Rheumatology and Immunology Practice Questions

Q: Which of the following has a male:female ratio of 1:1?

Behçet's disease. ***Behçet's disease*** - **Behçet's disease** is notable for its roughly equal incidence in males and females, giving it a **1:1 male:female ratio**. - It is a **systemic vasculitis** affecting multiple organs, characterized by recurrent oral and genital ulcers, skin lesions, and ocular involvement [1]. *Polymyalgia rheumatica* - This condition predominantly affects **women**, with a female-to-male ratio of approximately **2:1 or even higher**. - It is an inflammatory disorder causing muscle pain and stiffness, primarily in the shoulders and hips, and is closely associated with **giant cell arteritis**. *Gout* - **Gout** is significantly more common in **men**, especially before menopause in women, with a male-to-female ratio that can be as high as **7-9:1** [2]. - It is an inflammatory arthritis caused by the deposition of **urate crystals** in joints, leading to sudden, severe attacks of pain, redness, and swelling [2]. *Rheumatoid arthritis* - **Rheumatoid arthritis** is more prevalent in **women**, typically with a female-to-male ratio of about **2-3:1**. - It is a **chronic autoimmune inflammatory disease** primarily affecting the synovial lining of joints, leading to progressive joint destruction and systemic complications [3].

Q: Which feature best differentiates ANCA-associated vasculitis from anti-GBM disease?

Relapsing course. ### Relapsing course - ANCA-associated vasculitis (AAV) often has a **relapsing-remitting course**, meaning patients can experience periods of active disease followed by remission, and then subsequent flares [1]. - In contrast, **anti-glomerular basement membrane (anti-GBM) disease** is typically a monophasic illness without spontaneous relapses once treated. *Crescentic glomerulonephritis* - Both ANCA-associated vasculitis and anti-GBM disease can cause **crescentic glomerulonephritis**, which is a rapid, progressive inflammatory process in the glomeruli [2, 3]. - This feature is characteristic of **rapidly progressive glomerulonephritis (RPGN)** and does not differentiate between the two conditions [2]. *Rapid progression* - Both ANCA-associated vasculitis and anti-GBM disease are known for their **rapid progression** to end-stage renal disease if left untreated [1]. - This similarity highlights their severe nature but does not serve as a distinguishing factor. *Pulmonary hemorrhage* - **Pulmonary hemorrhage (diffuse alveolar hemorrhage)** can occur in both ANCA-associated vasculitis (especially in GPA and MPA) and anti-GBM disease (Goodpasture's syndrome) [2, 3]. - This overlapping manifestation means it cannot be used to differentiate between the two conditions.

Q: A 40-year-old with chronic hepatitis B develops fever, arthralgia, and palpable purpura. Most likely diagnosis?

Polyarteritis nodosa. ***Polyarteritis nodosa*** - The triad of **fever**, **arthralgia**, and **palpable purpura** in a patient with **chronic hepatitis B** is highly suggestive of polyarteritis nodosa [1]. - **Hepatitis B infection** is strongly associated with polyarteritis nodosa due to the formation of **immune complexes** that deposit in vessel walls, leading to inflammation and vasculitis [1]. *IgA vasculitis* - Typically presents with **palpable purpura**, arthralgia, and abdominal pain, often preceded by an **upper respiratory tract infection** [1]. - It is more common in **children** and is not typically linked to chronic hepatitis B infection [1]. *Wegener's granulomatosis* - Characterized by **granulomatous inflammation** of the upper and lower respiratory tracts, **glomerulonephritis**, and systemic vasculitis. - It is associated with **anti-neutrophil cytoplasmic antibodies (c-ANCA)**, which are not suggested by the clinical picture or hepatitis B status. *Cryoglobulinemia* - Often presents with **palpable purpura**, arthralgia, and neuropathy, and is strongly associated with **hepatitis C infection**. - While hepatitis B can rarely be a cause, hepatitis C is the dominant viral association with **mixed cryoglobulinemia**.

Q: 30-year male with recurrent sinopulmonary infections has low serum IgA. Most likely diagnosis?

Selective IgA deficiency. ***Selective IgA deficiency*** - This condition is characterized by **recurrent sinopulmonary infections** and **low serum IgA levels** while other immunoglobulin levels are typically normal [1]. - Due to IgA's role in mucosal immunity, its deficiency predisposes to infections of the respiratory and gastrointestinal tracts [1]. *X-linked agammaglobulinemia* - This disorder is marked by a severe deficiency of **all immunoglobulin classes** (IgG, IgA, IgM) due to a defect in **B-cell maturation** [1]. - Patients typically present with severe recurrent bacterial infections in **early infancy**, which is earlier than the age of presentation in this case (30-year-old male) [1]. *Hyper IgM syndrome* - This condition is characterized by **normal or elevated IgM levels** but **low IgG, IgA, and IgE levels**, resulting from a defect in **class switching**. - It often involves recurrent infections, but the specific pattern of immunoglobulin levels differs significantly from the description. *Common variable immunodeficiency* - This is a heterogeneous disorder characterized by **low levels of IgG** (and often IgA and/or IgM) leading to recurrent infections [1]. - Unlike selective IgA deficiency, CVID involves a broader defect in humoral immunity, with significant reductions in **IgG** in addition to IgA [1].

Q: A 50-year-old with rheumatoid arthritis has been on long-term NSAIDs. Which parameter should be monitored to assess for potential complications of NSAID use?

Urine protein levels. ***Urine protein levels*** - Long-term use of **NSAIDs** can lead to **renal damage**, including **interstitial nephritis** [3] and **papillary necrosis**, manifesting as **proteinuria** [2]. - Monitoring urine protein helps detect early signs of NSAID-induced nephropathy. *Erythrocyte sedimentation rate (ESR)* - **ESR** is a general marker of **inflammation** and disease activity in rheumatoid arthritis, not a specific indicator of NSAID complications. - While important for monitoring rheumatoid arthritis itself, it does not directly assess adverse effects of NSAID therapy. *Serum potassium* - While NSAIDs can sometimes cause **hyperkalemia** by inhibiting prostaglandin synthesis [4], which affects renal potassium excretion, this is less common and less specific than **proteinuria** as a primary monitoring parameter for general NSAID complications. - **Hyperkalemia** is usually associated with other risk factors like pre-existing renal insufficiency or concomitant use of certain medications. *Complete blood count (CBC)* - NSAIDs can sometimes cause **gastrointestinal bleeding**, leading to **anemia** [1], which would be reflected in a **CBC**. - However, **gastrointestinal complications** are more often monitored by symptoms (e.g., melena, abdominal pain) or endoscopy, and **renal effects** are a more direct and common long-term complication often requiring specific laboratory monitoring like urine protein.

Q: Most specific for diagnosing Ankylosing spondylitis?

B/l sacroilitis. ***B/l sacroiliitis*** - **Bilateral sacroiliitis** on imaging (X-ray or MRI) is the **hallmark** and most specific diagnostic criterion for ankylosing spondylitis, as it represents the primary pathology [1]. - While other features may suggest AS, definitive imaging evidence of inflammation and structural changes in the **sacroiliic joints** confirms the diagnosis [1]. *HLA B27* - **HLA-B27** is present in a high percentage of patients with ankylosing spondylitis but is not *specific*; it can be found in healthy individuals and other spondyloarthropathies [1]. - Its presence increases the *susceptibility* but does not confirm the diagnosis on its own [1]. *ESR* - **Erythrocyte sedimentation rate (ESR)** is a general marker of **inflammation** and can be elevated in many conditions, including ankylosing spondylitis. - However, it is **non-specific** and not diagnostic for AS; some patients with active disease may even have normal ESR levels. *Lumbar movement* - **Reduced lumbar movement** (e.g., restricted lumbar flexion and extension, positive **Schober's test**) is a *clinical feature* commonly found in ankylosing spondylitis due to spinal involvement. - While important for assessing disease progression and functional impairment, it is not as **specific** as radiological evidence of sacroiliitis, as other conditions can also limit lumbar mobility [1].

Q: All of the following regarding ankylosing spondylitis are true except:

Non-erosive arthritis. ***Non-erosive arthritis*** - Ankylosing spondylitis is characterized by **erosive changes**, particularly at the discovertebral and sacroiliac joints, which can lead to **syndesmophyte formation** and eventual *ankylosis* (fusion) of the spine [1]. - The disease involves inflammation and subsequent **ossification of ligaments**, leading to structural damage rather than being purely non-erosive [1]. *HLA B27 is positive in >90%* - A strong association with **HLA-B27** is a hallmark of ankylosing spondylitis, with over 90% of Caucasian patients testing positive, making it a key diagnostic marker [1]. - While not universally present, its high prevalence further supports this statement as being true [1]. *More common in males than females* - Ankylosing spondylitis typically has a male-to-female predominance, with males generally experiencing **more severe disease progression** and spinal involvement. - While the diagnostic criteria have evolved to recognize a more equitable distribution, the classic presentation often highlights male prevalence. *Typically occurs in late teens to early adulthood (peak onset 20s-30s), but can present after 40 in rare cases* - The onset of ankylosing spondylitis symptoms most commonly occurs in **young adults**, typically between the ages of 20 and 40 [1]. - While less common, a small percentage of patients may experience symptom onset later in life, though this is less typical of the disease's natural history.

Q: Which of the following is/are associated with an anaphylactic reaction?

Both a & c. ***Both a & c*** - Anaphylaxis is a **severe, life-threatening allergic reaction** characterized by rapid onset and diverse symptoms, including cutaneous (urticaria, swollen lips), respiratory, cardiovascular (hypotension, tachycardia), and gastrointestinal symptoms [1]. - **Urticaria (hives)** and **angioedema (swollen lips/face)** are common cutaneous manifestations [2], while **persistent abdominal cramps** can indicate gastrointestinal involvement, all typical of anaphylaxis [1]. *Urticaria & Swollen lips* - While **urticaria** and **swollen lips (angioedema)** are classic signs of anaphylaxis, they represent only one system involved in severe reactions [1]. - Anaphylaxis is a multi-system reaction, and these symptoms alone do not encompass the full clinical picture or severity [3]. *Hypertension* - Anaphylaxis primarily causes **hypotension** due to widespread vasodilation and increased vascular permeability, not hypertension. - **Hypotension** is a key diagnostic criterion for severe anaphylaxis, indicating cardiovascular collapse [1]. *Persistent abdominal cramps* - **Persistent abdominal cramps** are a recognized gastrointestinal symptom of anaphylaxis, resulting from smooth muscle contraction and fluid shifts [1]. - However, relying solely on this symptom would miss other vital signs and symptoms that define and confirm an anaphylactic reaction.

Q: A 30-year-old with recurrent ulcers on lips and genitalia, and positive pathergy test. Diagnosis?

Behcet's disease. Behcet's disease - The combination of recurrent oral and genital ulcers along with a positive pathergy test is highly characteristic of Behcet's disease. - Behcet's is a systemic vasculitis that can affect multiple organ systems, with mucocutaneous lesions being a hallmark [3]. Herpes simplex - While herpes simplex causes recurrent oral (cold sores) and genital ulcers, it does not typically involve a positive pathergy test [1]. - Herpes lesions are typically vesicular before ulcerating, and diagnosis is often confirmed by viral culture or PCR [1]. Pemphigus vulgaris - Characterized by painful oral blisters and erosions, but genital ulcers are less common, and it does not typically involve a positive pathergy test. - Pemphigus vulgaris is an autoimmune blistering disease due to antibodies against desmoglein, diagnosed by biopsy and immunofluorescence [2]. Syphilis - Can cause oral and genital ulcers (chancres in primary syphilis) and sometimes mucocutaneous lesions in secondary syphilis (e.g., mucous patches). - However, syphilis does not present with a positive pathergy test, and its diagnosis is made through serological tests (e.g., RPR, VDRL, FTA-ABS).

Q: Which of the following is not typically associated with systemic lupus erythematosus?

Auer rods. ***Auer rods*** - **Auer rods** are abnormal, rod-shaped inclusions found in the cytoplasm of myeloblasts and promyelocytes, characteristic of **acute myeloid leukemia (AML)**. - Their presence is a definitive diagnostic marker for AML and has no association with systemic lupus erythematosus. *Anti-dsDNA antibodies* - **Anti-dsDNA antibodies** are highly specific for systemic lupus erythematosus (SLE) and are commonly used in its diagnosis and monitoring of disease activity [1]. - They are associated with more severe disease, particularly **lupus nephritis**. *Malar rash* - A **malar rash**, also known as a **butterfly rash**, is a classic cutaneous manifestation of SLE, appearing over the cheeks and bridge of the nose [1]. - It is one of the American College of Rheumatology classification criteria for diagnosing SLE. *Thrombocytopenia* - **Thrombocytopenia** (low platelet count) is a common hematological manifestation of SLE due to autoimmune destruction of platelets. - It can contribute to bleeding tendencies and is one of the systemic criteria used in the diagnosis of SLE.

Question 1

Which of the following has a male:female ratio of 1:1?

Accepted Answer

Behçet's disease

Answer

Polymyalgia rheumatica

Answer

Gout

Answer

Rheumatoid arthritis

Question 2

Which feature best differentiates ANCA-associated vasculitis from anti-GBM disease?

Accepted Answer

Relapsing course

Answer

Crescentic glomerulonephritis

Answer

Rapid progression

Answer

Pulmonary hemorrhage

Question 3

A 40-year-old with chronic hepatitis B develops fever, arthralgia, and palpable purpura. Most likely diagnosis?

Accepted Answer

Polyarteritis nodosa

Answer

IgA vasculitis

Answer

Wegener's granulomatosis

Answer

Cryoglobulinemia

Question 4

30-year male with recurrent sinopulmonary infections has low serum IgA. Most likely diagnosis?

Accepted Answer

Selective IgA deficiency

Answer

X-linked agammaglobulinemia

Answer

Hyper IgM syndrome

Answer

Common variable immunodeficiency

Question 5

A 50-year-old with rheumatoid arthritis has been on long-term NSAIDs. Which parameter should be monitored to assess for potential complications of NSAID use?

Accepted Answer

Urine protein levels

Answer

Erythrocyte sedimentation rate (ESR)

Answer

Serum potassium

Answer

Complete blood count (CBC)

Question 6

Most specific for diagnosing Ankylosing spondylitis?

Accepted Answer

B/l sacroilitis

Answer

HLA B27

Answer

ESR

Answer

Lumbar movement

Question 7

All of the following regarding ankylosing spondylitis are true except:

Accepted Answer

Non-erosive arthritis

Answer

HLA B27 is positive in >90%

Answer

More common in males than females

Answer

Typically occurs in late teens to early adulthood (peak onset 20s-30s), but can present after 40 in rare cases

Question 8

Which of the following is/are associated with an anaphylactic reaction?

Accepted Answer

Both a & c

Answer

Urticaria & Swollen lips

Answer

Hypertension

Answer

Persistent abdominal cramps

Question 9

A 30-year-old with recurrent ulcers on lips and genitalia, and positive pathergy test. Diagnosis?

Accepted Answer

Behcet's disease

Answer

Herpes simplex

Answer

Pemphigus vulgaris

Answer

Syphilis

Question 10

Which of the following is not typically associated with systemic lupus erythematosus?

Accepted Answer

Auer rods

Answer

Anti-dsDNA antibodies

Answer

Malar rash

Answer

Thrombocytopenia

Rheumatology and Immunology — MCQs

Rheumatology and Immunology — MCQs

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