Rheumatology and Immunology Practice Questions

Q: Which of the following conditions is most commonly associated with disease activity in Systemic Lupus Erythematosus (SLE)?

Lymphopenia. ***Lymphopenia*** - **Lymphopenia** (low lymphocyte count) is the most common hematological manifestation associated with active SLE, occurring in approximately 50-80% of patients [1]. - It is often seen in individuals with active disease due to increased peripheral destruction of lymphocytes or their sequestration and redistribution. *Autoimmune Hemolytic anemia* - While **autoimmune hemolytic anemia (AIHA)** can occur in SLE, it is less common than lymphopenia, affecting about 5-10% of patients [1]. - AIHA is characterized by the destruction of red blood cells by autoantibodies, leading to **anemia**. *Thrombocytopenia* - **Thrombocytopenia** (low platelet count) is another hematologic manifestation of SLE, occurring in 10-25% of patients [1]. - It is caused by autoantibodies directed against platelets, leading to their premature destruction. *Neutropenia* - **Neutropenia** (low neutrophil count) is observed in about 10-15% of SLE patients. - Although it can be a sign of active disease, it is less frequent than lymphopenia [1].

Q: A 20-year-old female has an erythematous rash over her face, including both cheeks and the bridge of her nose, which is exacerbated by sunlight exposure when she is outdoors. In addition to the rash, she has experienced muscle and joint pain for several months. Although radiographs of the joints do not reveal any abnormalities, she has normal joint mobility without deformity. Which laboratory test finding is most characteristic of systemic lupus erythematosus (SLE)?

Antibodies to double-stranded DNA. A 20-year-old female has an erythematous rash over her face, including both cheeks and the bridge of her nose, which is exacerbated by sunlight exposure [1]. In addition to the rash, she has experienced muscle and joint pain for several months. Although radiographs of the joints do not reveal any abnormalities, she has normal joint mobility without deformity. Which laboratory test finding is most characteristic of systemic lupus erythematosus (SLE)? ***Antibodies to double-stranded DNA*** - In **systemic lupus erythematosus (SLE)** [2], which fits the description, the presence of **anti-double-stranded DNA antibodies** is a hallmark finding. - This antibody is associated with **renal involvement** and correlates with disease activity, making it a critical laboratory finding [2]. *Elevated anti-streptolysin 0 (ASO) titer* - This test indicates recent **streptococcal infection**, not related to the symptoms or autoimmune features of SLE. - It is mainly used in the context of **rheumatic fever** and **post-streptococcal glomerulonephritis**. *Markedly decreased serum level of immunoglobulin G (IgG)* - Decreased IgG levels do not specifically indicate **SLE**, which often shows **increased overall immunoglobulin levels** due to chronic inflammation. - Hypogammaglobulinemia can occur in various conditions but is not characteristic of lupus. *HLA-B27 genotype* - This genotype is primarily associated with **spondyloarthropathies** (e.g., ankylosing spondylitis), not systemic lupus erythematosus [2]. - It is not relevant to the **cutaneous symptoms** or the autoimmune nature of the presented disease.

Q: In ankylosing spondylitis, joint involvement is least in?

Wrist and hand. Detailed explanation of joint involvement in ankylosing spondylitis: ***Wrist and hand*** - **Ankylosing spondylitis (AS)** primarily affects the **axial skeleton** (spine, sacroiliac joints) and **large peripheral joints** like the hips and shoulders. - Involvement of the small joints of the **wrist and hand** is uncommon [1] and usually occurs late in the disease course, making it the least involved area among the options. *Sacroiliac joint* - The **sacroiliac joints** are almost universally affected in AS, often being the **first joints** to show inflammation [2]. - Inflammatory changes and eventual **fusion** of these joints are hallmarks of the disease [2]. *Costochondral junction* - Involvement of the **costochondral junctions** can lead to **chest wall pain** and stiffness, contributing to the typical "bamboo spine" appearance and reduced chest expansion in AS [2]. - This is a common extra-axial manifestation of the disease, contributing to respiratory compromise. *Acromio-clavicular joint* - The **acromio-clavicular joints** can be affected in AS, causing pain and restricted movement in the shoulder girdle [1]. - This is considered a common site of **peripheral enthesitis** and articular involvement in AS [1].

Q: A person presents with recurrent swelling on the face and lips due to emotional stress. The likely cause is?

C1 esterase inhibitor deficiency. C1 esterase inhibitor deficiency - This condition presents with recurrent episodes of angioedema (swelling of face, lips, airway, or gastrointestinal tract) that are often triggered by stress, trauma, or medical procedures. - The deficiency leads to unregulated activation of the complement and kallikrein-kinin pathways, resulting in excessive bradykinin production which causes increased vascular permeability and swelling. Allergy - Allergic reactions typically involve hives (urticaria) and itching, which are usually absent in C1 esterase inhibitor deficiency. - Allergic angioedema is often mediated by histamine and resolves with antihistamines or epinephrine, which are ineffective in C1 esterase inhibitor deficiency [2]. Anaphylaxis - Anaphylaxis is a severe, life-threatening allergic reaction that includes symptoms such as respiratory distress, hypotension, and generalized urticaria, in addition to angioedema [2]. - While angioedema can be a feature, the constellation of symptoms and the common triggers differ significantly from C1 esterase inhibitor deficiency [2]. ACE inhibitor-induced angioedema - This type of angioedema is a known side effect of ACE inhibitor medications and usually occurs after beginning the medication [1]. - It is also caused by increased bradykinin levels but is directly linked to the drug's action, not an underlying genetic deficiency, and typically resolves upon discontinuation of the medication.

Q: All of the following are true about Drug-Induced Lupus in comparison to Idiopathic SLE, except:

More common in females. ***More common in females*** - This statement is **false** for drug-induced lupus (DIL). While **idiopathic SLE** has a significant female predominance, DIL shows a **more even gender distribution** or a slightly higher incidence in males depending on the drug [1]. - Thus, DIL being *more common in females* compared to idiopathic SLE is incorrect; it is actually less pronounced. *Milder disease* - Drug-induced lupus (DIL) is generally characterized by a **milder clinical course** compared to idiopathic SLE. - Symptoms often resolve following cessation of the offending drug. *Presence of renal or CNS disease is rare* - **Major organ involvement**, such as severe **renal** (nephritis) or **central nervous system (CNS)** disease, is rare in drug-induced lupus. - These severe manifestations are characteristic of idiopathic SLE [1]. *Hypocomplementemia is less common* - **Hypocomplementemia** (low C3 and C4 levels) is a common finding in active idiopathic SLE, reflecting immune complex activity. - In contrast, **drug-induced lupus** typically presents with normal complement levels.

Q: Which of the following is an appropriate next step in the management of this patient?

Perform the McRoberts maneuver. ***Perform the McRoberts maneuver*** - The **McRoberts maneuver** is the critical first-line intervention for **shoulder dystocia**, involving hyperflexion of the mother's hips towards her abdomen to straighten the sacrum and rotate the symphysis pubis superiorly. - This maneuver is effective in increasing the functional diameter of the pelvis and dislodging the impacted anterior shoulder from behind the pubic symphysis, with a success rate of up to 42%. *Apply fundal pressure* - **Fundal pressure** is generally **contraindicated** in cases of shoulder dystocia as it can worsen the impaction of the fetal shoulder and potentially lead to uterine rupture or fetal injury. - By pushing the fetus deeper into the birth canal, it can increase the risk of brachial plexus injury or other fetal trauma. *Request additional assistance* - While requesting additional assistance is important for good teamwork and ensures adequate help is available for any further maneuvers or complications, it is not the *immediate* mechanical step for resolving the dystocia. - The initial mechanical steps, such as the McRoberts maneuver, should be initiated promptly while help is en route. *Perform a generous episiotomy* - An **episiotomy** in shoulder dystocia does not typically facilitate the release of the impacted shoulder because the obstruction is bony, not soft tissue. - While it may create more space for the operator's hands to perform internal maneuvers, it does not directly resolve the bony impaction and is not considered a primary intervention.

Q: Normal CRP with elevated ESR is seen in:

SLE. ***SLE*** - In **Systemic Lupus Erythematosus (SLE)**, the **ESR** is often significantly elevated due to hypergammaglobulinemia and increased fibrinogen, reflecting systemic inflammation [2]. - However, the **CRP** may be normal or only mildly elevated, which is a distinguishing feature compared to other inflammatory conditions [1], [2]. *RA* - In **Rheumatoid Arthritis (RA)**, both **CRP and ESR** are typically elevated, reflecting the acute phase response due to active inflammation. - An isolated elevated ESR with a normal CRP is less common in active RA and would prompt consideration of alternative or co-existing conditions. *Scleroderma* - For **Scleroderma (Systemic Sclerosis)**, the **ESR and CRP** are often normal or only mildly elevated, even in the presence of extensive organ involvement [1]. - Significant elevations in both ESR and CRP should prompt a search for alternative diagnoses or complications such such as an infection. *Polymyalgia rheumatica* - **Polymyalgia Rheumatica (PMR)** is characterized by marked inflammation, leading to a **significantly elevated ESR and CRP** [2]. - A normal CRP with an elevated ESR would be atypical for PMR, which is primarily diagnosed based on clinical symptoms and this distinct inflammatory marker pattern.

Q: Which type of arthritis is commonly associated with tarsal tunnel syndrome?

Rheumatoid arthritis. Rheumatoid arthritis (RA) is a systemic inflammatory disease that can lead to synovitis and inflammation of tendons, which can compress the posterior tibial nerve in the tarsal tunnel [1]. The chronic inflammation and proliferation of the synovial tissue around the ankle can directly impinge on the nerve, causing tarsal tunnel syndrome [1]. *Ankylosing spondylitis* - This is a chronic inflammatory disease primarily affecting the axial skeleton, particularly the spine and sacroiliac joints. - While it can have peripheral manifestations, it does not typically cause tarsal tunnel syndrome through direct nerve compression from inflammation in the ankle joint. *Osteoarthritis* - Osteoarthritis (OA) is a degenerative joint disease characterized by cartilage breakdown and bone spur formation. - While OA can affect the ankle, it is less directly associated with the inflammatory changes that lead to tarsal tunnel syndrome compared to RA. *Psoriatic arthritis* - Psoriatic arthritis (PsA) is an inflammatory arthritis associated with psoriasis, often involving joints of the hands, feet, and spine. - Although PsA can cause inflammation in the foot and ankle, it is not as commonly linked to tarsal tunnel syndrome as rheumatoid arthritis.

Q: A 35-year-old woman complains of aching all over, with symptoms that have progressed over several years, and reports pain and weakness causing her to drop things. Physical examination reveals multiple points of tenderness over the neck, shoulders, elbows, and wrists, with no joint swelling or deformity. Laboratory tests, including complete blood count and ESR, are normal, and rheumatoid factor is negative. Which is the best therapeutic option for this patient?

Graded aerobic exercise. Graded aerobic exercise - This patient's symptoms (widespread aching, tenderness at multiple points, normal labs) are classic for fibromyalgia [1], [3]. - Graded aerobic exercise is a cornerstone of fibromyalgia treatment, improving pain, fatigue, and functional capacity [2]. Prednisone - Prednisone is a corticosteroid used for inflammatory conditions, but fibromyalgia is not primarily an inflammatory disease [3]. - Long-term use can lead to significant side effects without long-term improvement in fibromyalgia. Weekly methotrexate - Methotrexate is a disease-modifying antirheumatic drug (DMARD) used for inflammatory arthritides like rheumatoid arthritis, which is ruled out by the negative rheumatoid factor and absence of joint swelling [3]. - It has no proven efficacy for fibromyalgia and carries significant risks of toxicity. Hydroxychloroquine - Hydroxychloroquine is an antimalarial drug also used as a DMARD for conditions like lupus and rheumatoid arthritis. - It is not indicated for the treatment of fibromyalgia and would not address the patient's symptoms effectively.

Q: In Familial Mediterranean fever, the gene encoding for which of the following proteins undergoes mutation?

Pyrin. ***Pyrin*** - Familial Mediterranean fever (FMF) is an **autoinflammatory disease** caused by mutations in the **MEFV gene**, which encodes the protein pyrin. - Pyrin plays a crucial role in regulating inflammation by modulating the **NLRP3 inflammasome**; mutations lead to uncontrolled inflammatory responses. *Transthyretin* - Mutations in the gene for **transthyretin** are associated with **hereditary amyloidosis**, particularly transthyretin amyloidosis (ATTR), which affects various organs. - This protein primarily functions in transporting **thyroid hormones** and **retinol**, and its misfolding leads to amyloid deposition, not FMF. *Perforin* - Mutations in the **perforin 1 gene (PRF1)** are linked to **familial hemophagocytic lymphohistiocytosis (HLH)**, a severe immunodeficiency disorder. - Perforin is a cytolytic protein crucial for T-cell and NK-cell mediated cytotoxicity, unrelated to FMF. *Immunoglobulin light chain* - Abnormal production or accumulation of **immunoglobulin light chains** is characteristic of **AL amyloidosis** (primary amyloidosis), where light chains misfold and deposit as amyloid fibrils. - This is a plasma cell dyscrasia and is not associated with the genetic cause of FMF.

Question 1

Which of the following conditions is most commonly associated with disease activity in Systemic Lupus Erythematosus (SLE)?

Accepted Answer

Lymphopenia

Answer

Neutropenia

Answer

Thrombocytopenia

Answer

Autoimmune Hemolytic anemia

Question 2

A 20-year-old female has an erythematous rash over her face, including both cheeks and the bridge of her nose, which is exacerbated by sunlight exposure when she is outdoors. In addition to the rash, she has experienced muscle and joint pain for several months. Although radiographs of the joints do not reveal any abnormalities, she has normal joint mobility without deformity. Which laboratory test finding is most characteristic of systemic lupus erythematosus (SLE)?

Accepted Answer

Antibodies to double-stranded DNA

Answer

Elevated anti-streptolysin 0 (ASO) titer

Answer

HLA-B27 genotype

Answer

Markedly decreased serum level of immunoglobulin G (IgG)

Question 3

In ankylosing spondylitis, joint involvement is least in?

Accepted Answer

Wrist and hand

Answer

Costochondral junction

Answer

Sacroiliac joint

Answer

Acromio-clavicular joint

Question 4

A person presents with recurrent swelling on the face and lips due to emotional stress. The likely cause is?

Accepted Answer

C1 esterase inhibitor deficiency

Answer

Allergy

Answer

Anaphylaxis

Answer

ACE inhibitor-induced angioedema

Question 5

All of the following are true about Drug-Induced Lupus in comparison to Idiopathic SLE, except:

Accepted Answer

More common in females

Answer

Hypocomplementemia is less common

Answer

Milder disease

Answer

Presence of renal or CNS disease is rare

Question 6

Which of the following is an appropriate next step in the management of this patient?

Accepted Answer

Perform the McRoberts maneuver

Answer

Request additional assistance

Answer

Apply fundal pressure

Answer

Perform a generous episiotomy

Question 7

Normal CRP with elevated ESR is seen in:

Accepted Answer

SLE

Answer

RA

Answer

Polymyalgia rheumatica

Answer

Scleroderma

Question 8

Which type of arthritis is commonly associated with tarsal tunnel syndrome?

Accepted Answer

Rheumatoid arthritis

Answer

Ankylosing spondylitis

Answer

Osteoarthritis

Answer

Psoriatic arthritis

Question 9

A 35-year-old woman complains of aching all over, with symptoms that have progressed over several years, and reports pain and weakness causing her to drop things. Physical examination reveals multiple points of tenderness over the neck, shoulders, elbows, and wrists, with no joint swelling or deformity. Laboratory tests, including complete blood count and ESR, are normal, and rheumatoid factor is negative. Which is the best therapeutic option for this patient?

Accepted Answer

Graded aerobic exercise

Answer

Prednisone

Answer

Weekly methotrexate

Answer

Hydroxychloroquine

Question 10

In Familial Mediterranean fever, the gene encoding for which of the following proteins undergoes mutation?

Accepted Answer

Pyrin

Answer

Transthyretin

Answer

Perforin

Answer

Immunoglobulin light chain

Rheumatology and Immunology — MCQs

Rheumatology and Immunology — MCQs

On this page

Practice by Chapter

Want unlimited practice?