Rheumatology and Immunology Practice Questions

Q: Lady presents with joint pain in both knees and low-grade fever, which occurs intermittently. On examination, she has a rash on sun-exposed areas. What is the clinical diagnosis?

Systemic lupus erythematosus (SLE). ***Systemic lupus erythematosus (SLE)*** - The combination of **polyarthralgia (joint pain)**, **low-grade fever**, and a **photosensitive rash** is highly characteristic of SLE. [1] - SLE is a **multisystem autoimmune disease** that can affect joints, skin, and cause constitutional symptoms like fever. [1] *Rheumatoid arthritis (RA)* - While RA causes **joint pain**, it typically presents with **symmetrical polyarthritis** primarily affecting small joints, and a photosensitive rash is not a common feature. [2] - **Fever** can be present in severe RA, but the triad of symptoms in this case points away from RA as the primary diagnosis. *Photodermatitis* - **Photodermatitis** is a skin inflammation caused by sunlight exposure, resulting in a rash. - This diagnosis does not account for the **joint pain** or **low-grade fever**, which are systemic manifestations. *Porphyria cutanea tarda* - This condition primarily affects the skin, causing **photosensitivity**, blistering lesions, and increased fragility, especially on sun-exposed areas. - It does **not typically present with joint pain or fever**, differentiating it from the presented symptoms.

Q: A 60-year-old female presenting with proximal muscle weakness, increased serum creatine kinase levels, and no skin manifestations is likely to have:

Polymyositis. ***Polymyositis*** - **Polymyositis** primarily affects adults, causing **proximal muscle weakness** (shoulders, hips, thighs) and elevated **creatine kinase (CK)** levels due to muscle damage [1]. - The absence of **skin manifestations** in this case strongly supports polymyositis over dermatomyositis, as dermatomyositis typically presents with characteristic cutaneous features [1]. *Dermatomyositis* - While dermatomyositis can present with identical **proximal muscle weakness** and elevated CK levels, it is characteristically associated with **skin manifestations** [1]. - The absence of **heliotrope rash**, **Gottron papules**, or **shawl sign** in this clinical scenario makes dermatomyositis less likely despite similar muscle involvement [1]. *Limb-girdle muscular dystrophy* - **Limb-girdle muscular dystrophy** is a genetically heterogeneous group of disorders, typically presenting in **childhood or adolescence**, though some forms can manifest later in life. - It causes **progressive symmetric muscle weakness** predominantly affecting the **shoulders and hips**, with highly variable CK levels depending on the specific genetic mutation. *Inclusion body myositis* - **Inclusion body myositis** typically affects older individuals (over 50), presenting with **progressive, asymmetrical muscle weakness**, more prominently in the **distal muscles** (e.g., forearm flexors, quadriceps). - While CK levels can be elevated, they are usually **less markedly increased** than in polymyositis or dermatomyositis, and the disease often progresses more slowly.

Q: Which of the following is a definite indication for systemic steroids in rheumatoid arthritis?

b) Lung involvement. ***Lung involvement*** - **Systemic steroids** are indicated for severe **extrapulmonary manifestations** of rheumatoid arthritis, such as **interstitial lung disease**, which can be life-threatening [2]. - They help to reduce **inflammation** and prevent further damage to lung tissue [1]. *Mononeuritis multiplex* - While a serious complication of rheumatoid arthritis, **mononeuritis multiplex** typically warrants treatment with disease-modifying antirheumatic drugs (DMARDs) and sometimes **intravenous immunoglobulins** (IVIG) or **cyclophosphamide**, rather than systemic steroids as a primary or definite indication. - Steroids might be used as an adjunct, but are not the definite first-line sole treatment. *Carpal tunnel syndrome* - **Carpal tunnel syndrome** in rheumatoid arthritis is often managed with **local corticosteroid injections**, **splinting**, or surgical release, depending on severity. - **Systemic steroids** are generally not indicated for localized musculoskeletal complications unless there is overwhelming active systemic disease. *Both 'a' & 'c'* - This option is incorrect because neither **mononeuritis multiplex** nor **carpal tunnel syndrome** is a definite standalone indication for systemic steroids in rheumatoid arthritis, as explained above. - Systemic steroids are reserved for more severe or diffuse manifestations.

Q: Behçet's disease is associated with which HLA?

HLA-B51. ***HLA-B51*** - Behçet's disease has a strong association with the **HLA-B51 allele**, particularly in populations from the Mediterranean, Middle East, and Asia. - This genetic predisposition contributes to the inflammatory and autoimmune nature of the disease. *HLA-B27* - This allele is strongly associated with **spondyloarthropathies**, such as **ankylosing spondylitis** and **reactive arthritis**, rather than Behçet's disease [1]. - Patients with HLA-B27 positive status often present with axial skeletal involvement and enthesitis [1]. *HLA-DQ* - HLA-DQ alleles are associated with various autoimmune conditions, including **celiac disease** and **type 1 diabetes mellitus**, but not specifically with Behçet's disease. - The association is generally weaker and less specific compared to HLA-B51 for Behçet's. *HLA-DR4* - **HLA-DR4** is primarily associated with **rheumatoid arthritis**, especially in severe forms of the disease. - It is also associated with other autoimmune diseases but does not play a significant role in the pathogenesis or diagnosis of Behçet's disease.

Q: Juvenile idiopathic arthritis includes all the following categories except:

Reactive arthritis. ***Reactive arthritis*** - **Reactive arthritis** is not a subtype of Juvenile Idiopathic Arthritis (JIA); instead, it is a **spondyloarthritis** that typically develops after an infection. - While it can occur in children, its pathogenesis and diagnostic criteria differentiate it from the various JIA categories. *Psoriatic arthritis* - **Psoriatic arthritis** is indeed a recognized category of JIA, characterized by arthritis accompanied by psoriasis or a family history of psoriasis. - It often presents with **dactylitis** (sausage digits) and nail changes in addition to joint involvement. *Enthesitis related arthritis* - **Enthesitis-related arthritis** is a JIA subtype primarily affecting the **entheses** (sites where tendons or ligaments attach to bone) and often the axial skeleton. - It is frequently associated with **HLA-B27 positivity** and occurs more commonly in boys. *Systemic arthritis* - **Systemic arthritis** (formerly Still's disease) is a distinct JIA category characterized by **daily fevers**, skin rash, and internal organ involvement such as hepatosplenomegaly, lymphadenopathy, and serositis. - Arthritis may develop later in the disease course or be less prominent initially compared to systemic symptoms.

Q: What is the primary condition associated with atrophic changes in the mucous membranes?

Sjogren's syndrome. ***Sjogren's syndrome*** - It is an **autoimmune disease** primarily characterized by the destruction of exocrine glands, leading to **dryness of mucous membranes** (xerostomia, xerophthalmia) [1]. - The chronic inflammation and lymphocytic infiltration of glands result in **atrophy and dysfunction**, particularly of the salivary and lacrimal glands. *Lupus erythematosus* - While it is an autoimmune disease that can affect multiple organs, its primary manifestation is not typically **atrophic changes in mucous membranes**. [1] - **Oral ulcers** and **photosensitivity** are common, but widespread glandular atrophy is not characteristic [2]. *Celiac disease* - This is an **autoimmune disorder** primarily affecting the **small intestine** due to gluten ingestion, leading to **villous atrophy**. - While it can cause some oral manifestations (e.g., aphthous ulcers), it does not directly cause widespread atrophy of mucous membranes in other parts of the body. *Vitamin B12 deficiency* - Can cause **glossitis** (inflammation of the tongue), which might appear smooth and atrophic due to the loss of papillae. - However, it does not typically lead to systemic atrophy of mucous membranes beyond specific areas like the tongue or a general dryness.

Q: A child presented with non-blanching purpura in the lower limb and recurrent abdominal pain. Which condition is characterized by the deposition of IgA?

Henoch-Schönlein purpura. ***Henoch-Schönlein purpura*** - This condition is a **leukocytoclastic vasculitis** [1] primarily affecting small vessels, characterized by palpable **purpura**, **abdominal pain** [2], and often **arthralgia** and **renal involvement**. - It is defined by the deposition of **IgA immune complexes** in affected small vessels, a key diagnostic feature. *Granulomatosis with polyangiitis* - This is an **ANCA-associated vasculitis** [1] characterized by granulomatous inflammation of the respiratory tract and necrotizing vasculitis, typically affecting small to medium-sized vessels. - It is associated with **antineutrophil cytoplasmic antibodies (ANCA)**, specifically **c-ANCA**, and does not involve IgA deposition. *Kawasaki syndrome* - This is an **acute febrile illness** primarily in young children [3], characterized by inflammation of medium-sized arteries, most notably the **coronary arteries**. - It presents with fever, rash, **conjunctivitis**, and **lymphadenopathy**, but not IgA deposition [3]. *Takayasu arteritis* - This is a **large-vessel vasculitis** [1] primarily affecting the **aorta** and its major branches, leading to stenoses, occlusions, and aneurysms. - It usually presents in young women with symptoms related to **ischemia** of affected organs, and is not associated with IgA deposition or purpura in the lower limbs.

Q: Which of the following is a feature of rheumatoid arthritis?

Symmetric joint involvement with erosive changes. ***Symmetric joint involvement with erosive changes*** - **Rheumatoid arthritis** is characterized by **symmetric polyarthritis**, meaning it affects the same joints on both sides of the body [1]. - The chronic inflammation leads to the destruction of cartilage and bone, resulting in **erosive changes** visible on radiographs [1]. *Heberden nodes* - **Heberden nodes** are characteristic bony enlargements of the **distal interphalangeal (DIP) joints**, typically seen in **osteoarthritis** [1]. - They are not a feature of **rheumatoid arthritis**, which primarily affects the metacarpophalangeal (MCP) and proximal interphalangeal (PIP) joints [1]. *Swan neck deformity* - **Swan neck deformity** is a classic hand deformity seen in **rheumatoid arthritis**, characterized by hyperextension of the PIP joint and flexion of the DIP joint. - While it is a feature of chronic rheumatoid arthritis, it represents an advanced stage of the disease and is not a defining characteristic of the initial presentation of RA. *Ulnar deviation of fingers at metacarpophalangeal joint* - **Ulnar deviation** is a common deformity in **rheumatoid arthritis**, where the fingers drift towards the ulnar side at the **metacarpophalangeal (MCP) joints**. - This is a clinical manifestation of the disease, but **symmetric joint involvement with erosive changes** is a more fundamental and diagnostic feature.

Q: Which of the following conditions is not associated with antiphospholipid syndrome?

Thrombocytosis. ***Thrombocytosis*** - **Antiphospholipid syndrome (APS)** is characterized by increased **thrombosis** (clotting), not an increase in platelet count (thrombocytosis). - Patients with APS may experience **thrombocytopenia** (low platelet count), which is distinct from thrombocytosis [1]. *Venous thrombosis* - **Venous thrombosis**, particularly deep vein thrombosis (DVT) and pulmonary embolism (PE), is a hallmark feature of APS [2]. - The presence of **antiphospholipid antibodies** promotes a procoagulant state, leading to clot formation in veins. *Recurrent foetal loss* - **Recurrent foetal loss**, including miscarriages and stillbirths, is a classic obstetric manifestation of APS. - The antibodies interfere with placental function and blood supply, leading to pregnancy complications. *Neurological manifestations* - Various **neurological manifestations** are associated with APS, including stroke, transient ischemic attacks (TIAs), and cognitive dysfunction. - These are often due to **microthrombosis** in the cerebral vasculature or direct antibody effects.

Question 1

Lady presents with joint pain in both knees and low-grade fever, which occurs intermittently. On examination, she has a rash on sun-exposed areas. What is the clinical diagnosis?

Accepted Answer

Systemic lupus erythematosus (SLE)

Answer

Rheumatoid arthritis (RA)

Answer

Photodermatitis

Answer

Porphyria cutanea tarda

Question 2

A 60-year-old female presenting with proximal muscle weakness, increased serum creatine kinase levels, and no skin manifestations is likely to have:

Accepted Answer

Polymyositis

Answer

Dermatomyositis

Answer

Limb-girdle muscular dystrophy

Answer

Inclusion body myositis

Question 3

Which of the following is a definite indication for systemic steroids in rheumatoid arthritis?

Accepted Answer

b) Lung involvement

Answer

a) Mononeuritis multiplex

Answer

c) Carpal tunnel syndrome

Answer

d) Both 'a' & 'c'

Question 4

Behçet's disease is associated with which HLA?

Accepted Answer

HLA-B51

Answer

HLA-B27

Answer

HLA-DQ

Answer

HLA-DR4

Question 5

Juvenile idiopathic arthritis includes all the following categories except:

Accepted Answer

Reactive arthritis

Answer

Psoriatic arthritis

Answer

Enthesitis related arthritis

Answer

Systemic arthritis

Question 6

What is the primary condition associated with atrophic changes in the mucous membranes?

Accepted Answer

Sjogren's syndrome

Answer

Lupus erythematosus

Answer

Celiac disease

Answer

Vitamin B12 deficiency

Question 7

What are the common complications associated with hypogammaglobulinemia?

Accepted Answer

Chronic recurrent sinusitis due to impaired antibody production

Answer

Nosebleeds due to vascular issues

Answer

Muscle contractures unrelated to immune function

Answer

Eczema as a result of immune dysregulation

Question 8

A child presented with non-blanching purpura in the lower limb and recurrent abdominal pain. Which condition is characterized by the deposition of IgA?

Accepted Answer

Henoch-Schönlein purpura

Answer

Granulomatosis with polyangiitis

Answer

Kawasaki syndrome

Answer

Takayasu arteritis

Question 9

Which of the following is a feature of rheumatoid arthritis?

Accepted Answer

Symmetric joint involvement with erosive changes

Answer

Swan neck deformity

Answer

Heberden nodes

Answer

Ulnar deviation of fingers at metacarpophalangeal joint

Question 10

Which of the following conditions is not associated with antiphospholipid syndrome?

Accepted Answer

Thrombocytosis

Answer

Venous thrombosis

Answer

Neurological manifestations

Answer

Recurrent foetal loss

Rheumatology and Immunology — MCQs

Rheumatology and Immunology — MCQs

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