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: All are seen in Behçet's syndrome except:

Pyoderma gangrenosum. ***Pyoderma gangrenosum*** - While Behçet's syndrome can involve skin lesions, **pyoderma gangrenosum** is not typically part of its diagnostic criteria or common manifestations. - Pyoderma gangrenosum is a distinct neutrophilic dermatosis characterized by rapidly enlarging, painful ulcers. *Erythema nodosum* - **Erythema nodosum** is a common dermatological manifestation in Behçet's syndrome, characterized by tender subcutaneous nodules, usually on the shins. - These lesions reflect the systemic inflammatory nature of the disease in various organs. *Genital ulcers* - **Genital ulcers** are one of the major diagnostic criteria for Behçet's syndrome, often painful and recurrent. - They tend to be deep and can form scars, appearing on the scrotum, penis, labia, or perianal area. *Oral ulcers* - **Recurrent oral aphthous ulcers** are the most common and often the initial symptom of Behçet's syndrome. - These ulcers are typically painful, varying in size, and can occur on any mucosal surface in the mouth.

Q: Which type of arthritis is characterized by the absence of a periosteal reaction?

Neuropathic arthritis. ***Neuropathic arthritis*** - **Neuropathic arthritis** (**Charcot joint**) is characterized by progressive joint destruction due to loss of proprioception and pain sensation, leading to severe bone and joint damage without a typical **periosteal reaction**. - The absence of normal protective reflexes results in repetitive microtrauma, often leading to bone resorption and fragmentation rather than new bone formation (periosteal reaction). *Psoriatic arthritis* - **Psoriatic arthritis** often presents with distinctive **periosteal reactions**, particularly at entheses and along the shafts of small bones (e.g., "pencil-in-cup" deformities and fluffy periostitis) [1]. - These periosteal changes are a hallmark of the inflammatory process affecting bone and connective tissues in psoriatic arthritis. *Rheumatoid arthritis* - **Rheumatoid arthritis** primarily involves the synovium, leading to erosions rather than prominent periosteal reactions in early stages [1]. - While periostitis can occur in chronic, destructive rheumatoid arthritis, it is not a primary or characteristic finding compared to other inflammatory arthritides. *Reactive arthritis* - **Reactive arthritis** frequently causes **periosteal reactions**, particularly at entheses (where tendons and ligaments attach to bone) and along the shafts of long bones, often described as "fluffy" periostitis [1]. - These new bone formations are a key radiological feature distinguishing it from other types of inflammatory arthritis.

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: 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: Most common cause of death in SLE in children

Anemia and infections. ***Anemia and infections*** - **Infections** are a leading cause of death in pediatric SLE patients, often due to immunosuppression from the disease itself or its treatment. Although pediatric Systemic Lupus Erythematosus (SLE) is not a primary immune deficiency, the susceptibility to encapsulated bacteria and recurrent infections seen in primary B- and T-lymphocyte deficiencies mirrors the infection risks managed in these patients [1]. - **Anemia** can contribute to overall morbidity and mortality, although it is less directly a cause of death than severe infections or organ failure. *Lupus nephritis* - While **lupus nephritis** is a common and severe manifestation of SLE in children and a major cause of morbidity, particularly long-term kidney failure, it is not the most frequent immediate cause of death. - Advancements in treatment for nephritis have improved prognosis, shifting the leading cause of mortality to other factors. *Lupus cerebritis* - **Lupus cerebritis** (neuropsychiatric SLE) can be life-threatening, causing seizures, stroke, or psychosis, but it is less common as the primary cause of death compared to infections. - Its presence usually indicates severe disease requiring intensive treatment, but not the most common direct cause of death. *Libman sacks endocarditis* - **Libman-Sacks endocarditis** involves sterile vegetations on heart valves and is a known complication of SLE, but it rarely causes acute mortality in children. - It is more often associated with chronic complications like valvular dysfunction or a source of emboli rather than being the most common cause of death.

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: 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: Antibodies most commonly seen in drug induced lupus are:

Antihistone Antibodies. ***Antihistone Antibodies*** - **Antihistone antibodies** are the most common laboratory finding, present in 95% of patients with **drug-induced lupus erythematosus (DIL)**. - This type of lupus is often triggered by medications such as **procainamide**, **hydralazine**, and **isoniazid**. *Anti ds DNA Antibodies* - **Anti-double-stranded DNA (dsDNA) antibodies** are highly specific for **systemic lupus erythematosus (SLE)**, particularly severe cases, but are rarely seen in DIL. - High titers of anti-dsDNA often correlate with **lupus nephritis** and disease activity. *Anti Sm Antibodies* - **Anti-Sm antibodies** are highly specific for **SLE**, sometimes associated with neuropsychiatric manifestations, but are rarely identified in drug-induced lupus. - Their presence helps to confirm the diagnosis of SLE but not DIL. *Anti-Ro Antibodies* - **Anti-Ro (SSA) antibodies** are most commonly associated with **Sjögren's syndrome** and **neonatal lupus**, and can be seen in a subset of SLE patients, especially those with photosensitivity. - While they can be present in some forms of SLE, they are not the hallmark autoantibody for drug-induced lupus.

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

All are seen in Behçet's syndrome except:

Accepted Answer

Pyoderma gangrenosum

Answer

Erythema nodosum

Answer

Genital ulcers

Answer

Oral ulcers

Question 3

Which type of arthritis is characterized by the absence of a periosteal reaction?

Accepted Answer

Neuropathic arthritis

Answer

Psoriatic arthritis

Answer

Rheumatoid arthritis

Answer

Reactive arthritis

Question 4

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 5

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 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

Most common cause of death in SLE in children

Accepted Answer

Anemia and infections

Answer

Libman sacks endocarditis

Answer

Lupus cerebritis

Answer

Lupus nephritis

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

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

Accepted Answer

SLE

Answer

RA

Answer

Scleroderma

Answer

PAN

Question 10

Antibodies most commonly seen in drug induced lupus are:

Accepted Answer

Antihistone Antibodies

Answer

Anti ds DNA Antibodies

Answer

Anti Sm Antibodies

Answer

Anti-Ro Antibodies

Rheumatology and Immunology — MCQs

Rheumatology and Immunology — MCQs

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