polyangiitis (MPA):** Similar to GPA but **lacks granulomatous inflammation** and primarily involves **p-ANCA (anti-MPO)**. Upper airway involvement is rare. * **D. Churg-Strauss Syndrome (EGPA):** Characterized by **asthma, eosinophilia**, and p-ANCA [2]. While it involves granulomas, the absence of eosinophilia and the presence of anti-PR3 make it unlikely here. **High-Yield Pearls for NEET-PG:** 1. **GPA Serology:** c-ANCA (anti-Proteinase 3); **MPA/EGPA Serology:** p-ANCA (anti-Myeloperoxidase) [2]. 2. **Classic Sign:** "Saddle nose deformity" due to nasal cartilage destruction. 3. **Chest X-ray:** May show bilateral nodular infiltrates with cavitation. 4. **Treatment:** Induction with Corticosteroids + Cyclophosphamide (or Rituximab) [2].

Q: What is a characteristic feature of Still's disease?

Rashes. **Adult-Onset Still’s Disease (AOSD)** is a systemic inflammatory disorder characterized by the classic triad of high-spiking fevers, arthritis, and a distinctive rash. [1] **Why Rashes are the correct answer:** The characteristic rash in Still’s disease is a **transient, salmon-pink, maculopapular eruption**. It is typically non-pruritic and "evanescent," meaning it appears during febrile peaks and disappears when the fever subsides. It is most commonly found on the trunk and proximal extremities and may exhibit the **Koebner phenomenon** (skin lesions appearing at sites of minor trauma). **Analysis of Incorrect Options:** * **A. Prominent kidney involvement:** Unlike SLE or Vasculitis, renal involvement is rare in Still’s disease. If it occurs, it is usually due to secondary amyloidosis from chronic inflammation. * **C. Positive Rheumatoid factor:** AOSD is classically **seronegative**. The absence of Rheumatoid Factor (RF) and Anti-CCP is a key diagnostic criterion (Yamaguchi Criteria). * **D. Neutropenia:** Still’s disease is associated with **leukocytosis** (WBC >10,000/mm³), specifically **neutrophilia** (≥80% granulocytes), rather than neutropenia. **High-Yield Clinical Pearls for NEET-PG:** 1. **Yamaguchi Criteria:** The most sensitive diagnostic tool. Major criteria include fever (≥39°C), arthralgia/arthritis, characteristic rash, and leukocytosis. [1] 2. **Hyperferritinemia:** Extremely high serum ferritin levels (often >1000–4000 ng/mL) are a hallmark and correlate with disease activity. 3. **Liver Function:** Mildly elevated transaminases and splenomegaly are common findings. 4. **Treatment:** NSAIDs for mild cases; Steroids and IL-1 inhibitors (Anakinra) or IL-6 inhibitors (Tocilizumab) for refractory cases. [1]

Q: All of the following are found in Primary Sjogren's syndrome except?

Connective tissue disorder. ### Explanation The key to answering this question lies in the classification of Sjögren’s Syndrome (SS) into **Primary** and **Secondary** forms. **Why "Connective Tissue Disorder" is the correct answer:** Primary Sjögren’s Syndrome (pSS) occurs as an **isolated** autoimmune condition in the absence of any other underlying connective tissue disease (CTD). If a patient has Sjögren’s symptoms (sicca complex) along with a diagnosed CTD—such as Rheumatoid Arthritis (most common), Systemic Lupus Erythematosus (SLE), or Systemic Sclerosis—it is classified as **Secondary Sjögren’s Syndrome**. Therefore, the presence of another CTD excludes the diagnosis of "Primary" Sjögren's. **Analysis of Incorrect Options:** * **Xerostomia (Dry mouth):** A hallmark clinical feature of pSS caused by lymphocytic infiltration and destruction of the salivary glands. * **Xerophthalmia (Dry eyes):** Another cardinal feature resulting from the involvement of the lacrimal glands (Keratoconjunctivitis sicca). * **Lymphoma:** Patients with Primary Sjögren’s have a **40-fold increased risk** of developing B-cell lymphomas (specifically MALT lymphoma). This is a well-known late complication of the chronic B-cell hyperreactivity seen in the disease. **NEET-PG High-Yield Pearls:** * **Antibodies:** Anti-Ro (SS-A) and Anti-La (SS-B) are the most specific markers [1]. * **Diagnosis:** The "Gold Standard" for diagnosis is a **minor salivary gland biopsy** (Lip biopsy) showing lymphocytic aggregates (Focus score ≥1). * **Schirmer’s Test:** Used to quantify tear production (Positive if <5 mm in 5 minutes). * **Extraglandular manifestations:** Include Raynaud’s phenomenon, cutaneous vasculitis, and interstitial nephritis.

Q: Which of the following conditions is associated with a positive C-ANCA?

Wegener's granulomatosis (WG). **Explanation:** **1. Why Wegener's Granulomatosis (WG) is Correct:** Wegener's Granulomatosis (now known as **Granulomatosis with Polyangiitis - GPA**) is a small-vessel vasculitis characterized by the presence of **C-ANCA** (Cytoplasmic Antineutrophil Cytoplasmic Antibodies). The primary target antigen for C-ANCA is **Proteinase-3 (PR3)**. In active systemic GPA, C-ANCA has a high sensitivity (>90%) and specificity, making it a hallmark diagnostic marker. The disease classically presents with a triad of upper respiratory tract involvement (saddle nose deformity), lower respiratory tract (pulmonary nodules/hemorrhage), and renal involvement (Pauci-immune glomerulonephritis). **2. Why the Other Options are Incorrect:** * **Takayasu Arteritis (TA) & Giant Cell Arteritis (GCA):** These are **Large Vessel Vasculitides**. They are not associated with ANCA; their diagnosis relies on imaging (angiography) or biopsy (temporal artery) and inflammatory markers like ESR/CRP. * **Polyarteritis Nodosa (Classic PAN):** This is a **Medium Vessel Vasculitis**. By definition, classic PAN is **ANCA-negative**. It is strongly associated with Hepatitis B infection and characterized by microaneurysms (beading appearance on angiography). **High-Yield Clinical Pearls for NEET-PG:** * **C-ANCA (PR3-ANCA):** Most specific for Granulomatosis with Polyangiitis (GPA). * **P-ANCA (MPO-ANCA):** Associated with Microscopic Polyangiitis (MPA), Churg-Strauss Syndrome (EGPA), and Primary Sclerosing Cholangitis (PSC). * **Rule of Thumb:** If a vasculitis involves the **upper airway** (sinuses/ears), think GPA (C-ANCA). If it involves **asthma/eosinophilia**, think EGPA (P-ANCA). * **Treatment:** Induction therapy usually involves Corticosteroids plus Cyclophosphamide or Rituximab.

Q: All of the following are suggestive of Behcet's disease except?

Livedo reticularis. Behcet’s Disease is a chronic, multisystem relapsing inflammatory disorder classified as a variable-vessel vasculitis. The diagnosis is primarily clinical, based on the International Study Group criteria. ### Explanation of the Correct Answer **D. Livedo reticularis:** This is a lace-like, purplish skin discoloration caused by swelling of venules or obstruction of capillaries. It is a hallmark of conditions like **Systemic Lupus Erythematosus (SLE), Antiphospholipid Syndrome (APS), and Polyarteritis Nodosa (PAN)** [1]. While Behcet’s involves skin lesions, they typically manifest as **Erythema nodosum** (on the lower limbs) or acneiform/pseudofolliculitis eruptions, rather than livedo reticularis. ### Analysis of Incorrect Options * **A. Orogenital ulcers:** These are the hallmark of Behcet’s. Oral aphthous ulcers are usually the first symptom (present in >95%), while genital ulcers are more specific and often lead to scarring. * **B. Polyarthritis:** Approximately 50% of patients develop a non-erosive, asymmetric inflammatory arthritis or arthralgia, most commonly affecting large joints like the knees and ankles. * **C. Uveitis:** Ocular involvement occurs in about 70% of patients. **Bilateral posterior uveitis** is a serious complication that can lead to blindness. Panuveitis and retinal vasculitis are also common. ### NEET-PG Clinical Pearls * **Pathergy Test:** A highly specific (but less sensitive) diagnostic test where a sterile needle prick results in a pustule or papule within 24–48 hours. * **HLA Association:** Strongly associated with **HLA-B51**. * **Vascular Involvement:** Unique among vasculitides for causing both **arterial aneurysms** (e.g., pulmonary artery aneurysms) and **venous thrombosis** (Budd-Chiari syndrome). * **Neurological (Neuro-Behcet’s):** Can present as parenchymal CNS lesions or dural sinus thrombosis.

Q: Non-erosive arthritis is seen in which of the following conditions?

Systemic lupus erythematosus. **Explanation:** The hallmark of arthritis in **Systemic Lupus Erythematosus (SLE)** is that it is typically **non-erosive** [1]. While SLE patients frequently present with symmetrical polyarthritis (often involving small joints of the hands), the underlying pathology involves synovial inflammation without the destruction of bone or cartilage. A classic manifestation is **Jaccoud’s arthropathy**, where reversible deformities (like ulnar deviation or swan-neck) occur due to ligamentous laxity rather than joint surface erosion. On X-ray, the joint spaces remain preserved. **Analysis of Incorrect Options:** * **Ankylosing Spondylitis:** This is a seronegative spondyloarthropathy characterized by **erosive changes**, particularly at the sacroiliac joints (sacroiliitis) [1], eventually leading to bony ankylosis (fusion) and the "Bamboo spine" appearance. * **Rheumatic Fever:** While the arthritis in Rheumatic Fever is "migratory" and leaves no permanent damage, it is typically an acute, transient febrile polyarthritis. However, in the context of chronic connective tissue diseases and NEET-PG patterns, SLE is the classic prototype for "non-erosive" chronic arthritis. * **Juvenile Idiopathic Arthritis (JIA):** Most subtypes of JIA (especially polyarticular and systemic) are associated with **joint destruction and erosions** if not managed aggressively [2], leading to growth disturbances and permanent joint damage. **High-Yield Clinical Pearls for NEET-PG:** * **Jaccoud’s Arthropathy:** Reversible joint deformities in SLE (non-erosive) vs. **Rheumatoid Arthritis** (irreversible, erosive). * **Rhupus Syndrome:** A rare overlap of SLE and Rheumatoid Arthritis where erosive changes *can* occur. * **Most common joint involved in SLE:** Proximal Interphalangeal (PIP) joints and Metacarpophalangeal (MCP) joints. * **Radiology Tip:** If a question mentions "deformity without erosions," always think SLE.

Question 1

An 18-year-old boy presents with digital gangrene of the third and fourth fingers over the last 2 weeks. On examination, the blood pressure is 170/110 mm of Hg, and all peripheral pulses were palpable. Blood and urine examinations were unremarkable. ANA, Anti-dsDNA, and ANCA were negative. Which of the following represents the most likely diagnosis?

Accepted Answer

Polyarteritis nodosa

Answer

Wegener's granulomatosis

Answer

Takayasu's arteritis

Answer

Systemic lupus erythematosus (SLE)

Question 2

A 13-year-old female child presented with recurrent sinusitis, fever, arthralgia, respiratory distress, hematuria, and hypertension. Renal biopsy showed necrotizing granuloma. The anti-proteinase-3 ANCA was positive. What is the most likely diagnosis?

Accepted Answer

Wegener's granulomatosis

Answer

Polyarteritis Nodosa

Answer

Microscopic polyangiitis

Answer

Churg-Strauss syndrome

Question 3

What is a characteristic feature of Still's disease?

Accepted Answer

Rashes

Answer

Prominent kidney involvement

Answer

Positive Rheumatoid factor

Answer

Neutropenia

Question 4

All of the following are found in Primary Sjogren's syndrome except?

Accepted Answer

Connective tissue disorder

Answer

Lymphoma

Answer

Xerostomia

Answer

Xerophthalmia

Question 5

Which of the following statements is true about Rheumatoid arthritis?

Accepted Answer

Associated with adrenal hyperplasia

Answer

Involves peripheral joints more commonly than axial joints

Answer

Does not decrease life expectancy

Answer

Associated with vasculitis

Question 6

A 67-year-old man with lung cancer presents with progressive weakness in his arms and legs. He reports difficulty rising from a chair and climbing stairs, with no associated pain or discomfort. Physical examination reveals proximal muscle strength of four out of five in both upper and lower limbs. His reflexes, muscle tone, and sensation are normal. He also has a heliotrope rash on his eyelids and Gottron's papules on his knuckles. Based on these findings, what is the most likely anatomic site of the disorder causing his muscle weakness?

Accepted Answer

Muscle

Answer

Anterior horn cell

Answer

Peripheral nerve

Answer

Neuromuscular junction

Question 7

A 50-year-old female presented with mild to moderate unilateral headache which becomes severe occasionally along with fever, jaw pain and visual disturbances, malaise, fatigue and weight loss. On examination, scalp tenderness was noted. Lab findings revealed normocytic normochromic anemia, elevated ESR and raised ANA titers. A biopsy was taken from the temporal artery. Which of the following cells are most importantly involved in the pathogenesis of the above disease?

Accepted Answer

CD4 T cells

Answer

CD8 T cells

Answer

B cells

Answer

Dendritic cells

Question 8

Which of the following conditions is associated with a positive C-ANCA?

Accepted Answer

Wegener's granulomatosis (WG)

Answer

Takayasu arteritis (TA)

Answer

Polyarteritis nodosa (Classic PAN)

Answer

Giant cell arteritis (GCA)

Question 9

All of the following are suggestive of Behcet's disease except?

Accepted Answer

Livedo reticularis

Answer

Orogenital ulcers

Answer

Polyarthritis

Answer

Uveitis

Question 10

Non-erosive arthritis is seen in which of the following conditions?

Accepted Answer

Systemic lupus erythematosus

Answer

Ankylosing spondylitis

Answer

Rheumatic fever

Answer

Juvenile Idiopathic Arthritis

Rheumatology and Immunology — MCQs

Rheumatology and Immunology — MCQs

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