Rheumatology and Immunology Practice Questions

Q: All of the following are indications for use of corticosteroids in SLE except:

Endocarditis. ***Endocarditis*** - While **Libman-Sacks endocarditis** is a known manifestation of SLE, it typically involves **non-infectious vegetations** and rarely causes significant valvular dysfunction requiring corticosteroid therapy for acute treatment. - Steroids are generally not indicated for the primary treatment of established endocarditis, whether infectious or non-infectious, unless there is a severe active inflammatory component or an autoimmune-driven valvulitis. *Nephritic syndrome* - **Lupus nephritis**, especially the proliferative forms (Class III and IV), often presents with nephritic syndrome and is a **primary indication for aggressive corticosteroid therapy** to suppress inflammation and prevent renal failure [1]. - Corticosteroids are crucial in combination with immunosuppressants to manage renal inflammation, proteinuria, and declining renal function. *Pericarditis* - **Pericarditis** in SLE can be symptomatic and is often managed with **corticosteroids** to reduce inflammation and alleviate chest pain [1]. - Severe or recurrent pericarditis may require higher doses of steroids, and tamponade is a rare but serious complication. *Neuropsychiatric lupus* - Manifestations such as **CNS vasculitis**, **seizures**, psychosis, and severe cognitive dysfunction are considered **major organ involvement** and are treated with **high-dose corticosteroids** to reduce inflammation and prevent permanent neurological damage. - Steroids are often part of the initial treatment regimen for acute and severe neuropsychiatric SLE.

Q: Most specific test for SLE

Anti- smith antibody. ***Anti-smith antibody*** - **Anti-Smith (anti-Sm) antibodies** are highly specific for **Systemic Lupus Erythematosus (SLE)**, meaning their presence strongly indicates the disease [1]. - While only present in about 20-30% of SLE patients, they are rarely found in other conditions, making them a key diagnostic marker [1]. *ds-DNA* - **Anti-dsDNA antibodies** are highly specific for SLE and often correlate with **disease activity**, especially **lupus nephritis**. - However, their specificity is slightly lower than anti-Sm antibodies, as they can sometimes be found in other autoimmune conditions (e.g., drug-induced lupus). *ss DNA* - **Anti-ssDNA antibodies** are less specific for SLE and can be found in various other **autoimmune diseases**, infections, and even in healthy individuals. - Therefore, their presence alone is not sufficient for a diagnosis of SLE. *Histone* - **Anti-histone antibodies** are most commonly associated with **drug-induced lupus (DIL)**, found in about 95% of patients with DIL. - While they can be present in some cases of SLE, they are not specific for spontaneous SLE and are a better indicator of DIL.

Q: Symmetrical persistent enlargement of the parotid gland is seen in:

Mikulicz disease.. ***Mikulicz disease*** - This condition is characterized by **chronic, symmetrical enlargement of the lacrimal and salivary glands**, predominantly the parotid glands [1]. - It is often considered a benign lymphoepithelial lesion, now frequently reclassified as an **IgG4-related disease** or a manifestation of **Sjögren syndrome**. *Sjögren syndrome* - While **parotid gland enlargement can occur** in Sjögren syndrome [1], it is not always "symmetrical and persistent" in the same distinct pattern as described for Mikulicz disease. - Sjögren syndrome is primarily an **autoimmune disorder** leading to xerostomia and xerophthalmia, with glandular enlargement being a possible, but not always defining, feature in all cases. *Cylindroma* - A cylindroma (**adenoid cystic carcinoma**) is a **malignant tumor** of salivary glands [1], and it typically presents as a **solitary, unilateral mass**, not a symmetrical, persistent enlargement. - This type of tumor is characterized by its **infiltrative growth pattern** and perineural invasion, which are distinct from the diffuse glandular enlargement seen in Mikulicz disease. *All of the options* - This option is incorrect because **Cylindroma does not present with symmetrical persistent parotid enlargement**, making an "all of the above" answer inappropriate. - While Sjögren syndrome can involve parotid enlargement, the specific presentation of "symmetrical persistent enlargement" is more definitively associated with Mikulicz disease or its contemporary understanding within IgG4-related diseases.

Q: Which is NOT a feature of polymyositis?

Ocular muscle involvement. ***Ocular muscle involvement*** - **Polymyositis** primarily affects **proximal skeletal muscles**, sparing the **ocular** and facial muscles. - Involvement of **ocular muscles** is more characteristic of other neuromuscular disorders, such as **myasthenia gravis**. *Proximal muscle weakness* - This is a hallmark symptom of **polymyositis**, manifesting as difficulty with activities like rising from a chair or lifting objects [1]. - The weakness is typically **symmetric** and progressive, affecting muscles of the **shoulders, hips, and neck** [1]. *Elevated serum creatine kinase (CK) levels* - Elevated **CK levels** are a key laboratory finding in polymyositis, indicating **muscle damage** and inflammation. - The degree of **CK elevation** often correlates with disease activity and muscle breakdown. *Endomysial inflammation on muscle biopsy* - A **muscle biopsy** is crucial for diagnosing polymyositis, revealing characteristic **inflammatory infiltrates** consisting mainly of **CD8+ T cells** surrounding and invading non-necrotic muscle fibers. - This **endomysial inflammation** differentiates polymyositis from other myopathies.

Q: A 65-year-old patient presents with severe headache, temporal artery tenderness, and decreased pulse. What is the most likely diagnosis?

Giant cell arteritis. ***Giant cell arteritis*** - This presentation with **severe headache**, **temporal artery tenderness**, and a **decreased pulse** in a 65-year-old patient is highly classic for giant cell arteritis (GCA). GCA characteristically affects **medium and large arteries**, often the **temporal artery**. - **Decreased pulse** can indicate involvement of other large vessels, such as the subclavian artery, which can occur in GCA. Urgent diagnosis and treatment are crucial due to the risk of **permanent vision loss** [1]. *Wegener's granulomatosis* - This condition (**granulomatosis with polyangiitis**) is characterized by **upper and lower respiratory tract granulomatous inflammation**, **glomerulonephritis**, and small vessel vasculitis. - While it can manifest with systemic symptoms, **temporal artery tenderness** and a **decreased pulse** are not primary features of Wegener's. *Microscopic polyangiitis* - This is a **small vessel vasculitis** that primarily affects capillaries, venules, and arterioles. - It typically presents with **glomerulonephritis** and **pulmonary capillaritis**, but without granuloma formation, and does not involve the temporal arteries or lead to a decreased pulse in the manner described. *Takayasu arteritis* - Takayasu arteritis primarily affects the **aorta and its major branches**, leading to **claudication**, **pulse deficits** in the extremities, and often occurs in **younger women**. - While it can cause a decreased pulse, it is less likely to present with **temporal artery tenderness** and severe headache in a 65-year-old, as these symptoms are more characteristic of GCA.

Q: Bamboo spine with sacroilitis -

Ankylosing spondylitis. ***Ankylosing spondylitis*** - **Bamboo spine** is a classic radiographic finding in advanced **ankylosing spondylitis**, resulting from ossification of the anulus fibrosus and spinal ligaments [1]. - **Sacroiliitis**, inflammation of the sacroiliac joints, is another hallmark feature and often the earliest radiographic sign of the disease [1], [3]. *Psoriatic arthritis* - While psoriatic arthritis can involve the spine and cause sacroiliitis, it typically presents with **asymmetric involvement** [2] and is less commonly associated with the widespread, progressive ossification characteristic of true "bamboo spine" [4]. - It often also involves **skin psoriasis** and **nail changes**, which are not indicated as the primary finding here [4]. *OA* - **Osteoarthritis (OA)** is a degenerative joint disease characterized by cartilage loss and osteophyte formation, often seen in weight-bearing joints and the spine [2]. - While OA can affect spinal joints and cause stiffness, it does **not cause inflammatory sacroiliitis** or the specific syndesmophyte formation that leads to "bamboo spine." *RA* - **Rheumatoid arthritis (RA)** primarily affects peripheral joints, particularly small joints of the hands and feet, and typically spares the sacroiliac joints and the thoracolumbar spine [2]. - Spinal involvement in RA is usually limited to the **cervical spine**, leading to atlantoaxial subluxation, and does not cause "bamboo spine" or widespread sacroiliitis.

Q: A 23-year-old male patient presented with a history of back pain, which is more in the morning and relieved by bathing in warm water. What is the likely additional finding present in this patient?

Decreased chest wall expansion. Decreased chest wall expansion - The patient's symptoms of morning back pain relieved by activity and warm baths are classic for ankylosing spondylitis. This condition commonly leads to fusion of the costovertebral joints, limiting chest wall expansion [1]. - Reduced chest wall expansion is a specific finding in ankylosing spondylitis, reflecting the ankylosis of the axial skeleton and enthesitis at various sites, including rib attachments [1]. Marrow fibrosis - Myelofibrosis is a bone marrow disorder characterized by fibrosis, typically leading to symptoms like fatigue, splenomegaly, and cytopenias, and is not directly associated with ankylosing spondylitis. - While inflammatory conditions can rarely cause reactive changes in bone marrow, widespread fibrosis is not a hallmark or common feature of ankylosing spondylitis. Pleural nodules - Pleural nodules are more characteristic of conditions like rheumatoid arthritis (rheumatoid nodules) or various lung malignancies/infections. - Although lung involvement, such as apical pulmonary fibrosis, can occur in ankylosing spondylitis, discrete pleural nodules are not common. Distal phalangeal joint involvement - Involvement of the distal interphalangeal (DIP) joints is a hallmark feature of psoriatic arthritis [2]. - Ankylosing spondylitis primarily affects the axial skeleton (spine and sacroiliac joints) and large peripheral joints, with DIP joint involvement being very rare [2].

Q: A 25-year-old man complains of low backache, decreased lumbar movements, morning stiffness, which clinical examination will further help:

Chest expansion. ***Chest expansion*** - In **ankylosing spondylitis** (suggested by the young age, low backache, decreased lumbar movements, and morning stiffness), **chest wall restriction** is common due to enthesitis of the costovertebral and sternocostal joints [1]. - Measuring **chest expansion** helps assess the degree of involvement and progression of the disease [1]. *Head circumference* - This measurement is typically used in the assessment of **pediatric growth and development** or certain neurological conditions, not relevant for adult back pain. - It provides no diagnostic information for conditions affecting the spine and joints. *Plantar arch* - Assessment of the **plantar arch** relates to foot mechanics and conditions like **pes planus (flat feet)** or **pes cavus (high arches)**. - While foot problems can cause pain, they are not directly linked to the typical presentation of inflammatory spondyloarthropathies affecting the lumbar spine. *Hyperextension of joints* - **Joint hyperextension** (hypermobility) is characteristic of conditions like **Ehlers-Danlos syndrome** or other hypermobility syndromes [2]. - The patient's symptoms of decreased lumbar movements and stiffness are actually the opposite of hypermobility.

Q: Extra articular manifestation of rheumatoid arthritis is:

Subcutaneous nodules. **Subcutaneous nodules** - **Subcutaneous nodules** are a common **extra-articular manifestation** of rheumatoid arthritis [1], particularly seen in patients with high titers of **rheumatoid factor**. - These firm, non-tender nodules often appear over **pressure points** like the elbows, hands, and Achilles tendons [1]. *Av fistula* - An **arteriovenous (AV) fistula** is a surgically created connection between an artery and a vein, primarily used for **hemodialysis access**, and is not an extra-articular manifestation of rheumatoid arthritis. - While patients with chronic diseases like RA may develop kidney disease requiring dialysis, the fistula itself is a **treatment intervention**, not a disease manifestation. *Splenic atrophy* - **Splenic atrophy** (or hyposplenism) is not a typical extra-articular manifestation of rheumatoid arthritis. - **Felty's syndrome**, a rare, severe form of RA, involves **splenomegaly** (enlarged spleen) along with neutropenia and arthritis, rather than splenic atrophy [2]. *Cutaneous hypomelanosis* - **Cutaneous hypomelanosis** refers to patches of skin with reduced pigmentation and is not a recognized extra-articular manifestation of rheumatoid arthritis. - While some skin changes can occur in RA (e.g., vasculitis, rheumatoid nodules in the skin), **pigmentation disorders** are not characteristic.

Q: Limited cutaneous systemic sclerosis have the following characteristic autoantibody positivity:

Anticentromere. ***Anticentromere*** - **Anticentromere antibodies (ACA)** are highly specific for **limited cutaneous systemic sclerosis (lcSSc)**, also known as **CREST syndrome** [1]. - Their presence correlates with a lower risk of **internal organ fibrosis** but a higher risk of **pulmonary hypertension** and **primary biliary cholangitis** [1]. *Anti-RNA polymerase III* - **Anti-RNA polymerase III antibodies** are typically associated with the **diffuse cutaneous systemic sclerosis (dcSSc)** subtype. - They are linked to an increased risk of **renal crisis** and **malignancy**. *Anti Topoisomerase I* - Also known as **anti-Scl-70 antibodies**, these are strongly associated with **diffuse cutaneous systemic sclerosis (dcSSc)**. - They indicate a higher risk of **pulmonary fibrosis** and **severe skin disease**. *Anti-U3-RNP (fibrillarin)* - **Anti-U3-RNP (fibrillarin) antibodies** are found in a subset of patients with **diffuse cutaneous systemic sclerosis (dcSSc)**. - They are associated with a higher likelihood of **myositis**, **pulmonary hypertension**, and **cardiac involvement**.

Question 1

All of the following are indications for use of corticosteroids in SLE except:

Accepted Answer

Endocarditis

Answer

Nephritic syndrome

Answer

Pericarditis

Answer

Neuropsychiatric lupus

Question 2

Most specific test for SLE

Accepted Answer

Anti- smith antibody

Answer

ds- DNA

Answer

ss DNA

Answer

Histone

Question 3

Symmetrical persistent enlargement of the parotid gland is seen in:

Accepted Answer

Mikulicz disease.

Answer

Sjögren syndrome.

Answer

Cylindroma.

Answer

All of the options.

Question 4

Which is NOT a feature of polymyositis?

Accepted Answer

Ocular muscle involvement

Answer

Proximal muscle weakness

Answer

Elevated serum creatine kinase (CK) levels

Answer

Endomysial inflammation on muscle biopsy

Question 5

A 65-year-old patient presents with severe headache, temporal artery tenderness, and decreased pulse. What is the most likely diagnosis?

Accepted Answer

Giant cell arteritis

Answer

Wegener's granulomatosis

Answer

Microscopic polyangiitis

Answer

Takayasu arteritis

Question 6

Bamboo spine with sacroilitis -

Accepted Answer

Ankylosing spondylitis

Answer

Psoriatic arthritis

Answer

OA

Answer

RA

Question 7

A 23-year-old male patient presented with a history of back pain, which is more in the morning and relieved by bathing in warm water. What is the likely additional finding present in this patient?

Accepted Answer

Decreased chest wall expansion

Answer

Marrow fibrosis

Answer

Pleural nodules

Answer

Distal phalangeal joint involvement

Question 8

A 25-year-old man complains of low backache, decreased lumbar movements, morning stiffness, which clinical examination will further help:

Accepted Answer

Chest expansion

Answer

Head circumference

Answer

Plantar arch

Answer

Hyperextension of joints

Question 9

Extra articular manifestation of rheumatoid arthritis is:

Accepted Answer

Subcutaneous nodules

Answer

Av fistula

Answer

Splenic atrophy

Answer

Cutaneous hypomelanosis

Question 10

Limited cutaneous systemic sclerosis have the following characteristic autoantibody positivity:

Accepted Answer

Anticentromere

Answer

Anti-RNA polymerase III

Answer

Anti Topoisomerase I

Answer

Anti-U3-RNP (fibrillarin)

Rheumatology and Immunology — MCQs

Rheumatology and Immunology — MCQs

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