Rheumatology and Immunology Practice Questions

Q: Which of the following is a characteristic feature of primary Sjogren's syndrome?

Enlargement of salivary glands. ***Enlargement of salivary glands*** - **Sjögren's syndrome** is characterized by chronic inflammation of **exocrine glands**, leading to swelling and dysfunction, most notably in the **parotid** and **submandibular glands**. - This glandular enlargement often presents as recurrent or persistent **bilateral swelling** of the major salivary glands. *Can occur in children* - While possible, Sjögren's syndrome is predominantly a disease of **adults**, with incidence peaking in individuals between **40 and 60 years old**. - **Juvenile Sjögren's syndrome** is rare and typically presents with more variable symptoms, making it less of a characteristic feature of the primary disease in the general population. *Increased complement C4 is associated with thymoma* - **Increased complement C4** levels are generally not associated with Sjögren's syndrome; instead, **hypocomplementemia** (low C4) can be seen in some patients, indicating immune complex activity. - **Thymoma** is primarily linked to **myasthenia gravis** and other paraneoplastic syndromes, not a direct or characteristic association with Sjögren's syndrome or C4 levels in this context. *Can be associated with rheumatoid arthritis* - Sjögren's syndrome can be classified as **primary** (occurring alone) or **secondary** (occurring in conjunction with another autoimmune disease). - Its association with **rheumatoid arthritis** defines **secondary Sjögren's syndrome**, meaning it's not a characteristic feature of the *primary* form of the disease itself.

Q: Oculoorogenital ulcers are associated with which of the following conditions?

Behcet's disease. ***Behcet's disease*** - This condition is characterized by **recurrent oral and genital ulcers**, along with **ocular inflammation** (e.g., uveitis), perfectly matching the "Oculoorogenital ulcers" description. - It is a **chronic, relapsing inflammatory disease** of unknown etiology involving vasculitis affecting various organ systems. *Lichen planus* - This is a chronic inflammatory condition affecting the **skin, hair, nails, and mucous membranes**. - While it can cause oral lesions, it typically presents as **purple, polygonal, pruritic papules** on the skin and does not cause genital ulcers or significant ocular involvement like Behcet's. *Systemic lupus erythematosus (SLE)* - SLE is a **systemic autoimmune disease** with diverse manifestations, including skin rashes, arthritis, and internal organ involvement. - Oral ulcers can occur, but **genital ulcers and severe ocular inflammation** as a prominent triad are not typical diagnostic features of SLE. *Psoriasis* - This is a common **chronic inflammatory skin disease** characterized by well-demarcated erythematous plaques with silvery scales. - Psoriasis primarily affects the skin and joints, and **does not typically present with oral, genital, or ocular ulcers** as described.

Q: Jaw tightness is typically seen in:

Giant cell arteritis (GCA). ***Giant cell arteritis (GCA)*** - **Jaw claudication** or tightness, difficulty chewing, and pain in the jaw are classic symptoms of GCA, resulting from **ischemia of the masticatory muscles**. [1] - This condition is a **large vessel vasculitis** that frequently affects the **temporal arteries** and can lead to blindness if untreated. [1] *Polyarteritis nodosa (PAN)* - PAN is a **necrotizing vasculitis** that typically affects **medium-sized arteries**, often sparing the pulmonary circulation. [1] - Common symptoms include **neuropathy**, abdominal pain, and skin lesions (e.g., livedo reticularis), but **jaw tightness is not a typical feature**. *Coarctation of aorta* - This is a **congenital narrowing of the aorta**, leading to symptoms like **upper extremity hypertension**, headache, and claudication in the lower extremities. - **Jaw tightness is not a symptom** associated with coarctation of the aorta. *Granulomatosis with Polyangiitis (GPA)* - GPA is a **small-vessel vasculitis** characterized by **granulomatous inflammation** involving the upper and lower respiratory tracts, and glomerulonephritis. - Symptoms often include **sinusitis**, lung nodules, and kidney disease, but **jaw claudication is not a characteristic presentation**.

Q: Hereditary angioneurotic edema is due to?

Deficiency of C1 inhibitor. ***Deficiency of C1 inhibitor*** - **Hereditary angioedema (HAE)** is caused by a genetic deficiency or dysfunction of the **C1 esterase inhibitor (C1-INH)** protein. - C1-INH normally regulates the **complement system**, kinin system (mediating **bradykinin** release), and coagulation pathways, and its deficiency leads to uncontrolled activation and excessive bradykinin production, causing angioedema. *Deficiency of NADPH oxidase* - A deficiency in **NADPH oxidase** is associated with **Chronic Granulomatous Disease (CGD)**, a primary immunodeficiency characterized by recurrent bacterial and fungal infections due to impaired phagocytic oxidative burst. - It does not cause angioedema, which involves fluid extravasation rather than impaired microbial killing. *Deficiency of MPO* - **Myeloperoxidase (MPO) deficiency** typically leads to mild to moderate impairment in the killing of certain microorganisms by phagocytes but is often asymptomatic or causes only recurrent candidal infections. - It is not linked to the pathogenesis of angioedema, which involves dysregulation of vasoactive peptides. *Deficiency of properdin* - A deficiency in **properdin** affects the **alternative pathway of the complement system**, increasing susceptibility to **Neisseria infections**. - While properdin is part of the complement system, its deficiency does not directly lead to the uncontrolled production of bradykinin, which is the primary mediator in angioedema due to C1-INH deficiency.

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.

Q: Raynaud's phenomenon is associated with which of the following conditions?

Systemic sclerosis (scleroderma). ***Systemic sclerosis (scleroderma)*** - **Raynaud's phenomenon** is a common and often the initial symptom of systemic sclerosis, occurring in over 90% of patients [1]. - It involves recurrent vasospasm of the digits, leading to characteristic **triphasic color changes** (white, blue, red) and can be a predictor of later systemic disease [1]. *Peripheral artery disease* - This condition involves **atherosclerosis** and arterial narrowing, primarily affecting larger arteries in the limbs, leading to exertional pain (claudication). - While it can restrict blood flow, its pathophysiology and symptoms are distinct from the vasospastic episodes seen in Raynaud's phenomenon. *Hyperthyroidism* - **Hyperthyroidism** causes a hypermetabolic state with symptoms like heat intolerance, weight loss, and tachycardia due to excessive thyroid hormone. - It does not directly cause vasospastic episodes or the characteristic color changes associated with Raynaud's phenomenon. *Lupus erythematosus* - While Raynaud's phenomenon can occur in **Lupus erythematosus** (SLE), it is less universally associated and less prominent than in systemic sclerosis. - SLE is a multisystem autoimmune disease characterized by a wide range of symptoms, including skin rashes, arthritis, and organ involvement, differing from the primary vascular focus of Raynaud's in scleroderma.

Q: What is the most likely diagnosis for a young patient presenting with iritis and joint pain?

AS. ***AS (Ankylosing Spondylitis)*** - **Iritis** (anterior uveitis) is a common extra-articular manifestation of **ankylosing spondylitis**, affecting up to 40% of patients [1]. - **Joint pain**, particularly in the axial skeleton (spine and sacroiliac joints), is a hallmark feature in young patients with AS [1]. *Gout* - Characterized by **recurrent attacks of acute inflammatory arthritis** due to **monosodium urate crystal deposition**. - While it causes severe joint pain, **iritis is not a typical manifestation** of gout. *RA (Rheumatoid Arthritis)* - RA is a **chronic autoimmune inflammatory disease** primarily affecting the **synovial joints** symmetrically, mostly in older adults. - While ocular manifestations like **scleritis** or **episcleritis** can occur, **iritis is less common** compared to AS. *Toxoplasma* - **Toxoplasmosis** primarily causes **chorioretinitis** (inflammation of the choroid and retina), not typically isolated iritis. - Although it can cause **arthralgia** (joint pain), it does not cause inflammatory arthritis like the spondyloarthropathies.

Q: HLA marker of Behcet's syndrome is what?

B51. ***B51*** - **HLA-B51** is the most strongly associated genetic marker with **Behçet's syndrome**, particularly in populations from the Middle East and East Asia. - While not universally present in all Behçet's patients, its presence significantly increases susceptibility and is considered a **risk factor**. *B27* - **HLA-B27** is strongly associated with **seronegative spondyloarthropathies** such as ankylosing spondylitis, reactive arthritis, and psoriatic arthritis, [1] not Behçet's syndrome. - It plays a role in the pathogenesis of these conditions by influencing antigen presentation. [1] *DR5* - While various **HLA-DR alleles** are linked to autoimmune diseases (e.g., DR4 with rheumatoid arthritis, DR3 with SLE), **HLA-DR5** does not have a primary association with Behçet's syndrome. - Behçet's syndrome's primary genetic link is to Class I MHC alleles, specifically HLA-B51. *CW6* - **HLA-Cw6** is strongly associated with **psoriasis vulgaris**, [2] a chronic inflammatory skin condition. - It is not a recognized genetic marker for **Behçet's syndrome**.

Q: Which of these is characteristic of gout?

Podagra. ***Podagra*** - This refers to the **inflammation of the big toe**, which is a classic presentation of gout [2]. - Gout is characterized by an **acute attack** of arthritis, commonly affecting peripheral joints, notably the **first metatarsophalangeal joint** [1]. *Cheiroarthropathy* - This condition involves **joint stiffness** and deformity, typically seen in **diabetes mellitus**, not gout. - It does not present with the **acute, inflammatory attacks** characteristic of gout [1]. *Calcinosis cutis* - This is a skin condition involving **calcium deposits**, often seen in **systemic sclerosis** or dermatomyositis. - It is not directly related to the **uric acid metabolism** issues or **joint inflammation** seen in gout. *Anasarca* - This term describes **generalized edema**, which can result from various conditions like heart failure or renal failure. - It is not specific to gout and does not involve the **acute joint inflammation** hallmark of the disease [1]. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Bones, Joints, and Soft Tissue Tumors, p. 1218. [2] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Bones, Joints, and Soft Tissue Tumors, pp. 1218-1220.

Question 1

Which of the following is a characteristic feature of primary Sjogren's syndrome?

Accepted Answer

Enlargement of salivary glands

Answer

Can occur in children

Answer

Increased complement C4 is associated with thymoma

Answer

Can be associated with rheumatoid arthritis

Question 2

Oculoorogenital ulcers are associated with which of the following conditions?

Accepted Answer

Behcet's disease

Answer

Lichen planus

Answer

Systemic lupus erythematosus (SLE)

Answer

Psoriasis

Question 3

Jaw tightness is typically seen in:

Accepted Answer

Giant cell arteritis (GCA)

Answer

Coarctation of aorta

Answer

Polyarteritis nodosa (PAN)

Answer

Granulomatosis with Polyangiitis (GPA)

Question 4

Hereditary angioneurotic edema is due to?

Accepted Answer

Deficiency of C1 inhibitor

Answer

Deficiency of NADPH oxidase

Answer

Deficiency of MPO

Answer

Deficiency of properdin

Question 5

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 6

Which condition is associated with calcification of the intervertebral disc?

Accepted Answer

Alkaptonuria

Answer

Gout

Answer

Rheumatoid

Answer

Psoriasis

Question 7

Raynaud's phenomenon is associated with which of the following conditions?

Accepted Answer

Systemic sclerosis (scleroderma)

Answer

Peripheral artery disease

Answer

Hyperthyroidism

Answer

Lupus erythematosus

Question 8

What is the most likely diagnosis for a young patient presenting with iritis and joint pain?

Accepted Answer

AS

Answer

Gout

Answer

RA

Answer

Toxoplasma

Question 9

HLA marker of Behcet's syndrome is what?

Accepted Answer

B51

Answer

B27

Answer

DR5

Answer

CW6

Question 10

Which of these is characteristic of gout?

Accepted Answer

Podagra

Answer

Anasarca

Answer

Calcinosis cutis

Answer

Cheiroarthropathy

Rheumatology and Immunology — MCQs

Rheumatology and Immunology — MCQs

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