Rheumatology and Immunology Practice Questions

Q: A 22-year-old man presents with low back pain and stiffness that began several months ago. The pain is now more intense, affecting his back and hips, and is worse at night, disrupting sleep. Physical examination reveals paravertebral muscle tenderness and limited lumbar spine flexion. Chest expansion is also reduced. Which of the following is the most likely diagnosis?

Ankylosing spondylitis. **Explanation:** The clinical presentation is classic for **Ankylosing Spondylitis (AS)**, a chronic inflammatory spondyloarthropathy. The key diagnostic features in this 22-year-old male are: 1. **Inflammatory Back Pain:** Onset at a young age (<40 years), insidious progression, and pain that is worse at night/rest but improves with activity [1]. 2. **Limited Spinal Mobility:** Reduced lumbar flexion (measured by the Modified Schober’s test) and paravertebral muscle spasms [1]. 3. **Reduced Chest Expansion:** This occurs due to involvement of the costovertebral and costosternal joints, a high-yield diagnostic criterion for AS. **Why other options are incorrect:** * **Osteoarthritis:** Typically affects older individuals and presents as "mechanical" pain (worsens with activity, improves with rest) [2]. It rarely involves the spine in this manner at age 22. * **Marfan Syndrome:** A connective tissue disorder characterized by arachnodactyly, ectopia lentis, and aortic root dilation. While it can cause scoliosis, it does not present with inflammatory spinal stiffness or reduced chest expansion. * **Rheumatoid Arthritis:** Primarily involves small joints of the hands and feet (symmetrical polyarthritis). While it can affect the cervical spine (atlantoaxial subluxation), it characteristically **spares** the lumbosacral spine and sacroiliic joints. **NEET-PG High-Yield Pearls:** * **Strongest Association:** HLA-B27 (present in >90% of white patients) [1]. * **Radiology:** "Bamboo Spine" (due to syndesmophytes) and sacroiliitis (earliest radiographic change) [1]. * **Extra-articular manifestation:** Acute Anterior Uveitis is the most common [1]. * **Modified New York Criteria:** Used for diagnosis, requiring clinical evidence of limited motion/expansion plus radiological evidence of sacroiliitis [1].

Q: Which of the following is an ANCA-positive vasculitis?

Wegener's granulomatosis. Explanation: Wegener’s granulomatosis (now known as Granulomatosis with Polyangiitis or GPA) is the correct answer because it belongs to the triad of ANCA-associated vasculitides (AAV). These are small-vessel vasculitides characterized by the presence of Antineutrophil Cytoplasmic Antibodies. Specifically, GPA is strongly associated with c-ANCA (cytoplasmic pattern) directed against Proteinase-3 (PR3). It typically presents with the clinical triad of upper respiratory tract involvement (e.g., saddle nose deformity), lower respiratory tract involvement (cavitary nodules), and renal involvement (pauci-immune glomerulonephritis). Why the other options are incorrect: * Henoch-Schönlein purpura (IgA Vasculitis): This is an immune-complex mediated small-vessel vasculitis [1]. It is characterized by IgA deposition in tissues and is typically ANCA-negative [1]. * Behçet's syndrome: This is a variable-vessel vasculitis (affecting arteries and veins of all sizes). Its pathogenesis is linked to HLA-B51 and autoinflammation rather than ANCA. Clinical Pearls for NEET-PG: * The ANCA Triad: 1. GPA (Wegener’s): c-ANCA (PR3) positive. 2. Microscopic Polyangiitis (MPA): p-ANCA (MPO) positive; lacks granulomas. 3. Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss): p-ANCA (MPO) positive; associated with asthma and eosinophilia [1]. * Pauci-immune: This term is high-yield; it means that on immunofluorescence of a renal biopsy, there is little to no antibody/complement deposition, distinguishing AAV from HSP or SLE. * Treatment: Induction is usually achieved with Cyclophosphamide or Rituximab plus corticosteroids [1].

Q: A 22-year-old patient presents with hemoptysis and hematuria, and has basement membrane antibodies. What is the most likely diagnosis?

Goodpasture's syndrome. **Explanation:** The clinical triad of **hemoptysis** (pulmonary hemorrhage), **hematuria** (glomerulonephritis), and the presence of **anti-glomerular basement membrane (anti-GBM) antibodies** is pathognomonic for **Goodpasture’s Syndrome**. This is a Type II hypersensitivity reaction where antibodies are directed against the alpha-3 chain of Type IV collagen, found specifically in the basement membranes of the renal glomeruli and pulmonary alveoli. **Why other options are incorrect:** * **Wegener’s Granulomatosis (GPA):** While it also presents with a pulmonary-renal syndrome, it is a small-vessel vasculitis characterized by **c-ANCA (anti-PR3)** positivity and granulomatous inflammation of the respiratory tract. It does not involve anti-GBM antibodies. * **Polyarteritis Nodosa (PAN):** This is a medium-vessel vasculitis. While it causes renal involvement (hypertension/renal failure), it characteristically **spares the lungs** and is not associated with basement membrane antibodies. * **Churg-Strauss Syndrome (EGPA):** This is characterized by **p-ANCA (anti-MPO)**, peripheral eosinophilia, and a strong history of adult-onset asthma and allergic rhinitis. **High-Yield Clinical Pearls for NEET-PG:** * **Immunofluorescence:** Goodpasture’s shows a characteristic **linear IgG deposition** along the glomerular capillaries (unlike the "lumpy-bumpous" granular pattern in PSGN). * **Demographics:** Typically affects young males (20s) or older females (60s). * **Treatment:** The mainstay of treatment is **Plasmapheresis** (to remove circulating antibodies) combined with corticosteroids and cyclophosphamide. * **HLA Association:** Strongly associated with **HLA-DR15** and **HLA-DR4**.

Q: A middle-aged male presents with back pain. X-ray reveals syndesmophytes involving four continuous vertebrae. What is the most likely diagnosis?

Ankylosing spondylitis. **Explanation:** The correct answer is **Ankylosing Spondylitis (AS)**. The hallmark radiographic feature of AS is the formation of **syndesmophytes**, which are thin, vertical, bony outgrowths originating from the corners of the vertebral bodies. These result from inflammation and subsequent ossification of the outer fibers of the annulus fibrosus. When these syndesmophytes bridge multiple continuous vertebrae, they create the classic **"Bamboo Spine"** appearance. **Why other options are incorrect:** * **Diffuse Idiopathic Skeletal Hyperostosis (DISH):** While DISH also involves ossification of the spine, it typically presents as thick, flowing calcification of the **Anterior Longitudinal Ligament (ALL)**, often described as "melted candle wax." Crucially, DISH preserves the disc space and does not involve the sacroiliac (SI) joints, unlike AS. * **Rheumatoid Arthritis:** This primarily affects the cervical spine (atlantoaxial subluxation) and small joints of the hands. It is an erosive disease and does not typically form syndesmophytes. * **Osteoarthritis:** This presents with **osteophytes**, which are horizontal, claw-like bony spurs resulting from degenerative changes, rather than the vertical syndesmophytes seen in inflammatory spondyloarthropathies. **High-Yield Clinical Pearls for NEET-PG:** * **Earliest Sign:** The earliest radiographic sign of AS is **Sacroiliitis** (blurring of the lower 2/3rd of the SI joint). * **Genetic Association:** Strongly linked with **HLA-B27** (>90% of cases). * **Clinical Test:** **Schober’s Test** is used to assess restricted lumbar flexion. * **Extra-articular manifestation:** The most common is **Acute Anterior Uveitis**. * **Key Radiographic Terms:** "Bamboo spine," "Dagger sign" (ossification of supraspinous/interspinous ligaments), and "Anderson lesion" (discovertebral inflammation).

Q: A 39-year-old man presents with several weeks of fever, weight loss, and fatigue. Three days prior to assessment, he developed a left foot drop. Physical examination reveals left peroneal nerve damage and a bilateral sensory peripheral neuropathy in both legs. Laboratory evaluation shows an ESR of 105 mm/h, neutrophilia of 14,000, and a negative serologic test for ANCA. Eosinophil count is normal. Angiography reveals small aneurysms of the celiac and renal arteries. What is the most likely diagnosis for this patient with a vasculitis syndrome?

Polyarteritis nodosa (PAN). This patient presents with a classic triad of **systemic symptoms** (fever, weight loss) [1], **mononeuritis multiplex** (left foot drop/peroneal nerve palsy), and **renal/celiac artery aneurysms**, which is pathognomonic for **Polyarteritis Nodosa (PAN)**. ### Why Polyarteritis Nodosa (PAN) is Correct: PAN is a systemic necrotizing vasculitis that typically affects **medium-sized muscular arteries**. * **Neurological involvement:** Mononeuritis multiplex (e.g., foot drop) is a hallmark of PAN due to vasculitis of the *vasa nervorum*. * **Angiography:** The presence of **microaneurysms** (beaded appearance) in the renal or mesenteric arteries is a key diagnostic feature. * **Serology:** PAN is characteristically **ANCA-negative**, distinguishing it from small-vessel vasculitides. * **Spared Organs:** PAN typically **spares the lungs**. ### Why Other Options are Incorrect: * **B. Churg-Strauss (EGPA):** While it causes mononeuritis multiplex, it is strongly associated with **asthma, peripheral eosinophilia** [2], and positive p-ANCA (in 40-50% of cases). This patient has a normal eosinophil count. * **C. Henoch-Schonlein Purpura (IgA Vasculitis):** This is a small-vessel vasculitis [2] characterized by the tetrad of palpable purpura, arthralgia, abdominal pain, and renal disease (hematuria). It does not typically cause arterial aneurysms. * **D. Vasculitis with Infectious Diseases:** While Hepatitis B is associated with PAN (10-30% of cases), the clinical constellation here points directly to the primary syndrome of PAN itself. ### NEET-PG High-Yield Pearls: * **Vessel Size:** PAN = Medium-sized arteries. * **Key Association:** Strongly associated with **Hepatitis B surface antigen (HBsAg)**. * **Classic Image:**

Q: Which of the following HLA types is associated with rheumatoid arthritis?

DR 4. **Explanation:** **Correct Option: C (HLA-DR4)** The association between Rheumatoid Arthritis (RA) and the Major Histocompatibility Complex (MHC) is well-established. Specifically, RA is strongly linked to the **HLA-DRB1** alleles, which encode a specific sequence of amino acids (70-74) known as the **"Shared Epitope."** Among these, **HLA-DR4** is the most common association. This genetic predisposition influences the presentation of self-antigens to T-cells, triggering the autoimmune inflammatory cascade characteristic of RA. Patients with HLA-DR4 often exhibit more severe, erosive disease and higher titers of Rheumatoid Factor (RF) and Anti-CCP antibodies. **Incorrect Options:** * **HLA-DR2:** Primarily associated with **Systemic Lupus Erythematosus (SLE)**, Multiple Sclerosis, and Goodpasture Syndrome. * **HLA-DR3:** Associated with **SLE**, Sjögren’s syndrome, and Type 1 Diabetes Mellitus. * **HLA-DR5:** Associated with Juvenile Rheumatoid Arthritis (Pauciarticular type) and Hashimoto’s thyroiditis [1]. **High-Yield Clinical Pearls for NEET-PG:** * **HLA-B27:** Strongly associated with Seronegative Spondyloarthropathies (Ankylosing Spondylitis, Reiter’s Syndrome, Psoriatic Arthritis). * **HLA-DRB1*0401 and *0404:** These specific subtypes of DR4 carry the highest risk for RA. * **Smoking & Genetics:** Smoking significantly increases the risk of RA specifically in individuals who are positive for the HLA-DRB1 shared epitope (Gene-Environment interaction). * **Anti-CCP:** This is the most specific prognostic marker for RA and is closely linked to the presence of the shared epitope.

Q: Calcification of meniscal cartilage is a feature of which condition?

Pseudogout. ### Explanation **Correct Answer: B. Pseudogout** **Concept:** Calcification of the articular or meniscal cartilage is known as **Chondrocalcinosis**. This is the hallmark radiological feature of **Pseudogout**, a condition caused by the deposition of **Calcium Pyrophosphate Dihydrate (CPPD)** crystals in the joints. In the knee, these crystals typically deposit within the fibrocartilaginous menisci and the hyaline articular cartilage, appearing as linear or punctate radiopaque densities on X-rays. **Analysis of Incorrect Options:** * **A. Gaucher’s Disease:** This is a lysosomal storage disorder characterized by the accumulation of glucocerebrosides. Orthopaedic manifestations include "Erlenmeyer flask" deformity of the femur, avascular necrosis (AVN), and bone crises, but not primary meniscal calcification. * **C. Achondroplasia:** This is a genetic disorder of endochondral ossification leading to dwarfism. It features rhizomelic shortening of limbs and spinal stenosis, but does not involve pathological cartilage calcification. * **D. Hyperparathyroidism:** While hyperparathyroidism *can* be an underlying metabolic cause associated with secondary CPPD, it is not the primary definition of meniscal calcification itself. Pseudogout is the specific clinical entity defined by this finding. **High-Yield Clinical Pearls for NEET-PG:** * **Crystal Morphology:** CPPD crystals are **positively birefringent** and **rhomboid-shaped** under polarized microscopy (unlike Gout, which has negatively birefringent, needle-shaped urate crystals). * **Common Site:** The **Knee** is the most common joint involved in Pseudogout, followed by the wrist (triangular fibrocartilage complex). * **Associated Conditions:** Always screen for the "4 Hs" in patients with chondrocalcinosis: **H**yperparathyroidism, **H**emochromatosis, **H**ypomagnesemia, and **H**ypothyroidism. * **Radiology:** Look for a thin, shaggy linear opacity parallel to the subchondral bone.

Q: Which antibody is characteristically seen in drug-induced Systemic Lupus Erythematosus (SLE)?

Anti-histone antibody. **Explanation:** **1. Why Anti-histone Antibody is Correct:** Anti-histone antibodies are the hallmark of **Drug-Induced Lupus Erythematosus (DILE)**. While Anti-Nuclear Antibodies (ANA) are present in almost all cases [1], anti-histone antibodies are highly characteristic, being positive in **>95%** of patients with DILE. In contrast, they are seen in only about 50-70% of patients with idiopathic SLE. The presence of these antibodies without the presence of anti-dsDNA is a strong diagnostic clue for a drug-induced etiology. **2. Analysis of Incorrect Options:** * **B. Anti-nuclear antibody (ANA):** While ANA is the best **screening test** (highly sensitive) for both idiopathic and drug-induced SLE, it is not specific to DILE [2]. * **C. Anti-Sm (Smith) antibody:** This is the most **specific** antibody for **idiopathic SLE** [1]. It is characteristically absent in drug-induced SLE. * **D. Anti-centromere antibody:** This antibody is the marker for **Limited Cutaneous Systemic Sclerosis** (formerly CREST syndrome), not SLE. **3. NEET-PG High-Yield Pearls:** * **Common Trigger Drugs:** Remember the mnemonic **SHIPP**: **S**ulfonamides, **H**ydralazine (highest risk), **I**soniazid, **P**rocainamide (highest frequency), and **P**henytoin. (Minocycline and Anti-TNF agents are also modern triggers). * **Clinical Presentation:** DILE typically presents with fever, arthralgia, and serositis. Notably, **CNS and Renal involvement are rare**, and the classic malar rash is often absent. * **Prognosis:** Symptoms and antibody titers usually resolve within weeks to months after discontinuing the offending drug. * **Exception:** In DILE caused by **Hydralazine**, anti-histone antibodies may occasionally be absent.

Q: Which of the following antibodies is specific for myositis?

Anti-Jo-1. ### Explanation **Correct Option: A. Anti-Jo-1** Anti-Jo-1 (anti-histidyl-tRNA synthetase) is the most common and classic **Myositis-Specific Antibody (MSA)**. It is found in approximately 20–30% of patients with Polymyositis (PM) and Dermatomyositis (DM). Its presence is highly diagnostic and is strongly associated with **Antisynthetase Syndrome**, characterized by the triad of interstitial lung disease (ILD), inflammatory arthritis, and "mechanic’s hands" [1]. **Analysis of Incorrect Options:** * **B. Anti-Scl-70 (Anti-topoisomerase I):** This is highly specific for **Diffuse Cutaneous Systemic Sclerosis**. It is a marker for increased risk of severe interstitial lung disease and poor prognosis in scleroderma patients. * **C. Anti-Sm (Anti-Smith):** This is the most specific antibody for **Systemic Lupus Erythematosus (SLE)** [1]. While only present in about 30% of SLE patients, its presence is pathognomonic. * **D. Anti-Ku:** This is a **Myositis-Associated Antibody (MAA)** rather than a specific one. It is typically seen in **overlap syndromes**, particularly the combination of Systemic Sclerosis and Myositis. **High-Yield Clinical Pearls for NEET-PG:** * **Anti-Mi-2:** Highly specific for Dermatomyositis; usually carries a good prognosis and classic skin findings (Gottron papules, Heliotrope rash). * **Anti-SRP (Signal Recognition Particle):** Associated with severe, necrotizing myopathy and poor response to therapy. * **Anti-MDA5:** Associated with clinically amyopathic dermatomyositis and rapidly progressive ILD. * **Initial Screening:** ANA is positive in about 80% of myositis cases [1], but MSAs like Anti-Jo-1 are required for definitive subtyping.

Question 1

A 22-year-old man presents with low back pain and stiffness that began several months ago. The pain is now more intense, affecting his back and hips, and is worse at night, disrupting sleep. Physical examination reveals paravertebral muscle tenderness and limited lumbar spine flexion. Chest expansion is also reduced. Which of the following is the most likely diagnosis?

Accepted Answer

Ankylosing spondylitis

Answer

Osteoarthritis

Answer

Marfan syndrome

Answer

Rheumatoid arthritis

Question 2

Which of the following is an ANCA-positive vasculitis?

Accepted Answer

Wegener's granulomatosis

Answer

Henoch-Schönlein purpura

Answer

Behçet's syndrome

Answer

None of the above

Question 3

A 22-year-old patient presents with hemoptysis and hematuria, and has basement membrane antibodies. What is the most likely diagnosis?

Accepted Answer

Goodpasture's syndrome

Answer

Wagner's Granulomatosis

Answer

Polyarteritis Nodosa (PAN)

Answer

Churg-Strauss syndrome

Question 4

A middle-aged male presents with back pain. X-ray reveals syndesmophytes involving four continuous vertebrae. What is the most likely diagnosis?

Accepted Answer

Ankylosing spondylitis

Answer

Diffuse idiopathic skeletal hyperostosis (DISH)

Answer

Rheumatoid arthritis

Answer

Osteoarthritis

Question 5

A 39-year-old man presents with several weeks of fever, weight loss, and fatigue. Three days prior to assessment, he developed a left foot drop. Physical examination reveals left peroneal nerve damage and a bilateral sensory peripheral neuropathy in both legs. Laboratory evaluation shows an ESR of 105 mm/h, neutrophilia of 14,000, and a negative serologic test for ANCA. Eosinophil count is normal. Angiography reveals small aneurysms of the celiac and renal arteries. What is the most likely diagnosis for this patient with a vasculitis syndrome?

Accepted Answer

Polyarteritis nodosa (PAN)

Answer

Churg-Strauss disease

Answer

Henoch-Schonlein purpura

Answer

Vasculitis associated with infectious diseases

Question 6

Which of the following HLA types is associated with rheumatoid arthritis?

Accepted Answer

DR 4

Answer

DR 2

Answer

DR 3

Answer

DR 5

Question 7

Calcification of meniscal cartilage is a feature of which condition?

Accepted Answer

Pseudogout

Answer

Gaucher's disease

Answer

Achondroplasia

Answer

Hyperparathyroidism

Question 8

Which of the following is not a seronegative spondyloarthropathy?

Accepted Answer

Rheumatoid arthritis

Answer

Psoriatic arthritis

Answer

Ankylosing spondylitis

Answer

Reiter's arthritis

Question 9

Which antibody is characteristically seen in drug-induced Systemic Lupus Erythematosus (SLE)?

Accepted Answer

Anti-histone antibody

Answer

Anti-nuclear antibody

Answer

Anti-Sm antibody

Answer

Anti-centromere antibody

Question 10

Which of the following antibodies is specific for myositis?

Accepted Answer

Anti-Jo-1

Answer

Anti-Scl-70

Answer

Anti-Sm

Answer

Anti-Ku

Rheumatology and Immunology — MCQs

Rheumatology and Immunology — MCQs

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