Rheumatology and Immunology Practice Questions

Q: A 35-year-old female with heliotrope rash and Gottron's papules has a muscle biopsy showing inflammatory myopathy. What is the diagnosis?

Dermatomyositis. ***Dermatomyositis*** - The combination of a **heliotrope rash**, **Gottron's papules**, and **inflammatory myopathy** on muscle biopsy is pathognomonic for dermatomyositis [1], [2]. - This condition is characterized by **proximal muscle weakness** and distinctive dermatological manifestations [1], [2]. *Systemic lupus erythematosus (SLE)* - While SLE can cause a rash and myositis, the specific presentation of a **heliotrope rash** and **Gottron's papules** is not characteristic of SLE [2]. - SLE rashes typically include a **malar rash** or discoid lesions, and myositis is less common and usually milder [3]. *Polymyositis* - Polymyositis also presents with **inflammatory myopathy** and proximal muscle weakness, but it lacks the characteristic dermatological features seen in this patient [1]. - It does not involve **heliotrope rash** or **Gottron's papules** [1]. *Systemic sclerosis (SSc)* - SSc primarily affects the **skin** with thickening and hardening, and can involve internal organs, but does not typically present with the specific rash and papules described. - Muscle involvement in SSc is usually secondary to **fibrosis** or vasculopathy, distinct from the inflammatory myopathy of dermatomyositis.

Q: A 40-year-old female presents with a chronic dry cough and progressive dyspnea. A CT scan shows diffuse interstitial infiltrates, and pulmonary function tests indicate a restrictive pattern. Serology is positive for anti-SSA/Ro antibodies. What is the most likely diagnosis?

Sjögren's syndrome. ***Sjögren's syndrome*** - The presentation of **diffuse interstitial infiltrates** and **restrictive pulmonary function tests**, along with a **positive anti-SSA/Ro antibody**, strongly suggests Sjögren's syndrome, which can affect the lungs [1]. - While commonly associated with sicca symptoms (**dry eyes** and **mouth**), Sjögren's can also manifest as significant **extraglandular involvement**, including interstitial lung disease [2]. *Systemic sclerosis* - Although systemic sclerosis can cause **interstitial lung disease** and restrictive lung patterns, the primary serological marker in this case is **anti-SSA/Ro**, which is not typical for systemic sclerosis [2]. - Characteristic findings for systemic sclerosis usually include skin thickening and specific antibodies like **anti-Scl-70** or **anti-centromere antibodies**. [2] *Rheumatoid arthritis* - **Rheumatoid arthritis** can cause interstitial lung disease, but a positive **anti-SSA/Ro antibody** is not a primary diagnostic marker for RA; **rheumatoid factor** and **anti-CCP antibodies** are more characteristic [2]. - While RA can present with cough and dyspnea stemming from lung involvement, the serological profile points elsewhere. *Systemic lupus erythematosus* - While **anti-SSA/Ro antibodies** can be positive in **Systemic Lupus Erythematosus (SLE)**, the primary manifestation often involves other organ systems like kidneys, joints, and skin, with lung involvement being variable [2]. - Although SLE can cause pleurisy or lupus pneumonitis, **diffuse interstitial infiltrates** leading to restrictive patterns, combined with anti-SSA/Ro positivity, are more classically associated with Sjögren's syndrome as a **primary manifestation**.

Q: Ankylosing spondylitis most commonly affects which region of the spine?

Sacroiliac joints. ***Sacroiliac joints*** - Ankylosing spondylitis characteristically begins with inflammation of the **sacroiliac joints**, leading to **sacroiliitis** [1]. - This involvement is often **bilateral** and symmetrical, serving as a hallmark feature for diagnosis. *Cervical spine* - While the cervical spine can eventually be affected in later stages of ankylosing spondylitis, it is typically **not the initial or most common site** of involvement. - Involvement here can lead to neck stiffness and reduced range of motion, but usually **after** sacroiliac and lumbar spine disease. *Thoracic spine* - The thoracic spine may become involved in the progression of ankylosing spondylitis, contributing to the characteristic **"bamboo spine"** appearance. - However, it is **less commonly the primary site** of disease onset compared to the sacroiliac joints. *Lumbar spine* - The lumbar spine is frequently affected **after the sacroiliac joints**, leading to inflammation, stiffness, and eventual fusion [1]. - However, the **sacroiliiac joints are almost universally involved first** or concurrently, making them the most common initial presentation.

Q: A 60-year-old woman with rheumatoid arthritis presents with severe pain and swelling in her right knee. Arthrocentesis reveals cloudy fluid with a high white blood cell count. What is the most likely diagnosis?

Septic arthritis. ***Septic arthritis*** - The combination of **rheumatoid arthritis (RA)**, a severe inflammatory condition, with acute **monoarticular pain and swelling**, along with **cloudy synovial fluid** and a **high WBC count on arthrocentesis**, strongly suggests septic arthritis [1]. - Patients with RA are at **increased risk for septic arthritis** due to compromised immune systems from the disease itself and immunosuppressive medications [3]. *Gout* - While gout presents with acute inflammatory arthritis, it typically involves **negatively birefringent, needle-shaped crystals** of **monosodium urate** in the synovial fluid. - The synovial fluid in this case is described as cloudy with a high WBC count, requiring further crystal analysis to confirm gout, but **infection is a more pressing concern** given the RA history. *Pseudogout* - Pseudogout, caused by **calcium pyrophosphate dihydrate (CPPD) crystals**, would show **positively birefringent, rhomboid-shaped crystals** in the synovial fluid [2]. - Like gout, it is an inflammatory arthritis, but the primary concern with a high WBC count and RA history is ruling out bacterial infection. *Osteoarthritis* - Osteoarthritis typically causes **chronic, progressive joint pain** and is associated with **clear to mildly cloudy synovial fluid** with a **low WBC count** (<2000 cells/mm³). - The acute, severe symptoms and high WBC count are inconsistent with uncomplicated osteoarthritis [2].

Q: A 54-year-old woman presents with proximal muscle weakness and a skin rash over her knuckles. Laboratory tests show elevated muscle enzymes and a positive anti-Jo-1 antibody. What is the most likely diagnosis?

Dermatomyositis. Dermatomyositis - The classic triad of **proximal muscle weakness**, a characteristic **skin rash over the knuckles** (Gottron's papules), and **elevated muscle enzymes** strongly indicates dermatomyositis [1]. - The presence of **anti-Jo-1 antibody** further supports the diagnosis, as this is an autoantibody specifically associated with dermatomyositis and polymyositis, particularly with interstitial lung disease [1, 2]. *Rheumatoid arthritis* - Primarily characterized by **symmetric polyarthritis** affecting small joints, not proximal muscle weakness. - While elevated inflammatory markers can be present, **muscle enzymes are typically normal**, and the rash over the knuckles is not typical [2]. *Systemic lupus erythematosus* - Can cause a wide range of symptoms including muscle pain and skin rashes (e.g., malar rash), but **proximal muscle weakness with elevated muscle enzymes** is not its primary presentation. - Specific autoantibodies like **anti-dsDNA** or **anti-Sm** are characteristic, rather than anti-Jo-1 [2]. *Scleroderma* - Characterized by **skin thickening** and **fibrosis of internal organs**, which are not described in this patient [3]. - While muscle involvement can occur, it's typically not the predominant feature, and the specific rash and anti-Jo-1 antibody are not indicative of scleroderma.

Q: Which of the following is NOT a typical feature of seropositive rheumatoid arthritis?

Asymmetrical joint involvement. ***Asymmetrical joint involvement*** - **Seropositive rheumatoid arthritis** typically presents with **symmetrical polyarthritis**, meaning that joints on both sides of the body are affected similarly [1]. - While some asymmetry can occur in early stages or with atypical presentations, it is not considered a **typical feature** of established rheumatoid arthritis [1]. *Inflammation in multiple joints* - **Polyarthritis**, or inflammation in multiple joints, is a hallmark of rheumatoid arthritis, distinguishing it from **mono-** or **oligoarthritis** [1], [2]. - This widespread involvement contributes to the systemic nature and disability associated with the disease. *Symmetrical joint involvement* - **Symmetry** in joint involvement is a classic and highly characteristic feature of rheumatoid arthritis, often affecting the same joints on both sides of the body [1]. - This symmetrical pattern helps differentiate rheumatoid arthritis from other forms of **inflammatory arthritis** that tend to be unilateral or asymmetrical [1]. *Joint pain and swelling* - **Joint pain** and **swelling** are among the most common and typical symptoms of active rheumatoid arthritis, reflecting the underlying inflammatory process [1]. - These symptoms are often accompanied by **morning stiffness** lasting more than 30 minutes, which resolves with activity [1].

Q: Which condition is characterized by the triad of skin lesions, mononeuritis multiplex, and elevated eosinophils?

Churg - Strauss syndrome. ***Churg - Strauss syndrome*** - **Churg-Strauss syndrome** (now known as **Eosinophilic Granulomatosis with Polyangiitis, EGPA**) is characterized by the triad of **skin lesions**, **mononeuritis multiplex** (nerve damage), and **elevated eosinophils** [1]. - It is a **small-vessel vasculitis** with a strong association with **asthma** and **allergic rhinitis** [1]. *Alports syndrome* - **Alport syndrome** is an inherited disorder affecting **type IV collagen**, primarily manifesting as **glomerulonephritis**, **sensorineural hearing loss**, and **ocular abnormalities**. - It does not present with skin lesions, mononeuritis multiplex, or eosinophilia. *Wegeners granulomatosus* - **Wegener's granulomatosis** (now **Granulomatosis with Polyangiitis, GPA**) is characterized by **granulomatous inflammation** of the upper and lower respiratory tracts, **glomerulonephritis**, and **systemic vasculitis** [2]. - While it can cause skin lesions and neuropathy, it is typically associated with **antineutrophil cytoplasmic antibodies (ANCAs)** and does not typically involve eosinophilia as a defining feature [1]. *Cryoglobulinemia* - **Cryoglobulinemia** is a condition where abnormal immunoglobulins (cryoglobulins) precipitate in cold temperatures, leading to **vasculitis-like symptoms** in small and medium-sized vessels. - It often manifests as **palpable purpura**, **arthralgia**, and **glomerulonephritis**, but mononeuritis multiplex and elevated eosinophils are not primary or defining features.

Q: Patients with which of the following conditions are at greatest risk of pernio

Raynaud's phenomenon. ***Raynaud's phenomenon*** - Patients with **Raynaud's phenomenon** have an underlying **vasospastic disorder** where small arteries, especially in the fingers and toes, constrict in response to cold or stress. - This predisposition to **vasoconstriction** makes them more susceptible to developing pernio (chilblains), which is also a localized inflammatory lesion triggered by exposure to cold in susceptible individuals. *Kawasaki disease* - **Kawasaki disease** is a form of **vasculitis** primarily affecting children, characterized by fever, rash, conjunctivitis, and changes in the oral mucosa and extremities. [1] - While it affects blood vessels, its pathology and clinical manifestations are distinct from pernio, and it does not predispose individuals to chilblains. *Henoch-Schönlein purpura* - **Henoch-Schönlein purpura** (HSP) is a **small-vessel vasculitis** characterized by a classic tetrad of palpable purpura, arthritis, abdominal pain, and renal disease, predominantly affecting children. [1] - The mechanism of HSP involves IgA deposition in vessel walls and is not associated with an increased risk of pernio. *Hepatitis C infection* - **Hepatitis C infection** is a viral infection that primarily affects the liver but can have various **extrahepatic manifestations**, including mixed cryoglobulinemia, which causes vasculitis. [1] - While cryoglobulinemic vasculitis can lead to skin lesions, it is distinct from pernio, and hepatitis C infection itself does not directly increase the risk of developing pernio.

Question 1

A 35-year-old female with heliotrope rash and Gottron's papules has a muscle biopsy showing inflammatory myopathy. What is the diagnosis?

Accepted Answer

Dermatomyositis

Answer

Systemic lupus erythematosus (SLE)

Answer

Polymyositis

Answer

Systemic sclerosis (SSc)

Question 2

A 45-year-old patient presents with skin thickening, Raynaud's phenomenon, and digital ulcers. Which enzyme is the target of autoantibodies in this autoimmune disorder?

Accepted Answer

Histone deacetylase

Answer

DNA polymerase

Answer

Protein kinase

Answer

RNA polymerase II

Question 3

A 40-year-old female presents with a chronic dry cough and progressive dyspnea. A CT scan shows diffuse interstitial infiltrates, and pulmonary function tests indicate a restrictive pattern. Serology is positive for anti-SSA/Ro antibodies. What is the most likely diagnosis?

Accepted Answer

Sjögren's syndrome

Answer

Systemic sclerosis

Answer

Rheumatoid arthritis

Answer

Systemic lupus erythematosus

Question 4

Ankylosing spondylitis most commonly affects which region of the spine?

Accepted Answer

Sacroiliac joints

Answer

Cervical spine

Answer

Thoracic spine

Answer

Lumbar spine

Question 5

A 60-year-old woman with rheumatoid arthritis presents with severe pain and swelling in her right knee. Arthrocentesis reveals cloudy fluid with a high white blood cell count. What is the most likely diagnosis?

Accepted Answer

Septic arthritis

Answer

Gout

Answer

Pseudogout

Answer

Osteoarthritis

Question 6

A 54-year-old woman presents with proximal muscle weakness and a skin rash over her knuckles. Laboratory tests show elevated muscle enzymes and a positive anti-Jo-1 antibody. What is the most likely diagnosis?

Accepted Answer

Dermatomyositis

Answer

Rheumatoid arthritis

Answer

Systemic lupus erythematosus

Answer

Scleroderma

Question 7

Which of the following is NOT a typical feature of seropositive rheumatoid arthritis?

Accepted Answer

Asymmetrical joint involvement

Answer

Inflammation in multiple joints

Answer

Symmetrical joint involvement

Answer

Joint pain and swelling

Question 8

All are true about Marie-Strumpell disease except which of the following?

Accepted Answer

Roentgenogram is the most sensitive investigation for Marie-Strumpell disease.

Answer

Enhesitis is common

Answer

Most commonly involves the sacro - iliac joints

Answer

More common in males

Question 9

Which condition is characterized by the triad of skin lesions, mononeuritis multiplex, and elevated eosinophils?

Accepted Answer

Churg - Strauss syndrome

Answer

Alports syndrome

Answer

Wegeners granulomatosus

Answer

Cryoglobulinemia

Question 10

Patients with which of the following conditions are at greatest risk of pernio

Accepted Answer

Raynaud's phenomenon

Answer

Kawasaki disease

Answer

Hepatitis C infection

Answer

Henoch-Schönlein purpura

Rheumatology and Immunology — MCQs

Rheumatology and Immunology — MCQs

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