Rheumatology and Immunology Practice Questions

Q: Which of the following is a characteristic feature of Granulomatosis with polyangiitis?

Perforated nasal septum. ***Perforated nasal septum*** - **Granulomatosis with polyangiitis (GPA)**, previously known as **Wegener's granulomatosis**, is characterized by **granulomatous inflammation** and **vasculitis** of small to medium-sized vessels, particularly affecting the upper and lower respiratory tracts, and the kidneys [1]. - Damage to the nasal cartilage and bone due to inflammation and vasculitis can lead to a **perforation of the nasal septum**, which is a classic feature. *Nasal polyp* - While nasal polyps can occur in inflammatory conditions, they are **not a specific or highly characteristic feature** of Granulomatosis with polyangiitis compared to septal perforation. - **Allergic rhinitis** or **chronic rhinosinusitis** are more commonly associated with nasal polyps. *Persistent sinusitis* - **Persistent sinusitis** (rhinosinusitis) is a very common initial manifestation of Granulomatosis with polyangiitis due to upper airway inflammation. - However, it's a **less specific finding** than a perforated septum, as sinusitis can be caused by many other conditions. *Collapse of nasal bridge* - **Collapse of the nasal bridge**, also known as a **saddle nose deformity**, can occur in advanced or destructive cases of Granulomatosis with polyangiitis due to extensive cartilage and bone destruction. - While possible, a **perforated nasal septum often precedes** or is a more direct, earlier indicator of the destructive process in the nose.

Q: Most common hematological malignancy associated with Rheumatoid Arthritis (RA)?

Large granular lymphocytic leukemia (LGLL). ***Large granular lymphocytic leukemia (LGLL)*** - **LGLL** is the most common hematological malignancy strongly associated with **rheumatoid arthritis (RA)**, often presenting with features such as **neutropenia** and splenomegaly. - Approximately 80% of patients with LGLL have a **T-cell phenotype**, and a significant subset experiences **autoimmune diseases**, with RA being the most frequent. *Diffuse large B cell lymphoma* - While patients with **RA** have an increased risk of **lymphoma**, **diffuse large B-cell lymphoma (DLBCL)** is a more aggressive type but not the most common hematologic malignancy directly associated with the disease itself in terms of prevalence [3]. - Inflammatory conditions like **RA** can contribute to chronic immune stimulation, increasing the risk of certain lymphomas, but LGLL holds a more direct and prevalent association [1]. *Chronic lymphocytic leukemia* - **Chronic lymphocytic leukemia (CLL)** is a lymphoproliferative disorder of **B lymphocytes**, but it does not have a particularly strong or common association with **RA** compared to LGLL [2]. - The elevated risk of hematological malignancies in RA patients typically points more towards lymphoproliferative disorders driven by specific immune dysregulations characteristic of RA. *T-cell prolymphocytic leukemia* - **T-cell prolymphocytic leukemia (T-PLL)** is a rare and aggressive **T-cell leukemia** that generally presents with a high white blood cell count and splenomegaly, but it is not commonly linked with **RA**. - Its clinical presentation and biology are distinct from the more indolent leukemias like LGLL that are often seen in conjunction with autoimmune conditions.

Q: Which of the following is NOT a characteristic feature of systemic sclerosis?

Gottron's papules. ***Gottron's papules*** - **Gottron's papules** are pathognomonic for **dermatomyositis**, not systemic sclerosis. They are red, scaling papules found over the extensor surfaces of the metacarpophalangeal (MCP) and interphalangeal (IP) joints. - While both systemic sclerosis and dermatomyositis are connective tissue diseases, their distinct cutaneous manifestations aid in differentiation. *Acroosteolysis* - **Acroosteolysis** refers to the resorption of the distal phalanges, a common feature in systemic sclerosis, particularly in severe cases. - This symptom contributes to the characteristic digital abnormalities seen in the disease. *Calcinosis cutis* - **Calcinosis cutis** is the deposition of calcium in the skin and subcutaneous tissues, often seen in subsets of systemic sclerosis, especially the CREST syndrome. - It can manifest as firm, white-yellow nodules or plaques and contribute to skin breakdown. *Digital ulcers* - **Digital ulcers** are a frequent and debilitating complication of systemic sclerosis, resulting from severe **vasculopathy** [1] and **ischemia** [1]. - They are often painful and can lead to significant tissue loss and infection.

Q: Which of the following is NOT a feature of scleroderma?

Syndactyly. ***Syndactyly*** - **Syndactyly** (fusion of digits) is a congenital anomaly and is **not** a typical feature of scleroderma. - Scleroderma primarily involves **fibrosis** and vascular changes, leading to skin thickening, not digit fusion [1]. *Decrease in tone of LES* - A **decrease in tone of the lower esophageal sphincter (LES)** is a common gastrointestinal manifestation of scleroderma. - This leads to **gastroesophageal reflux disease (GERD)** and related symptoms due to smooth muscle atrophy and fibrosis. *Restrictive cardiomyopathy* - **Restrictive cardiomyopathy** can occur in scleroderma due to **myocardial fibrosis**, leading to impaired diastolic filling. - This is a serious cardiac complication that can cause **heart failure**. *Halitosis* - **Halitosis** (bad breath) can be an indirect manifestation of scleroderma, often associated with severe **GERD**. - Impaired esophageal motility and reflux are common in scleroderma and can contribute to dental problems and **oral dysbiosis**, which can cause halitosis.

Q: In inflammatory myopathy, which group of muscles is not affected

Ocular. ***Ocular*** - The **extraocular muscles** responsible for eye movement are generally spared in typical inflammatory myopathies, distinguishing them from other neuromuscular disorders. - Inflammatory myopathies primarily affect **skeletal muscles**, but **ocular muscles** have unique immunological and physiological properties that often protect them. *Facial* - While less commonly affected early in the disease, some inflammatory myopathies, particularly **dermatomyositis**, can eventually involve **facial muscles**, leading to weakness. - Involvement of **facial muscles** can manifest as difficulty with smiling, whistling, or closing the eyelids. *Proximal muscles of limb* - Inflammatory myopathies characteristically cause **proximal muscle weakness**, affecting muscles of the **shoulders, hips, and thighs** [1]. - This weakness often presents as difficulty climbing stairs, getting up from a chair, or lifting objects overhead [1]. *Distal muscles of limb* - While less common than proximal involvement, **distal muscle weakness** (affecting hands and feet) can occur in some subsets of inflammatory myopathies, such as **inclusion body myositis**. - This can lead to difficulties with fine motor tasks or foot drop.

Q: Which antibodies in a mother with SLE are responsible for congenital heart disease in the child?

Anti-Ro & Anti-La. ***Anti-Ro & Anti-La*** - These antibodies are actively transported across the **placenta** and can target fetal cardiac tissue, leading to **congenital heart block** [2]. - They are classically associated with **neonatal lupus erythematosus**, which includes congenital heart block and transient skin lesions [1], [2]. *Anti-histone* - **Anti-histone antibodies** are primarily associated with **drug-induced lupus erythematosus**, rather than congenital heart defects. - While present in some SLE patients, they do not have a direct causal link to fetal cardiac abnormalities. *Anti-ds DNA* - **Anti-dsDNA antibodies** are highly specific for **systemic lupus erythematosus (SLE)** and are associated with disease activity, especially **lupus nephritis** [1]. - They are not directly implicated in causing congenital heart disease in the offspring. *Anti-centromere* - **Anti-centromere antibodies** are characteristic of **CREST syndrome**, a limited form of systemic sclerosis. - They are not found in SLE and thus have no role in SLE-related congenital heart disease.

Q: What is the primary condition associated with positive anti-dsDNA antibodies?

SLE. ***SLE*** - **Anti-dsDNA antibodies** are a highly specific marker for **Systemic Lupus Erythematosus (SLE)** and are included in its diagnostic criteria [1]. - The levels of **anti-dsDNA antibodies** can also correlate with disease activity, particularly in cases of **lupus nephritis** [1]. *RA* - **Rheumatoid Arthritis (RA)** is primarily associated with **rheumatoid factor (RF)** and **anti-citrullinated protein antibodies (ACPA)** or **anti-CCP antibodies**. - While ANA (antinuclear antibodies) can be positive in RA, **anti-dsDNA antibodies** are not a characteristic serological marker [1]. *Scleroderma* - **Scleroderma**, or systemic sclerosis, is characterized by specific antibodies such as **anti-Scl-70 (topoisomerase I)**, **anti-centromere antibodies**, and **anti-RNA polymerase III antibodies**, depending on the subtype. - **Anti-dsDNA antibodies** are not typically found in scleroderma and do not play a role in its diagnosis [1]. *PAN* - **Polyarteritis Nodosa (PAN)** is a **necrotizing vasculitis** of medium-sized arteries and is not associated with **anti-dsDNA antibodies**. - PAN is generally considered an **ANCA-negative vasculitis**, and its diagnosis relies more on clinical features, angiography, and biopsy findings.

Q: In a patient presenting with symptoms of drug-induced lupus erythematosus, which antibody is most commonly found?

Anti-histone antibody. ***Anti-histone antibody*** - **Anti-histone antibodies** are present in approximately 90-95% of patients with **drug-induced lupus erythematosus**. - Their presence, especially with a history of exposure to an inducing drug such as **hydralazine** or **procainamide**, strongly suggests this diagnosis. *Anti-Ro* - **Anti-Ro antibodies** are more commonly associated with **Sjogren's syndrome** and **neonatal lupus**, rather than drug-induced lupus [1]. - While they can be present in systemic lupus erythematosus, they are not the hallmark antibody for the drug-induced form. *Ds-DNA* - **Anti-double-stranded DNA (anti-dsDNA) antibodies** are highly specific for **systemic lupus erythematosus (SLE)** and often correlate with disease activity, particularly **lupus nephritis**. - They are typically **negative** or present in very low titers in drug-induced lupus erythematosus. *Anti-Sm* - **Anti-Smith (anti-Sm) antibodies** are highly specific for **systemic lupus erythematosus (SLE)** and are considered a diagnostic marker for the disease [1]. - They are rarely found in patients with drug-induced lupus erythematosus.

Q: Which of the following is not a feature of Systemic Lupus Erythematosus (SLE)?

Sterility. ***Sterility*** - While SLE can affect fertility due to **gonadal dysfunction** or **treatment-related factors**, it is not a *direct* or *defining feature* of the disease itself. - Sterility is a less common manifestation compared to the widespread organ system involvement that characterizes SLE [2]. *Recurrent abortion* - **Recurrent abortions** are a well-recognized complication in SLE, particularly when associated with **antiphospholipid syndrome**, which frequently co-occurs with SLE [1]. - **Antiphospholipid antibodies** can lead to thrombosis in placental vessels, causing fetal loss. *Psychosis* - **Psychosis** is a significant neuropsychiatric manifestation of SLE, classified under **neuropsychiatric lupus (NPSLE)**. - It can result from **inflammation**, **autoantibody effects**, or **ischemia** within the central nervous system. *Coomb's positive hemolytic anemia* - **Coomb's positive hemolytic anemia** is a common hematological complication in SLE, indicating the presence of **autoantibodies** against red blood cells [3]. - This **autoimmune destruction** of red blood cells leads to anemia, and a positive direct Coombs test confirms antibody sensitization [3].

Q: Which condition is associated with calcification of the intervertebral disc?

Alkaptonuria. ***Alkaptonuria*** - **Alkaptonuria** is a rare autosomal recessive disorder characterized by a deficiency of the enzyme **homogentisate 1,2-dioxygenase**, leading to the accumulation of **homogentisic acid**. - This accumulation results in the deposition of a **dark pigment (ochre pigment)** in connective tissues, including the intervertebral discs, causing **calcification (ochronosis)** and early degenerative arthritis. *Gout* - **Gout** is caused by the deposition of **monosodium urate crystals** in joints and soft tissues, leading to inflammation and pain [1]. - While it can affect various joints, it does not typically cause **calcification of intervertebral discs**; rather, it can form tophi in periarticular soft tissues [1]. *Rheumatoid* - **Rheumatoid arthritis** is a chronic autoimmune inflammatory disease primarily affecting the **synovial lining of joints**, leading to joint destruction and deformity. - It does not involve the **calcification of intervertebral discs**; spinal involvement is usually restricted to the cervical spine, particularly the atlantoaxial joint. *Psoriasis* - **Psoriasis** is a chronic inflammatory skin condition, which in some cases can be associated with **psoriatic arthritis**, an inflammatory arthritis. - Psoriatic arthritis primarily affects peripheral joints, the sacroiliac joints, and the spine, but it does not cause **calcification of the intervertebral discs** as seen in alkaptonuria.

Question 1

Which of the following is a characteristic feature of Granulomatosis with polyangiitis?

Accepted Answer

Perforated nasal septum

Answer

Nasal polyp

Answer

Persistent sinusitis

Answer

Collapse of nasal bridge

Question 2

Most common hematological malignancy associated with Rheumatoid Arthritis (RA)?

Accepted Answer

Large granular lymphocytic leukemia (LGLL)

Answer

Diffuse large B cell lymphoma

Answer

Chronic lymphocytic leukemia

Answer

T-cell prolymphocytic leukemia

Question 3

Which of the following is NOT a characteristic feature of systemic sclerosis?

Accepted Answer

Gottron's papules

Answer

Calcinosis cutis

Answer

Digital ulcers

Answer

Acroosteolysis

Question 4

Which of the following is NOT a feature of scleroderma?

Accepted Answer

Syndactyly

Answer

Restrictive cardiomyopathy

Answer

Halitosis

Answer

Decrease in tone of LES

Question 5

In inflammatory myopathy, which group of muscles is not affected

Accepted Answer

Ocular

Answer

Facial

Answer

Proximal muscles of limb

Answer

Distal muscles of limb

Question 6

Which antibodies in a mother with SLE are responsible for congenital heart disease in the child?

Accepted Answer

Anti-Ro & Anti-La

Answer

Anti-ds DNA

Answer

Anti-centromere

Answer

Anti-histone

Question 7

What is the primary condition associated with positive anti-dsDNA antibodies?

Accepted Answer

SLE

Answer

RA

Answer

Scleroderma

Answer

PAN

Question 8

In a patient presenting with symptoms of drug-induced lupus erythematosus, which antibody is most commonly found?

Accepted Answer

Anti-histone antibody

Answer

Ds-DNA

Answer

Anti-Sm

Answer

Anti-Ro

Question 9

Which of the following is not a feature of Systemic Lupus Erythematosus (SLE)?

Accepted Answer

Sterility

Answer

Recurrent abortion

Answer

Psychosis

Answer

Coomb's positive hemolytic anemia

Question 10

Which condition is associated with calcification of the intervertebral disc?

Accepted Answer

Alkaptonuria

Answer

Gout

Answer

Rheumatoid

Answer

Psoriasis

Rheumatology and Immunology — MCQs

Rheumatology and Immunology — MCQs

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