Rheumatology and Immunology Practice Questions

Q: CREST syndrome is an autoimmune condition associated with atrophy and fibrosis of the oesophageal musculature, resulting in dysphagia and reflux-type symptoms. Which of the following is NOT a feature of CREST syndrome?

Erythematous malar rash. CREST syndrome is a **limited cutaneous form of Systemic Sclerosis (SSc)**. The diagnosis is clinical and defined by its eponymous acronym. [1] **1. Why "Erythematous malar rash" is the correct answer:** An erythematous malar (butterfly) rash is the hallmark clinical feature of **Systemic Lupus Erythematosus (SLE)**, not Systemic Sclerosis. While both are connective tissue diseases, they have distinct cutaneous manifestations. In CREST, skin involvement is typically limited to the face and distal extremities (distal to elbows/knees). **2. Analysis of incorrect options (Features of CREST):** * **C - Calcinosis cutis:** Soft tissue calcifications, often seen as painful nodules on fingertips or pressure points (Option D). * **R - Raynaud’s phenomenon:** Episodic vasospasm of digits in response to cold or stress; usually the earliest manifestation (Option A). * **E - Esophageal dysmotility:** Due to atrophy and fibrosis of the lower two-thirds of the esophageal smooth muscle. * **S - Sclerodactyly:** Thickening and tightening of the skin of the fingers or toes, giving them a "sausage-like" appearance (Option C). [1] * **T - Telangiectasia:** Dilated capillaries on the face, mucous membranes, and palms. **Clinical Pearls for NEET-PG:** * **Serology:** CREST syndrome is highly associated with **Anti-centromere antibodies** (highly specific). In contrast, Diffuse SSc is associated with **Anti-Scl-70 (topoisomerase I)** antibodies. * **Prognosis:** Limited SSc (CREST) generally has a better prognosis than diffuse SSc but carries a significant risk of **Pulmonary Arterial Hypertension (PAH)** later in the disease course. * **Organ Involvement:** Unlike diffuse SSc, CREST rarely involves the kidneys (Scleroderma Renal Crisis) or causes early interstitial lung disease.

Q: A 25-year-old woman complains of low-grade fever, fatigue, and a persistent rash over her nose and upper chest. She also notes pain in her knees and elbows. A skin biopsy shows dermal inflammation and granular deposits of IgG and C3 complement along the basement membrane at the epidermal/dermal junction. Urinalysis reveals microscopic hematuria and proteinuria. The ANA is positive. What is commonly associated with the development of thromboembolic complications in this patient?

Phospholipids. ### Explanation **Correct Answer: D. Phospholipids** **Reasoning:** The clinical presentation—malar rash, photosensitivity, polyarthritis, hematuria/proteinuria (lupus nephritis), and a positive ANA—is classic for **Systemic Lupus Erythematosus (SLE)** [1]. The biopsy finding of granular IgG and C3 at the dermo-epidermal junction is known as the **Lupus Band Test**. In SLE patients, thromboembolic complications (both arterial and venous) are most commonly associated with **Antiphospholipid Syndrome (APS)**. This occurs due to the presence of **antiphospholipid antibodies** (e.g., Lupus Anticoagulant, Anti-cardiolipin, and Anti-̢-glycoprotein I). These antibodies target **phospholipid-binding proteins**, leading to a prothrombotic state, recurrent miscarriages, and thrombocytopenia. **Incorrect Options:** * **A. ABO blood group antigens:** While certain blood groups (like Type A) have a slightly higher risk of general VTE, they are not the specific driver of thrombosis in the context of SLE. * **B. Class II HLA molecules:** HLA-DR2 and DR3 are strongly associated with the *genetic predisposition* to SLE, but they do not directly mediate the coagulation cascade or thromboembolic events. * **C. Clotting factors:** While deficiencies in Protein C or S cause thrombosis, the specific association with SLE-related hypercoagulability is the presence of autoantibodies against phospholipids, not a primary deficiency of clotting factors. **High-Yield Clinical Pearls for NEET-PG:** * **Lupus Band Test:** Positive in both involved and uninvolved skin in SLE, but only in involved skin in Discoid Lupus (DLE). * **APS Paradox:** Patients with Lupus Anticoagulant have a **prolonged aPTT** *in vitro* (due to interference with phospholipids in the assay) but a **prothrombotic state** *in vivo*. * **Libman-Sacks Endocarditis:** Non-bacterial verrucous vegetations on both sides of heart valves, also associated with APS in SLE patients.

Q: Which of the following antibodies is most frequently seen in Antiphospholipid Syndrome?

Anti- beta 2 glycoprotein antibody. **Explanation:** Antiphospholipid Syndrome (APS) is an autoimmune prothrombotic state characterized by arterial or venous thrombosis and pregnancy complications. The diagnosis relies on the presence of at least one clinical criterion and one laboratory criterion. **Why Option D is Correct:** The laboratory criteria for APS include three specific antibodies: **Lupus Anticoagulant (LA)**, **Anti-cardiolipin (aCL) antibodies**, and **Anti-beta 2 glycoprotein I (̢2-GPI) antibodies**. Among these, Anti-̢2 glycoprotein I is considered the most specific and is frequently seen in patients with APS. It targets the ̢2-GPI protein, which normally binds to anionic phospholipids, playing a crucial role in the pathogenic clotting cascade of the syndrome. **Why Other Options are Incorrect:** * **Option A:** **Beta 2 microglobulin** is a component of MHC Class I molecules. It is used as a tumor marker (e.g., Multiple Myeloma) or a marker of renal function, but it is not an antibody associated with APS. * **Option B:** **Anti-nuclear antibody (ANA)** is the screening test for Systemic Lupus Erythematosus (SLE) [1]. While APS can occur secondary to SLE, ANA is not specific to APS itself [1], [2]. * **Option C:** **Anti-centromere antibody** is highly specific for **Limited Cutaneous Systemic Sclerosis (CREST syndrome)**, not APS. **High-Yield Clinical Pearls for NEET-PG:** * **The "Lupus Anticoagulant" Paradox:** Despite its name, LA is *prothrombotic* in vivo but causes a *prolonged aPTT* in vitro (which does not correct with a mixing study). * **False Positive VDRL:** Patients with APS often show a false positive syphilis test (VDRL/RPR) because the reagin antibodies cross-react with the cardiolipin used in the test. * **Catastrophic APS (Asherson’s Syndrome):** A rare, life-threatening form involving small-vessel thrombosis in at least three organ systems simultaneously.

Q: What is the most commonly affected organ in polyarteritis nodosa?

Kidneys. **Explanation:** **Polyarteritis Nodosa (PAN)** is a systemic necrotizing vasculitis that typically affects small and medium-sized muscular arteries [1]. **1. Why Kidneys are the Correct Answer:** The **kidneys** are the most frequently involved organ in PAN (occurring in 70–80% of cases) [1]. The pathology involves necrotizing inflammation of the renal arteries, leading to microaneurysms (the classic "string of beads" appearance on angiography) and renal ischemia [1]. This typically manifests as **renovascular hypertension** or renal insufficiency. Importantly, unlike many other vasculitides, PAN is a non-glomerular disease; it affects the vessels, not the glomeruli themselves [2]. **2. Why Other Options are Incorrect:** * **Lungs (A):** This is a high-yield "negative" fact. PAN characteristically **spares the lungs**. If a patient presents with systemic vasculitis and pulmonary involvement (like nodules or hemorrhage), clinicians should suspect Granulomatosis with Polyangiitis (GPA) or Eosinophilic Granulomatosis with Polyangiitis (EGPA) instead. * **Pancreas (C) and Spleen (D):** While PAN can affect any organ (including the GI tract and gallbladder), involvement of the pancreas and spleen is significantly less common than the kidneys, skin, or peripheral nerves. **Clinical Pearls for NEET-PG:** * **Association:** Strongly associated with **Hepatitis B virus (HBV)** infection (HBsAg positivity in ~10-30% of cases). * **ANCA Status:** PAN is typically **ANCA-negative**. * **Clinical Triad:** Often presents with fever, abdominal pain (mesenteric ischemia), and mononeuritis multiplex (foot/wrist drop) [1]. * **Diagnosis:** Confirmed via tissue biopsy or visceral angiography showing microaneurysms.

Q: Thymomas may be associated with all of the following, except?

Hypergammaglobulinemia. ### Explanation Thymomas are epithelial neoplasms of the thymus gland and are notorious for their association with various paraneoplastic autoimmune syndromes. **Why Option B is the Correct Answer:** Thymomas are characteristically associated with **Hypogammaglobulinemia** (specifically **Good’s Syndrome**), not hypergammaglobulinemia. Good’s syndrome is a rare immunodeficiency triad consisting of thymoma, hypogammaglobulinemia, and low or absent B-cells. This leads to an increased susceptibility to bacterial, viral, and fungal infections. **Analysis of Other Options:** * **A. Myasthenia Gravis:** This is the most common association. Approximately 30–45% of patients with thymoma have Myasthenia Gravis (MG), and conversely, about 10–15% of MG patients have a thymoma [1], [2]. Screening for internal thymomas via thoracic CT is standard in MG patients [1]. * **C. Panhypopituitarism:** While rare, thymomas have been documented in association with various endocrine disorders and multi-organ autoimmunity, including cases of lymphocytic hypophysitis leading to panhypopituitarism. * **D. Systemic Lupus Erythematosus (SLE):** Thymomas are associated with a wide spectrum of autoimmune connective tissue diseases, including SLE, polymyositis, and rheumatoid arthritis, due to the failure of central tolerance and T-cell maturation within the neoplastic thymus. **High-Yield Clinical Pearls for NEET-PG:** 1. **Good’s Syndrome:** Remember the triad: Thymoma + Hypogammaglobulinemia + B-cell deficiency. 2. **Pure Red Cell Aplasia (PRCA):** About 5–10% of thymoma patients develop PRCA; it is a classic "favorite" association in exams. 3. **Imaging:** Contrast-enhanced CT (CECT) of the chest is the gold standard for evaluating an anterior mediastinal mass [1]. 4. **Surgical Management:** Thymectomy is indicated for thymomas, but it does not always guarantee the resolution of associated autoimmune symptoms (like MG).

Q: Patients with antiphospholipid antibodies may manifest with any of the following, except:

Thrombocytosis. **Explanation:** Antiphospholipid Syndrome (APS) is an autoimmune prothrombotic state characterized by the presence of antiphospholipid antibodies (aPL) such as Lupus Anticoagulant, Anti-cardiolipin, and Anti-̢2-glycoprotein I. **Why Thrombocytosis is the Correct Answer:** In APS, the hallmark hematological finding is **Thrombocytopenia** (low platelet count) [2], not thrombocytosis. This occurs because the antibodies bind to platelets, leading to their activation, sequestration, and subsequent destruction. Therefore, thrombocytosis is not a manifestation of this condition. **Analysis of Incorrect Options:** * **Venous Thrombosis (A):** This is the most common clinical manifestation of APS. It typically presents as Deep Vein Thrombosis (DVT) or Pulmonary Embolism. * **Arterial Thrombosis (B):** APS is unique because it causes both venous and arterial clots. Arterial involvement often manifests as a Stroke or Transient Ischemic Attack (TIA) in young patients. * **Hemolytic Anemia (C):** Autoimmune hemolytic anemia (AIHA) is a recognized secondary feature of APS, often associated with Evans Syndrome (AIHA + Thrombocytopenia). **High-Yield Clinical Pearls for NEET-PG:** * **Obstetric Manifestations:** Recurrent pregnancy loss (usually >10 weeks), premature births due to eclampsia, or placental insufficiency. * **Laboratory Paradox:** APS causes a **prolonged aPTT** *in vitro* (due to interference with phospholipids in the test), but causes **thrombosis** *in vivo*. * **Libman-Sacks Endocarditis:** Non-bacterial verrucous vegetations on heart valves can be seen in APS/SLE. * **Livedo Reticularis:** A lace-like purplish skin discoloration is a common cutaneous sign [1].

Q: HLA B27 is positive in which of the following conditions?

Ankylosing spondylitis. **Explanation:** **HLA-B27** is a Class I surface antigen encoded by the B locus in the Major Histocompatibility Complex (MHC). It is most strongly associated with **Ankylosing Spondylitis (AS)**, a chronic inflammatory disease of the axial skeleton. Approximately **90-95%** of patients with AS are HLA-B27 positive, making it a hallmark diagnostic marker for the Seronegative Spondyloarthropathies [1]. **Analysis of Options:** * **A. Ankylosing Spondylitis (Correct):** The strong association is linked to the "molecular mimicry" theory, where immune responses against certain bacteria (like *Klebsiella*) cross-react with HLA-B27 molecules in the joints. * **B. Rheumatoid Arthritis:** This is primarily associated with **HLA-DR4** (specifically the "shared epitope"). It is characterized by symmetric small joint involvement and positive Rheumatoid Factor/anti-CCP. * **C. Systemic Lupus Erythematosus (SLE):** SLE is strongly linked to **HLA-DR2 and HLA-DR3**. It is a multi-system autoimmune disease characterized by ANA and anti-dsDNA antibodies. * **D. Behcet Syndrome:** This multi-system inflammatory disorder (characterized by oral/genital ulcers and uveitis) is most strongly associated with **HLA-B51**. **High-Yield Facts for NEET-PG:** * **Mnemonic for HLA-B27 (PAIR):** **P**soriatic arthritis, **A**nkylosing spondylitis, **I**nflammatory bowel disease-associated arthritis, and **R**eactive arthritis (formerly Reiter’s) [1]. * **Radiology Sign:** Look for the **"Bamboo Spine"** on X-ray due to marginal syndesmophytes and sacroiliitis. * **Clinical Test:** The **Schober’s test** is used to assess restricted lumbar forward flexion in AS patients. * **Extra-articular manifestation:** Acute anterior uveitis is the most common non-skeletal feature.

Q: A 40-year-old male presents with an upper respiratory tract infection (URTI), hemoptysis, and an elevated c-ANCA value. What is the most likely diagnosis?

Wegener's granulomatosis. The clinical triad of **upper respiratory tract involvement** (sinusitis, otitis media, or saddle nose deformity), **lower respiratory tract involvement** (hemoptysis, nodules), and **renal involvement** (glomerulonephritis) is classic for **Wegener’s Granulomatosis** (now known as Granulomatosis with Polyangiitis or GPA) [1]. The presence of **c-ANCA** (anti-proteinase 3 antibodies) is highly specific (approx. 90%) for this condition, making it the most likely diagnosis. **Analysis of Incorrect Options:** * **Churg-Strauss Syndrome (EGPA):** Characterized by asthma, peripheral eosinophilia, and **p-ANCA** (anti-MPO) [2]. It typically lacks the destructive upper airway lesions seen in GPA. * **Microscopic Polyangiitis (MPA):** A small-vessel vasculitis that involves the lungs and kidneys but characteristically **spares the upper respiratory tract** [1]. It is most commonly associated with **p-ANCA**. * **Goodpasture Syndrome:** Presents with a pulmonary-renal syndrome (hemoptysis and hematuria) but is caused by **anti-GBM antibodies**, not ANCA [1]. It does not involve the upper respiratory tract (sinuses/ears). **NEET-PG High-Yield Pearls:** * **GPA Triad:** Upper Respiratory + Lower Respiratory + Kidneys. * **Serology:** c-ANCA (PR3) is the marker for GPA; p-ANCA (MPO) is for MPA and EGPA [2]. * **Biopsy Gold Standard:** Shows necrotizing granulomatous inflammation [1]. * **Treatment:** Induction with Corticosteroids + Cyclophosphamide (or Rituximab) [2]. * **Radiology:** GPA often shows "cavitary nodules" on a chest X-ray [1].

Q: What is the typical sequence of color changes observed in Raynaud's disease?

White, blue, red. **Explanation:** Raynaud’s phenomenon is a reversible vasospastic disorder of the digital arteries, typically triggered by cold exposure or emotional stress. The classic "triphasic" color change follows a specific physiological sequence: 1. **White (Pallor):** This is the initial phase caused by intense **vasoconstriction** of the precapillary arterioles, leading to a lack of blood flow (ischemia) to the digits. 2. **Blue (Cyanosis):** As the ischemia persists, the stagnant blood in the capillaries and venules becomes deoxygenated, resulting in a bluish discoloration. 3. **Red (Rubor):** Once the stimulus is removed, the vasospasm resolves, leading to **reactive hyperemia** (sudden reperfusion). The rapid return of oxygenated blood causes the digits to turn bright red, often accompanied by throbbing or pain. **Analysis of Incorrect Options:** * **Options A, C, and D:** These sequences are incorrect because they do not follow the physiological progression from ischemia (white) to deoxygenation (blue) and finally to reperfusion (red). **NEET-PG High-Yield Pearls:** * **Primary Raynaud’s (Disease):** Usually idiopathic, symmetric, and lacks underlying tissue damage (gangrene). * **Secondary Raynaud’s (Phenomenon):** Often associated with connective tissue diseases, most commonly **Systemic Sclerosis (Scleroderma)**. * **Drug of Choice:** Calcium Channel Blockers (specifically **Nifedipine**) are the first-line treatment. * **Capillaroscopy:** Used to differentiate primary from secondary; "giant capillaries" or "dropout areas" suggest an underlying systemic disease.

Question 1

CREST syndrome is an autoimmune condition associated with atrophy and fibrosis of the oesophageal musculature, resulting in dysphagia and reflux-type symptoms. Which of the following is NOT a feature of CREST syndrome?

Accepted Answer

Erythematous malar rash

Answer

Raynaud's phenomenon

Answer

Sclerodactyly

Answer

Soft tissue calcification

Question 2

A 25-year-old woman complains of low-grade fever, fatigue, and a persistent rash over her nose and upper chest. She also notes pain in her knees and elbows. A skin biopsy shows dermal inflammation and granular deposits of IgG and C3 complement along the basement membrane at the epidermal/dermal junction. Urinalysis reveals microscopic hematuria and proteinuria. The ANA is positive. What is commonly associated with the development of thromboembolic complications in this patient?

Accepted Answer

Phospholipids

Answer

ABO blood group antigens

Answer

Class II HLA molecules

Answer

Clotting factors

Question 3

Which of the following antibodies is most frequently seen in Antiphospholipid Syndrome?

Accepted Answer

Anti- beta 2 glycoprotein antibody

Answer

Beta 2 microglobulin antibody

Answer

Anti-nuclear antibody

Answer

Anti-centromere antibody

Question 4

What is the most commonly affected organ in polyarteritis nodosa?

Accepted Answer

Kidneys

Answer

Lungs

Answer

Pancreas

Answer

Spleen

Question 5

Which antibody is found in myositis?

Accepted Answer

Anti-Jo-1

Answer

Anti-Scl-70

Answer

Anti-Sm

Answer

Anti-Ku

Question 6

Thymomas may be associated with all of the following, except?

Accepted Answer

Hypergammaglobulinemia

Answer

Myasthenia Gravis

Answer

Panhypopituitarism

Answer

Systemic Lupus Erythematosus (SLE)

Question 7

Patients with antiphospholipid antibodies may manifest with any of the following, except:

Accepted Answer

Thrombocytosis

Answer

Venous thrombosis

Answer

Arterial thrombosis

Answer

Hemolytic anemia

Question 8

HLA B27 is positive in which of the following conditions?

Accepted Answer

Ankylosing spondylitis

Answer

Rheumatoid arthritis

Answer

Systemic lupus erythematosus

Answer

Behcet syndrome

Question 9

A 40-year-old male presents with an upper respiratory tract infection (URTI), hemoptysis, and an elevated c-ANCA value. What is the most likely diagnosis?

Accepted Answer

Wegener's granulomatosis

Answer

Churg-Strauss syndrome

Answer

Microscopic polyangiitis

Answer

Goodpasture syndrome

Question 10

What is the typical sequence of color changes observed in Raynaud's disease?

Accepted Answer

White, blue, red

Answer

Red, blue, white

Answer

Blue, red, white

Answer

White, red, blue

Rheumatology and Immunology — MCQs

Rheumatology and Immunology — MCQs

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