Rheumatology and Immunology Practice Questions

Q: Scleroderma is characterized by which of the following?

All of the above. **Explanation:** Scleroderma (Systemic Sclerosis) is a multisystem autoimmune disorder characterized by excessive collagen deposition leading to fibrosis of the skin and internal organs, alongside significant microvascular injury [1]. **Why "All of the Above" is correct:** * **Raynaud’s Phenomenon (Option C):** This is the most common initial manifestation (seen in >95% of patients). It results from episodic vasospasm of digital arteries in response to cold or stress. In scleroderma, it is "secondary Raynaud’s," often leading to digital ulcers due to structural vascular damage [1]. * **Oral and Periodontal Involvement (Option A):** Fibrosis affects the perioral tissues, leading to **microstomia** (limited mouth opening) and a "purse-string" appearance of the mouth. Radiographically, a classic high-yield finding is the **uniform thickening of the periodontal ligament (PDL) space**, even in the absence of tooth mobility. * **Palmar Keratosis (Option B):** While less discussed than sclerodactyly, skin thickening (sclerosis) can manifest as hyperkeratosis and induration of the palms [1]. The skin becomes tight, shiny, and loses its normal creases. **Clinical Pearls for NEET-PG:** 1. **Antibody Associations:** * **Limited Scleroderma (CREST):** Anti-Centromere antibodies (highly specific). * **Diffuse Scleroderma:** Anti-Scl-70 (Anti-topoisomerase I) and Anti-RNA polymerase III (associated with Scleroderma Renal Crisis). 2. **Gastrointestinal:** The most common site involved is the **Esophagus** (lower 2/3rd dysmotility), leading to GERD and "Watermelon Stomach" (GAVE). 3. **Pulmonary:** Interstitial Lung Disease (ILD) is the leading cause of mortality in diffuse disease, while Pulmonary Arterial Hypertension (PAH) is more common in limited disease. 4. **Diagnosis:** Look for "Salt and Pepper" pigmentation and telangiectasias on clinical vignettes [1].

Q: Gottron sign is seen in which of the following conditions?

Dermatomyositis. **Explanation:** **Gottron sign** is a pathognomonic cutaneous manifestation of **Dermatomyositis (DM)**. It is characterized by symmetric, erythematous to violaceous scales or macules overlying the dorsal aspect of the interphalangeal (IP) and metacarpophalangeal (MCP) joints [1]. This occurs due to underlying microvascular damage and inflammation specific to the pathophysiology of DM. **Why the other options are incorrect:** * **Polymyositis (PM):** While PM shares similar proximal muscle weakness with DM [2], it is strictly an inflammatory myopathy **without** the characteristic skin involvement. If a patient has the clinical features of PM plus Gottron sign, the diagnosis automatically shifts to Dermatomyositis [2]. * **Sjogren’s Syndrome:** This is primarily characterized by sicca symptoms (dry eyes/mouth). While it can overlap with other connective tissue diseases, Gottron sign is not a feature. * **Systemic Lupus Erythematosus (SLE):** SLE presents with a malar rash that typically **spares the nasolabial folds**. On the hands, SLE rashes usually occur **between the joints** (interarticular), whereas Gottron sign specifically occurs **over the joints**. **High-Yield Clinical Pearls for NEET-PG:** * **Gottron Papules:** Raised, lichenoid papules over the knuckles (more specific than the "sign") [1]. * **Heliotrope Rash:** Violaceous eruption on the upper eyelids with periorbital edema [1]. * **Shawl Sign/V-sign:** Erythema over the upper back/shoulders or anterior chest [1]. * **Mechanic’s Hands:** Hyperkeratotic, cracked skin on the lateral fingers (associated with **Anti-Jo-1** antibodies and Interstitial Lung Disease) [2]. * **Malignancy:** Dermatomyositis has a strong association with internal malignancies (e.g., ovarian, lung, gastric); age-appropriate cancer screening is mandatory upon diagnosis [1].

Q: Which condition is specifically associated with anti-ds DNA antibodies?

Systemic Lupus Erythematosus (SLE). **Explanation:** **Systemic Lupus Erythematosus (SLE)** is the correct answer because anti-double-stranded DNA (anti-dsDNA) antibodies are highly specific (approx. 95–97%) for this condition. While Antinuclear Antibody (ANA) is the best screening test due to its high sensitivity, anti-dsDNA is a **confirmatory marker** [1]. Clinically, anti-dsDNA levels correlate with **disease activity**, particularly the development and severity of **Lupus Nephritis**. **Analysis of Incorrect Options:** * **Systemic Sclerosis (Diffuse):** Characteristically associated with **anti-Scl-70 (anti-topoisomerase I)** antibodies. * **CREST Syndrome (Limited Scleroderma):** Strongly associated with **anti-centromere** antibodies. * **Sjogren’s Syndrome:** Characterized by **anti-Ro (SS-A)** and **anti-La (SS-B)** antibodies [1]. **High-Yield Clinical Pearls for NEET-PG:** * **Most Specific Antibody for SLE:** Anti-Smith (anti-Sm) antibody (does not fluctuate with disease activity) [1]. * **Most Sensitive Antibody for SLE:** ANA (best initial screening test) [1]. * **Drug-Induced Lupus:** Associated with **anti-histone** antibodies; anti-dsDNA is typically absent. * **Neonatal Lupus/Congenital Heart Block:** Associated with maternal **anti-Ro/SSA** antibodies. * **Monitoring:** Anti-dsDNA and **complement levels (C3, C4)** are the two primary laboratory markers used to monitor SLE flares and renal involvement [1].

Q: A 34-year-old woman presents with a red rash over her cheeks, frequent oral ulcers, and pain and swelling in both wrists and small joints of her hands. Laboratory investigations reveal a positive ANA and 3+ proteinuria. Which of the following organ involvement will cause the most symptoms during the course of this disease?

Musculoskeletal pathology. ### Explanation The clinical presentation (malar rash, oral ulcers, inflammatory arthritis, and proteinuria) in a young female is diagnostic of **Systemic Lupus Erythematosus (SLE)**. [1] **1. Why Musculoskeletal pathology is correct:** While renal involvement is the most significant predictor of mortality and morbidity, **musculoskeletal involvement** is the **most common clinical manifestation** of SLE, affecting over 90% of patients. [2] It is typically the earliest symptom and causes the most frequent symptoms (pain, stiffness, and swelling) throughout the disease course. The arthritis in SLE is characteristically inflammatory, symmetrical, and non-erosive (unlike Rheumatoid Arthritis). [4] **2. Why other options are incorrect:** * **Renal pathology:** Although 3+ proteinuria indicates Lupus Nephritis (a major cause of morbidity), it is often asymptomatic in early stages. Only about 50% of patients develop clinically significant renal disease. * **Cardiopulmonary pathology:** While pleuritis and pericarditis are common, they occur less frequently than joint involvement. [3] * **Thrombotic events:** These are associated with secondary Antiphospholipid Syndrome (APS). While life-threatening, they occur in a minority of SLE patients (approx. 30% have APL antibodies, but fewer have clinical events). **Clinical Pearls for NEET-PG:** * **Most common symptom/initial presentation:** Arthritis/Arthralgia (>90%). * **Most common cardiac manifestation:** Pericarditis. * **Most common pulmonary manifestation:** Pleuritis. * **Jaccoud’s Arthropathy:** A reversible, non-erosive deformity seen in SLE due to ligamentous laxity. * **Best Screening Test:** ANA (High sensitivity). [3] * **Most Specific Test:** Anti-dsDNA and Anti-Smith (Anti-dsDNA levels also correlate with disease activity/nephritis). [3]

Q: Anti-nuclear antibodies are not found in which of the following conditions?

Sarcoidosis. The presence of **Anti-nuclear antibodies (ANA)** is a hallmark of systemic autoimmune rheumatic diseases (SARDs). ANA serves as a screening tool for conditions where the immune system loses tolerance to self-antigens within the cell nucleus [4]. **Why Sarcoidosis is the Correct Answer:** Sarcoidosis is a multisystem inflammatory disease characterized by the formation of **non-caseating granulomas**. Its pathogenesis is primarily driven by a **Type IV (cell-mediated) hypersensitivity** reaction involving T-lymphocytes and macrophages [2], rather than a B-cell mediated autoantibody response. Therefore, ANA is typically negative in Sarcoidosis. **Analysis of Incorrect Options:** * **Systemic Lupus Erythematosus (SLE):** ANA is the best initial screening test for SLE, with a sensitivity of **>95-99%** [1]. A negative ANA virtually rules out SLE (high negative predictive value). * **Drug-induced Lupus (DILE):** ANA is positive in nearly 100% of cases. The classic marker is **Anti-histone antibodies** (found in >90% of DILE cases). * **Diffuse Scleroderma (Systemic Sclerosis):** ANA is positive in approximately 95% of patients [1]. Specific markers include **Anti-Scl-70 (anti-topoisomerase I)** for diffuse disease and **Anti-centromere** for limited disease (CREST syndrome). **High-Yield Clinical Pearls for NEET-PG:** 1. **ANA Pattern:** A "Homogeneous" pattern is common in SLE, while a "Nucleolar" pattern is highly suggestive of Systemic Sclerosis. 2. **Most Specific Test for SLE:** Anti-dsDNA and Anti-Smith (Anti-Sm) antibodies [1]. 3. **Sarcoidosis Markers:** Elevated **Serum ACE levels**, hypercalciuria/hypercalcemia, and bilateral hilar lymphadenopathy on CXR (Stage I) [3]. 4. **ANA-Negative SLE:** Extremely rare; historically associated with Anti-Ro (SS-A) antibodies [1].

Q: C-ANCA is associated with which of the following conditions?

Wegener's granulomatosis. Explanation: **C-ANCA (Cytoplasmic Antineutrophil Cytoplasmic Antibody)** is highly specific for **Wegener’s Granulomatosis** (now known as Granulomatosis with Polyangiitis or GPA). The target antigen for C-ANCA is **Proteinase-3 (PR3)**. In GPA, the classic clinical triad involves the upper respiratory tract (sinusitis, saddle nose deformity), lower respiratory tract (hemoptysis, cavitary nodules), and kidneys (pauci-immune glomerulonephritis). **Analysis of Options:** * **Microscopic Polyangiitis (MPA):** This is primarily associated with **P-ANCA** (Perinuclear-ANCA), which targets the enzyme **Myeloperoxidase (MPO)**. Unlike GPA, MPA lacks granulomatous inflammation. * **Churg-Strauss Syndrome (EGPA):** This condition is also associated with **P-ANCA** (MPO-ANCA) in about 40-50% of cases [1]. It is characterized by asthma, peripheral eosinophilia, and extravascular granulomas [1]. * **Polyarteritis Nodosa (PAN):** PAN is a medium-vessel vasculitis that is characteristically **ANCA-negative**. It is strongly associated with Hepatitis B infection and involves microaneurysms (seen on angiography). **High-Yield Clinical Pearls for NEET-PG:** 1. **C-ANCA = PR3-ANCA** (Wegener’s/GPA). 2. **P-ANCA = MPO-ANCA** (Microscopic Polyangiitis, Churg-Strauss, Primary Sclerosing Cholangitis, and Ulcerative Colitis) [1]. 3. **Wegener’s Triad:** Sinus + Lung + Kidney. 4. **Drug-induced Lupus** is associated with anti-histone antibodies, while **Drug-induced ANCA vasculitis** (e.g., by Hydralazine or Propylthiouracil) is usually P-ANCA positive. 5. **Negative ANCA** in a systemic vasculitis should immediately make you think of **PAN** or **Kawasaki disease**.

Q: Anti-phospholipid syndrome is characterized by all of the following EXCEPT:

Pancytopenia. Explanation: Anti-phospholipid Syndrome (APS) is an autoimmune prothrombotic state characterized by the presence of antiphospholipid antibodies (aPL) in the setting of venous/arterial thrombosis or pregnancy complications. **Why Pancytopenia is the Correct Answer:** APS is primarily a **thrombotic and obstetric** disorder. While it frequently involves the hematologic system, it typically manifests as **isolated thrombocytopenia** [2] (due to platelet consumption or anti-platelet antibodies) and occasionally autoimmune hemolytic anemia. It does **not** cause bone marrow suppression or leukopenia; therefore, **pancytopenia** is not a characteristic feature of APS. **Analysis of Other Options:** * **Association with SLE:** APS can be primary or secondary. Secondary APS is most commonly associated with **Systemic Lupus Erythematosus (SLE)** [2], occurring in approximately 30-40% of SLE patients. * **Thrombocytopenia and Rash:** Thrombocytopenia is a classic laboratory finding in APS. The characteristic rash associated with APS is **Livedo Reticularis** [1] (a mottled, purplish, net-like pattern), which results from small vessel occlusion [1]. * **Thrombotic Disorders:** This is the hallmark of the disease. APS causes both **venous thrombosis** (most commonly DVT) and **arterial thrombosis** (most commonly Stroke/TIA). **High-Yield Clinical Pearls for NEET-PG:** 1. **Diagnostic Criteria (Sapporo):** Requires at least one clinical criteria (Thrombosis or Pregnancy loss) AND one laboratory criteria (Lupus anticoagulant, Anti-cardiolipin, or Anti-β2-glycoprotein 1 antibodies). 2. **Paradoxical Finding:** APS causes a **prolonged aPTT** *in vitro* (due to interference with phospholipids in the assay), but causes **thrombosis** *in vivo*. 3. **False Positive VDRL:** Patients with APS often have a false-positive syphilis test (VDRL/RPR) because the reagin antibodies cross-react with the cardiolipin used in the test.

Question 1

A 45-year-old female, known case of Irritable Bowel Syndrome, presented with poorly localized pain, especially involving the axial skeleton. The pain was severe, associated with tenderness, and had been occurring for the past 4 months. She also reported fatigue, stiffness, sleep disturbance, cognitive dysfunction, anxiety, and depression. Lab and radiographic studies were normal. Which of the following drugs is NOT approved for the management of this patient's condition?

Accepted Answer

Tetrabenazine

Answer

Cyclobenzaprine

Answer

Duloxetine

Answer

Pregabalin

Question 2

Scleroderma is characterized by which of the following?

Accepted Answer

All of the above

Answer

Tightening of oral mucosa and periodontal involvement

Answer

Multiple palmar keratosis

Answer

Raynaud's phenomenon

Question 3

Gottron sign is seen in which of the following conditions?

Accepted Answer

Dermatomyositis

Answer

Polymyositis

Answer

Polymyositis and Sjogren's syndrome

Answer

Polymyositis and SLE

Question 4

Which condition is specifically associated with anti-ds DNA antibodies?

Accepted Answer

Systemic Lupus Erythematosus (SLE)

Answer

Systemic sclerosis

Answer

CREST syndrome

Answer

Sjogren's syndrome

Question 5

A 34-year-old woman presents with a red rash over her cheeks, frequent oral ulcers, and pain and swelling in both wrists and small joints of her hands. Laboratory investigations reveal a positive ANA and 3+ proteinuria. Which of the following organ involvement will cause the most symptoms during the course of this disease?

Accepted Answer

Musculoskeletal pathology

Answer

Renal pathology

Answer

Cardiopulmonary pathology

Answer

Thrombotic events

Question 6

Anti-neutrophil cytoplasmic antibodies (ANCA) are seen in which of the following conditions?

Accepted Answer

Wegener's Granulomatosis

Answer

Diabetes mellitus

Answer

Rheumatoid arthritis

Answer

Churg-Strauss syndrome

Question 7

Anti-nuclear antibodies are not found in which of the following conditions?

Accepted Answer

Sarcoidosis

Answer

Systemic Lupus Erythematosus (SLE)

Answer

Diffuse Scleroderma

Answer

Drug-induced lupus

Question 8

C-ANCA is associated with which of the following conditions?

Accepted Answer

Wegener's granulomatosis

Answer

Microscopic polyangitis

Answer

Churg Strauss syndrome

Answer

Polyarteritis nodosa (PAN)

Question 9

Anti-phospholipid syndrome is characterized by all of the following EXCEPT:

Accepted Answer

Pancytopenia

Answer

Association with SLE

Answer

Thrombocytopenia and rash

Answer

Thrombotic disorders

Question 10

A 50-year-old male presents with symptoms of cutaneous vasculitis, glomerulonephritis, and synovitis. Which of the following investigations will be helpful in the diagnosis?

Accepted Answer

Anti-HCV antibody

Answer

P-ANCA

Answer

C-ANCA

Answer

Anti-HAV antibody

Rheumatology and Immunology — MCQs

Rheumatology and Immunology — MCQs

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