Rheumatology and Immunology Practice Questions

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 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: 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: 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: 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 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

All are seen in Behçet's syndrome except:

Accepted Answer

Pyoderma gangrenosum

Answer

Erythema nodosum

Answer

Genital ulcers

Answer

Oral ulcers

Question 2

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 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

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 6

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 7

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 8

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

Accepted Answer

SLE

Answer

RA

Answer

Scleroderma

Answer

PAN

Question 9

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 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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