Rheumatology and Immunology Practice Questions

Q: Thymoma with hypogammaglobulinemia is also known as:

Good's syndrome. **Explanation:** **1. Correct Answer: Good’s Syndrome** Good’s syndrome is a rare secondary immunodeficiency characterized by the triad of **thymoma, hypogammaglobulinemia, and low or absent B-cells**. It typically presents in the 4th or 5th decade of life. The underlying pathophysiology involves a defect in hematopoietic stem cells, leading to reduced humoral and cell-mediated immunity. Patients are highly susceptible to opportunistic infections (fungal, viral, and bacterial) and sinopulmonary infections. Unlike Myasthenia Gravis, surgical removal of the thymoma does not usually reverse the hypogammaglobulinemia. **2. Analysis of Incorrect Options:** * **Capgras Syndrome:** A psychiatric delusional misidentification syndrome where a person believes that a friend, spouse, or close family member has been replaced by an identical-looking impostor. * **Fregoli Syndrome:** Another psychiatric delusion where the patient believes that different people are actually a single person in disguise who is following or persecuting them. * **Todd Syndrome (Alice in Wonderland Syndrome):** A neurological condition characterized by distorted perception (metamorphopsia), where objects or body parts appear much larger (macropsia) or smaller (micropsia) than they are. It is often associated with migraines or epilepsy. **3. High-Yield Clinical Pearls for NEET-PG:** * **Classic Triad:** Thymoma + Hypogammaglobulinemia + Low B-cells. * **Associated Conditions:** Thymoma is most commonly associated with **Myasthenia Gravis** (30-45% of cases) [1], but Good’s syndrome occurs in only about 5% of thymoma patients. * **Immunology:** Look for low serum IgG, IgA, and IgM levels and a reversed CD4/CD8 T-cell ratio. * **Management:** Treatment involves IVIG (Intravenous Immunoglobulin) replacement and management of the thymoma [1].

Q: Which vasculitis is positive for pANCA?

Churg-Strauss syndrome. The correct answer is **Churg-Strauss syndrome** (now known as Eosinophilic Granulomatosis with Polyangiitis or EGPA). **1. Why Churg-Strauss syndrome is correct:** ANCA-associated vasculitides are characterized by the presence of Antineutrophil Cytoplasmic Antibodies. **pANCA** (perinuclear) primarily targets the enzyme **myeloperoxidase (MPO)**. Approximately 40–60% of patients with Churg-Strauss syndrome are pANCA positive [1]. It is clinically defined by the triad of asthma, hypereosinophilia, and necrotizing vasculitis [1]. **2. Why the other options are incorrect:** * **Wegener’s granulomatosis (GPA):** This is classically associated with **cANCA** (cytoplasmic), which targets **Proteinase-3 (PR3)**. While pANCA can rarely occur, cANCA is the hallmark (90% sensitivity in active disease). * **Polyarteritis nodosa (PAN):** PAN is a medium-vessel vasculitis that is characteristically **ANCA-negative**. Its pathogenesis is often linked to Hepatitis B surface antigen, not antineutrophil antibodies. * **All of the above:** Incorrect because PAN is ANCA-negative and Wegener’s is predominantly cANCA-positive. **3. NEET-PG High-Yield Clinical Pearls:** * **pANCA (MPO-ANCA) Positive:** Churg-Strauss syndrome, Microscopic Polyangiitis (MPA), and Primary Sclerosing Cholangitis (PSC). * **cANCA (PR3-ANCA) Positive:** Granulomatosis with Polyangiitis (Wegener’s). * **Rule of Thumb:** If a question mentions "pauci-immune glomerulonephritis" with lung involvement, think of ANCA-associated vasculitis. * **Churg-Strauss Key Feature:** Look for "Asthma" or "Eosinophilia" in the clinical vignette to differentiate it from MPA or Wegener’s [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.

Q: Which of the following statements is FALSE about reactive arthritis?

Migratory polyarthritis. **Explanation:** Reactive arthritis (formerly Reiter’s Syndrome) is an **asymmetric inflammatory oligoarthritis** that develops following an extra-articular infection, typically of the gastrointestinal (e.g., *Salmonella, Shigella, Campylobacter*) or genitourinary tract (e.g., *Chlamydia trachomatis*) [1]. **Why Option D is the correct (False) statement:** Reactive arthritis characteristically presents as an **additive, asymmetric oligoarthritis** (affecting 2–4 joints), primarily involving the large weight-bearing joints of the lower extremities (knees and ankles) [1]. **Migratory polyarthritis** is a classic feature of **Acute Rheumatic Fever** or disseminated gonococcal infection, not reactive arthritis. **Analysis of other options:** * **Option A:** It is most common among young men (aged 20–40), particularly the post-venereal form associated with *Chlamydia* [1]. * **Option B:** There is a strong genetic predisposition; approximately **30–50%** of patients are **HLA-B27 positive** [1]. This association is even stronger in patients with sacroiliitis or uveitis. * **Option C:** The hallmark clinical presentation is a painful, asymmetric oligoarthritis, often accompanied by enthesitis (e.g., Achilles tendonitis) and dactylitis ("sausage digits") [1]. **High-Yield Clinical Pearls for NEET-PG:** * **Classic Triad:** "Can't see (Uveitis/Conjunctivitis), can't pee (Urethritis), can't climb a tree (Arthritis)" [1]. * **Dermatological findings:** Look for **Keratoderma blennorrhagicum** (vesicular/pustular lesions on palms/soles) and **Circinate balanitis** [1]. * **Synovial Fluid:** Culture is typically **negative** (hence "reactive"), distinguishing it from septic arthritis. * **Treatment:** NSAIDs are first-line; antibiotics treat the triggering infection but do not usually resolve the arthritis itself.

Q: Which of the following is NOT a minor Jones criterion?

Chorea. The **Jones Criteria** are used to diagnose the first episode of **Acute Rheumatic Fever (ARF)**, a non-suppurative sequela of Group A Streptococcal (GAS) pharyngitis [1]. The criteria are divided into Major and Minor categories based on their diagnostic specificity. ### Why Chorea is the Correct Answer **Chorea (Sydenham’s Chorea)** is a **Major Jones Criterion**, not a minor one. It is characterized by rapid, purposeless, involuntary movements and emotional lability [1]. It often has a long latent period (months) after the initial infection and can be the sole manifestation of ARF. ### Explanation of Incorrect Options (Minor Criteria) The following are classified as **Minor Criteria** because they are non-specific and can occur in many inflammatory conditions: * **A. Arthralgia:** Joint pain without objective findings (swelling or redness). Note: If polyarthritis is used as a major criterion, arthralgia cannot be counted as a minor criterion. * **B. Fever:** Usually $\geq 38.5^\circ\text{C}$ in low-risk populations [2]. * **C. Prolonged PR interval:** Evidence of first-degree heart block on ECG (unless carditis is used as a major criterion). * *Other Minor Criteria include elevated acute phase reactants (ESR $\geq 60$ mm/hr or CRP $\geq 3.0$ mg/dL).* ### NEET-PG High-Yield Pearls * **Diagnosis Requirement:** 2 Major OR 1 Major + 2 Minor criteria, **PLUS** evidence of preceding GAS infection (e.g., elevated ASO titer, positive throat culture, or Rapid Antigen Test) [1]. * **Mnemonic for Major Criteria (J♥NES):** * **J** - Joints (Migratory Polyarthritis) * **♥** - Carditis (Pancarditis) [1] * **N** - Nodules (Subcutaneous) * **E** - Erythema Marginatum * **S** - Sydenham’s Chorea * **Most Common Manifestation:** Arthritis. * **Most Serious Manifestation:** Carditis (can lead to chronic Rheumatic Heart Disease, most commonly affecting the **Mitral Valve**).

Q: HLA-B27 antigen is associated with all of the following conditions except?

Rheumatoid arthritis. **Explanation:** The association between HLA-B27 and specific inflammatory conditions is a classic high-yield topic in Rheumatology. HLA-B27 is a Class I surface antigen of the Major Histocompatibility Complex (MHC) strongly linked to the **Seronegative Spondyloarthropathies (SpA)** [1]. **Why Rheumatoid Arthritis (Option D) is the correct answer:** Rheumatoid Arthritis (RA) is **not** part of the Seronegative Spondyloarthropathy group. Instead of HLA-B27, RA is strongly associated with **HLA-DR4** (specifically the "shared epitope" on the DRB1 allele). RA is characterized by symmetric small joint involvement and the presence of Rheumatoid Factor (RF) or Anti-CCP antibodies, whereas HLA-B27-associated diseases are typically RF-negative. **Analysis of Incorrect Options (HLA-B27 Associated):** The mnemonic **"PEAR"** is commonly used to remember the Seronegative Spondyloarthropathies associated with HLA-B27: * **Psoriatic arthropathy (Option C):** Approximately 40-50% of patients with axial involvement are HLA-B27 positive [1]. * **Enteropathic arthritis:** Associated with Inflammatory Bowel Disease (UC/Crohn’s). * **Ankylosing spondylitis (Option A):** Has the strongest association; >90% of patients are HLA-B27 positive [1]. * **Reiter's syndrome / Reactive Arthritis (Option B):** Typically follows a GI or GU infection; 60-80% association with HLA-B27 [1]. **NEET-PG Clinical Pearls:** 1. **Strongest Association:** Ankylosing Spondylitis has the highest correlation with HLA-B27 (>90%) [1]. 2. **Common Features:** These conditions share clinical features like sacroiliitis, enthesitis (inflammation at tendon insertion sites), and uveitis [1]. 3. **HLA-DR4:** Always remember DR4 for Rheumatoid Arthritis to differentiate it from the B27 group. 4. **B27 Subtypes:** While there are many subtypes, **B*2705** is the most common globally associated with disease.

Q: Which of the following are characteristics of Sjögren's syndrome?

All of the above. Sjögren's syndrome (SS) is a chronic, systemic autoimmune disorder characterized by lymphocytic infiltration of exocrine glands, primarily the lacrimal and salivary glands. 1. **Dry Eyes (Keratoconjunctivitis Sicca):** Lymphocytic infiltration leads to decreased tear production [1]. Patients often complain of a "gritty" or "sandy" sensation in the eyes. 2. **Dry Mouth (Xerostomia):** Reduced salivary flow leads to difficulty swallowing dry food, dental caries, and oral candidiasis. 3. **Rheumatoid Arthritis (RA):** Sjögren's syndrome is classified into two types: * **Primary SS:** Occurs in isolation. * **Secondary SS:** Occurs in association with another autoimmune disease, most commonly **Rheumatoid Arthritis** [1], but also SLE or Scleroderma. Since Sjögren's syndrome encompasses both the "sicca complex" (dry eyes/mouth) and can frequently occur secondary to Rheumatoid Arthritis, **Option D (All of the above)** is the correct answer. **High-Yield Clinical Pearls for NEET-PG:** * **Antibodies:** Anti-Ro (SS-A) and Anti-La (SS-B) are the most specific markers. * **Diagnosis:** The **Schirmer’s test** measures tear production (positive if <5mm in 5 mins). The gold standard for diagnosis is a **minor salivary gland biopsy** showing lymphocytic aggregates (Focus score ≥1). * **Malignancy Risk:** Patients with Sjögren's have a 40-fold increased risk of developing **B-cell Non-Hodgkin Lymphoma** (MALToma). * **Extraglandular features:** May include Raynaud’s phenomenon, cutaneous vasculitis, and interstitial nephritis.

Q: In a case of undifferentiated arthritis, the presence of anti-cyclic citrullinated peptide (anti-CCP) antibodies strongly indicates the possibility of which of the following conditions?

Rheumatoid arthritis. The presence of **anti-cyclic citrullinated peptide (anti-CCP)** antibodies in a patient with undifferentiated arthritis is highly predictive of **Rheumatoid Arthritis (RA)**. 1. **Why Rheumatoid Arthritis is correct:** Anti-CCP antibodies are produced against proteins containing citrulline, an amino acid formed by the post-translational modification of arginine [3]. While Rheumatoid Factor (RF) is sensitive, it lacks specificity. In contrast, anti-CCP has a **specificity of >95%** for RA. Its presence in early or undifferentiated arthritis is a strong predictor of progression to erosive RA and is now included in the **ACR/EULAR 2010 classification criteria**. 2. **Why the other options are incorrect:** * **Systemic Lupus Erythematosus (SLE):** The hallmark autoantibody is **Anti-dsDNA** (specific) and ANA (sensitive). While SLE can cause arthritis, anti-CCP is rarely positive unless there is "Rhupus" overlap [1]. * **Mixed Connective Tissue Disorder (MCTD):** This is characterized specifically by high titers of **Anti-U1 RNP** antibodies [1]. * **Reactive Arthritis:** This is a seronegative spondyloarthropathy. It is typically associated with **HLA-B27** and occurs following a gastrointestinal or urogenital infection; autoantibodies like anti-CCP are absent. **High-Yield Pearls for NEET-PG:** * **Most Specific Marker for RA:** Anti-CCP (Anti-citrullinated protein antibody/ACPA). * **Best Initial Test for RA:** Rheumatoid Factor (RF). * **Prognostic Value:** High titers of anti-CCP correlate with a higher risk of **radiographic joint damage** and extra-articular manifestations [2]. * **Smoking Link:** Smoking is a major environmental risk factor that promotes the citrullination of proteins in the lungs, triggering anti-CCP production in genetically susceptible individuals (HLA-DRB1 "shared epitope").

Question 1

Thymoma with hypogammaglobulinemia is also known as:

Accepted Answer

Good's syndrome

Answer

Capgras syndrome

Answer

Fregoli syndrome

Answer

Todd syndrome

Question 2

Which vasculitis is positive for pANCA?

Accepted Answer

Churg-Strauss syndrome

Answer

Wegener's granulomatosis

Answer

Polyarteritis nodosa

Answer

All of the above

Question 3

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

Question 4

A 29-year-old man presents with a 3-day history of burning pain on urination and urethral discharge. ELISA testing of the urethral exudate is positive for Chlamydia trachomatis. Three weeks later, he develops increasing stiffness of his knees and ankles and lower back pain. Radiographs of his lumbar spine reveal narrowing and sclerosis of the sacroiliac joints. One month later, he experiences painful erythema of the glans penis, and his conjunctivae become red. A follow-up examination reveals a slightly irregular heart rate and a murmur suggestive of aortic regurgitation. The back pain recurs intermittently for 5 more months. Which of the following test results is most likely to be positive in this man?

Accepted Answer

HLA-B27 genotype

Answer

ANCA

Answer

ANA

Answer

Anti-Borrelia antibodies

Question 5

Which of the following statements is FALSE about reactive arthritis?

Accepted Answer

Migratory polyarthritis

Answer

Most common among young men

Answer

Linked to HLA-B27

Answer

Painful asymmetric oligoarthritis is a feature

Question 6

Which of the following is NOT a minor Jones criterion?

Accepted Answer

Chorea

Answer

Arthralgia

Answer

Fever

Answer

Prolonged PR interval

Question 7

HLA-B27 antigen is associated with all of the following conditions except?

Accepted Answer

Rheumatoid arthritis

Answer

Ankylosing spondylitis

Answer

Reiter's syndrome

Answer

Psoriatic arthropathy

Question 8

Which of the following are characteristics of Sjögren's syndrome?

Accepted Answer

All of the above

Answer

Dry eyes

Answer

Dry mouth

Answer

Rheumatoid arthritis

Question 9

Palpable purpura is seen in which of the following conditions?

Accepted Answer

Henoch-Schönlein Purpura

Answer

Idiopathic thrombocytopenic purpura (ITP)

Answer

Scurvy

Answer

Monoclonal cryoglobulinemia

Question 10

In a case of undifferentiated arthritis, the presence of anti-cyclic citrullinated peptide (anti-CCP) antibodies strongly indicates the possibility of which of the following conditions?

Accepted Answer

Rheumatoid arthritis

Answer

Systemic lupus erythematosus

Answer

Mixed connective tissue disorder

Answer

Reactive arthritis

Rheumatology and Immunology — MCQs

Rheumatology and Immunology — MCQs

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