Immunology and Allergies Practice Questions

Q: In which of the following disorders, vaccines are not contraindicated in the person suffering from that disease?

Complement deficiency disorders. ***Complement deficiency disorders*** - While patients with **complement deficiencies** are susceptible to certain infections (especially by encapsulated bacteria), their adaptive immune system is generally intact. - Therefore, most vaccines, including **live attenuated vaccines**, are not contraindicated; in fact, vaccination is crucial for preventing infections in these patients. *Digeorge syndrome* - This syndrome involves **thymic hypoplasia or aplasia**, leading to severe **T-cell immunodeficiency**. - **Live attenuated vaccines** (e.g., MMR, varicella) are contraindicated due to the risk of uncontrolled replication of the vaccine strain in immunocompromised individuals. *Wiskott Aldrich syndrome* - This is an **X-linked immunodeficiency** characterized by immunodeficiency, eczema, and thrombocytopenia, involving defects in both T and B cell function, and **platelet dysfunction**. - Due to profound immune defects, particularly in T-cell function, **live attenuated vaccines** are contraindicated. *Ataxia telangiectasia* - This is an autosomal recessive disorder causing **progressive cerebellar ataxia**, telangiectasias, and severe **combined immunodeficiency (SCID)-like features** affecting both T and B cells, as well as an increased risk of malignancy. - Due to the severe immunodeficiency, **live attenuated vaccines** are contraindicated.

Q: A 10 year old male child presents with purpuric rashes on the lower extremities, hematuria, abdominal pain, and arthritis but has no history of fever. What is the likely diagnosis ?

Henoch-Schonlein purpura. ***Henoch-Schonlein purpura*** - **Henoch-Schonlein purpura (HSP)** is a **vasculitis** characterized by the classic tetrad of palpable **purpura**, **abdominal pain**, **arthralgia**, and **renal involvement** (hematuria). - The child's presentation, including purpuric rashes on lower extremities, hematuria, abdominal pain, and arthritis, fits the criteria for HSP, which typically affects children. - The **absence of fever** helps distinguish HSP from infectious causes like meningococcemia. *Hemolytic uremic syndrome* - **Hemolytic uremic syndrome (HUS)** is characterized by a triad of **hemolytic anemia**, **thrombocytopenia**, and **acute kidney injury**. - While there is renal involvement (hematuria), the prominent purpuric rash and arthritis point away from HUS as the primary diagnosis. *Idiopathic thrombocytopenic purpura* - **Idiopathic thrombocytopenic purpura (ITP)** involves isolated **thrombocytopenia**, leading to increased bleeding and bruising (purpura). - It does not typically cause the significant abdominal pain, arthritis, or gross hematuria observed in this patient. *Meningococcal meningitis* - **Meningococcal meningitis** is a severe bacterial infection characterized by fever, headache, stiff neck, and a rapidly progressing **petechial or purpuric rash**. - The absence of fever and meningeal signs, and the presence of prominent arthritis and hematuria, make meningococcal meningitis less likely.

Q: All are common features of juvenile idiopathic arthritis EXCEPT

Rheumatoid factor positive. ***Rheumatoid factor positive*** - While some subtypes of **juvenile idiopathic arthritis (JIA)** can be **rheumatoid factor (RF) positive**, the majority of cases, especially the systemic and oligoarticular forms, are **RF negative**. Therefore, a positive RF is not a common feature overall. - The presence of **RF-positive JIA** typically indicates a polyarticular course similar to adult rheumatoid arthritis, but it's less prevalent in the broader JIA population. *Fever with rash* - **Systemic JIA** (Still's disease) is characterized by a high intermittent fever and a salmon-pink macular rash, making this a common feature in a significant subset of JIA. - This specific subtype often presents with prominent systemic inflammation rather than solely articular symptoms. *Hepatosplenomegaly* - **Hepatosplenomegaly** is a common systemic manifestation, particularly in **systemic JIA**, indicating multi-organ involvement due to widespread inflammation. - Along with lymphadenopathy and serositis, it reflects the systemic nature of the disease in acutely ill children. *Increased ESR* - An **elevated erythrocyte sedimentation rate (ESR)** is a general marker of inflammation and is frequently observed across various subtypes of JIA. - This is a common finding in active disease due to the underlying inflammatory process affecting the joints and potentially other organs.

Q: Pauciarticular JRA is characterized by all except:

Scleritis. ***Scleritis*** - **Scleritis** is a rare ocular manifestation in juvenile idiopathic arthritis (JIA) and is not a characteristic feature of **pauciarticular JRA**. - Its presence would suggest other systemic inflammatory conditions or more severe forms of JIA, but not typical pauciarticular JRA. *Uveitis* - **Uveitis**, specifically **chronic anterior uveitis**, is a common and often asymptomatic complication in pauciarticular JRA, particularly in ANA-positive girls. - Regular ophthalmologic screening is crucial for early detection and prevention of long-term vision impairment. *Keratopathy* - While not a direct primary manifestation, **keratopathy** (corneal disease) can occur as a secondary complication of chronic **uveitis** in pauciarticular JRA, often due to inflammation and prolonged use of corticosteroids. - Corneal band keratopathy, in particular, is associated with chronic anterior uveitis. *Cataract* - **Cataract formation** is a known complication associated with chronic **uveitis** in pauciarticular JRA, often exacerbated by long-term corticosteroid use. - It results from chronic inflammation affecting the lens of the eye and can lead to significant vision loss if untreated.

Q: Henoch Schonlein purpura commonly involves which age group?

3-10 years. ***Correct: 3-10 years*** - **Henoch-Schönlein purpura (HSP)** is primarily a disease of **childhood**, with most cases occurring in this age range - The peak incidence is between the ages of **4 and 6 years** - Approximately **90% of cases occur before age 10**, making this the most commonly affected age group - HSP is the **most common systemic vasculitis in children** *Incorrect: >25 years* - While HSP can occur in adults, it is **far less common** and represents less than 10% of all cases - Adult-onset HSP tends to have **more severe presentation** with higher risk of **renal complications** and chronic kidney disease - This is not the typical age group for common involvement *Incorrect: 15-20 years* - This **adolescent/young adult** age group can be affected, but the incidence is significantly lower compared to younger children - The vast majority of cases affect **pre-school and early school-aged children** (3-10 years) - By adolescence, the incidence of HSP has declined substantially *Incorrect: 10-15 years* - While this still falls within the **pediatric age range**, it is beyond the peak incidence - The likelihood of encountering HSP decreases with age after 6-8 years - A 10-year-old has lower risk compared to a 5-year-old

Q: Which category of patients with juvenile idiopathic arthritis, who are mostly HLA-B27 positive and present with enthesitis, lower limb arthritis involving the knees and ankles, and inflammatory low back pain, are most likely to be?

Enthesitis related arthritis. **Enthesitis related arthritis** * **Enthesitis-related arthritis (ERA)** in juvenile idiopathic arthritis (JIA) is characterized by **enthesitis** (inflammation at tendon/ligament insertion sites), **lower limb arthritis** (often of knees and ankles), and **inflammatory low back pain** [1]. * This subtype of JIA is strongly associated with **HLA-B27 positivity**, aligning perfectly with the patient's presentation [1]. *Oligoarticular JIA* * **Oligoarticular JIA** involves **four or fewer joints** during the first six months of disease and typically affects large joints, but enthesitis and inflammatory back pain are not primary features [1]. * While it can involve the knee, the combination of enthesitis and low back pain, especially with HLA-B27 positivity, points away from oligoarticular JIA. *Polyarticular JIA (RF negative)* * **Polyarticular JIA (RF negative)** affects **five or more joints** within the first six months and is characterized by a widespread inflammatory arthritis, but **enthesitis and significant spinal involvement** are not its defining features [1]. * This subtype also doesn't have the strong association with **HLA-B27** seen in the described case. *Systemic JIA* * **Systemic JIA** is characterized by prominent **systemic features** like daily fevers, rash, lymphadenopathy, and serositis, which are not mentioned in the patient's presentation [1]. * While systemic JIA can have joint involvement, the specific combination of **enthesitis, lower limb arthritis, inflammatory back pain, and HLA-B27 positivity** is not typical of this subtype.

Q: Which of the following is NOT true about Henoch-Schönlein purpura?

Non-palpable purpura. ***Non-palpable purpura*** - **Henoch-Schönlein purpura (HSP)** is characterized by **palpable purpura**, typically on the lower extremities and buttocks, which is a hallmark clinical feature [1]. - Non-palpable purpura would suggest a **platelet disorder** or other coagulopathy rather than a vasculitic process like HSP. *Glomerulonephritis* - **Renal involvement**, presenting as glomerulonephritis, is a common and serious complication of HSP that can lead to chronic kidney disease [1]. - This typically presents with **hematuria** and **proteinuria**, and in some cases, **renal failure** [2]. *IgA deposition* - **IgA immune complex deposition** in small blood vessels is the defining **pathological feature** of HSP, leading to inflammation and vessel damage [1]. - This can be seen in affected organs, including the skin (vasculitis), kidneys (glomerulonephritis), and gastrointestinal tract [3]. *Abdominal pain* - **Gastrointestinal involvement** is common in HSP, manifesting as colicky abdominal pain, nausea, vomiting, and sometimes **gastrointestinal bleeding** or **intussusception** [1]. - This pain is often severe and can mimic acute abdominal conditions requiring surgical evaluation.

Q: Which of the following is not true about Juvenile rheumatoid arthritis?

Raynaud's phenomenon. ***Raynaud's phenomenon*** - While other conditions, like **systemic lupus erythematosus** or scleroderma, are associated with Raynaud's, it is **not a typical feature** of **juvenile idiopathic arthritis (JIA)**. - Raynaud's phenomenon is characterized by **vasospasm** of the small arteries and arterioles in response to cold or stress. *Uveitis* - **Uveitis** (inflammation of the uvea) is a common and serious extra-articular manifestation of **JIA**, particularly in the **oligoarticular** and **polyarticular subtypes**. - It often presents asymptomatically and can lead to significant vision loss if not detected and treated early. *Rheumatoid nodules* - **Rheumatoid nodules** can occur in a subset of JIA patients, particularly those with the **polyarticular rheumatoid factor-positive** subtype. - These are subcutaneous nodules found in areas of pressure, similar to those seen in adult rheumatoid arthritis. *Fever* - **Fever** is a hallmark symptom of the **systemic subtype** of JIA (Still's disease). - It typically presents as a **quotidian spiking fever**, often accompanied by an evanescent rash.

Q: A 5-year-old boy has recurrent infections, low platelet count, and rash as shown below. What is the likely diagnosis?

Wiskott Aldrich Syndrome. ***Wiskott Aldrich Syndrome*** - **Wiskott-Aldrich Syndrome (WAS)** is an X-linked recessive disorder characterized by the triad of **thrombocytopenia (low platelets)**, **eczema (rash)**, and **recurrent infections**. - The classic presentation in a young boy with these three features is highly indicative of WAS due to a mutation in the **WAS gene**, which affects immune cell and platelet function. *Bruton's Agammaglobulinemia* - This disorder primarily involves a defect in **B cell development**, leading to a severe deficiency of immunoglobulins and recurrent bacterial infections. - It is not typically associated with **thrombocytopenia** or **eczema** as defining features. *Severe Combined Immunodeficiency* - **SCID** is characterized by a severe defect in both T-cell and B-cell immunity, leading to profound immunodeficiency and recurrent, severe opportunistic infections. - While it causes recurrent infections, it does not typically present with the specific combination of **thrombocytopenia** and **eczema** seen in WAS. *Job Syndrome* - **Job Syndrome (Hyper-IgE Syndrome)** is characterized by very high IgE levels, recurrent skin abscesses, eczema, and coarse facial features. - While **eczema** and **recurrent infections** are present, **thrombocytopenia** is not a characteristic feature of Job Syndrome.

Q: A 5 yr old male child presented with H/O recurrent infection. O/E the child has rashes as shown below. Routine blood investigation reveal patient has low platelets. Which of the following diagnosis is possible?

Wiskott Aldrich syndrome. ***Wiskott Aldrich syndrome*** - This syndrome classically presents with a triad of **eczema**, **thrombocytopenia** (low platelets), and **immunodeficiency** (recurrent infections). The rash is consistent with eczema, and the low platelets and recurrent infections match the clinical picture. - It is an **X-linked recessive disorder** primarily affecting boys, explaining the male gender of the child. *Henoch Schoenlein purpura* - This is a **vasculitis** characterized by palpable purpura, arthralgia, abdominal pain, and renal involvement, which would not typically include low platelets or severe recurrent infections as primary features. - The rash of Henoch-Schönlein purpura is often **non-blanching purpura** on the buttocks and lower extremities, distinct from the eczematous rash described. *Job syndrome* - Also known as **hyper-IgE syndrome**, Job syndrome is characterized by recurrent skin abscesses (staph aureus), severe eczema, and elevated IgE levels, but **thrombocytopenia is not a typical feature**. - While recurrent infections and eczema are present, the **low platelet count** makes Wiskott-Aldrich syndrome a more fitting choice. *Measles* - Measles is an **acute viral infection** causing a characteristic maculopapular rash, fever, cough, coryza, and conjunctivitis. - It does not cause chronic recurrent infections or a persistent low platelet count; rather, it's an acute self-limiting illness.

Question 1

In which of the following disorders, vaccines are not contraindicated in the person suffering from that disease?

Accepted Answer

Complement deficiency disorders

Answer

Digeorge syndrome

Answer

Wiskott Aldrich syndrome

Answer

Ataxia telangiectasia

Question 2

A 10 year old male child presents with purpuric rashes on the lower extremities, hematuria, abdominal pain, and arthritis but has no history of fever. What is the likely diagnosis ?

Accepted Answer

Henoch-Schonlein purpura

Answer

Hemolytic uremic syndrome

Answer

Idiopathic thrombocytopenic purpura

Answer

Meningococcal meningitis

Question 3

All are common features of juvenile idiopathic arthritis EXCEPT

Accepted Answer

Rheumatoid factor positive

Answer

Fever with rash

Answer

Hepatosplenomegaly

Answer

Increased ESR

Question 4

Pauciarticular JRA is characterized by all except:

Accepted Answer

Scleritis

Answer

Uveitis

Answer

Keratopathy

Answer

Cataract

Question 5

Henoch Schonlein purpura commonly involves which age group?

Accepted Answer

3-10 years

Answer

>25 years

Answer

15-20 years

Answer

10-15 years

Question 6

Which category of patients with juvenile idiopathic arthritis, who are mostly HLA-B27 positive and present with enthesitis, lower limb arthritis involving the knees and ankles, and inflammatory low back pain, are most likely to be?

Accepted Answer

Enthesitis related arthritis

Answer

Oligoarticular JIA

Answer

Polyarticular JIA (RF negative)

Answer

Systemic JIA

Question 7

Which of the following is NOT true about Henoch-Schönlein purpura?

Accepted Answer

Non-palpable purpura

Answer

Glomerulonephritis

Answer

IgA deposition

Answer

Abdominal pain

Question 8

Which of the following is not true about Juvenile rheumatoid arthritis?

Accepted Answer

Raynaud's phenomenon

Answer

Uveitis

Answer

Rheumatoid nodules

Answer

Fever

Question 9

A 5-year-old boy has recurrent infections, low platelet count, and rash as shown below. What is the likely diagnosis?

Accepted Answer

Wiskott Aldrich Syndrome

Answer

Bruton's Agammaglobulinemia

Answer

Severe Combined Immunodeficiency

Answer

Job Syndrome

Question 10

A 5 yr old male child presented with H/O recurrent infection. O/E the child has rashes as shown below. Routine blood investigation reveal patient has low platelets. Which of the following diagnosis is possible?

Accepted Answer

Wiskott Aldrich syndrome

Answer

Henoch Schoenlein purpura

Answer

Job syndrome

Answer

Measles

Immunology and Allergies — MCQs

Immunology and Allergies — MCQs

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