Rheumatology and Immunology Practice Questions

Q: A 58-year-old man with a diagnosis of chronic myeloid leukemia is treated with intensive chemotherapy. He goes into remission but develops pain in the left wrist. On physical examination, there is swelling and warmth on the palpation of the wrist. Polarized light microscopy of fluid aspirated from the wrist joint shows needle-shaped crystals that display negative birefringence. Which of the following processes most likely played an important role in the pathogenesis of the patient's wrist pain?

Excessive production of uric acid. ***Excessive production of uric acid*** - The patient's history of **chronic myeloid leukemia (CML)** and subsequent intensive chemotherapy can lead to rapid **cell turnover** and lysis, releasing large amounts of purines. The metabolism of these purines results in a significant increase in **uric acid production** [2]. - The presence of **needle-shaped crystals with negative birefringence** in the synovial fluid is pathognomonic for **gout**, which is caused by the precipitation of **monosodium urate crystals** due to hyperuricemia [1]. *Cytokine-induced synovial proliferation* - While cytokines can play a role in inflammatory arthropathies, **cytokine-induced synovial proliferation** is not directly indicated by the presence of **needle-shaped crystals** with negative birefringence. - This process is more typical of **rheumatoid arthritis** or other inflammatory conditions where crystal deposition is not the primary pathology. *Abnormal platelet function with joint hemorrhages* - **Abnormal platelet function** leading to joint hemorrhages (hemarthrosis) causes a different clinical picture, usually presenting with a history of bleeding disorders or trauma. - The microscopic finding of **needle-shaped crystals with negative birefringence** is not consistent with hemarthrosis. *Chemotherapeutic toxicity to the articular cartilage* - Although chemotherapy can have various side effects, **direct toxicity to articular cartilage** causing acute, painful arthritis with crystal deposition is not a classic presentation. - Chemotherapy-induced arthralgia is usually diffuse and does not typically involve the formation of diagnostic crystals in the joint fluid.

Q: True regarding felty's syndrome is all, except -

Nephropathy. ***Nephropathy*** - **Nephropathy** is not a characteristic component of Felty's syndrome; the triad specifically involves rheumatoid arthritis, neutropenia, and splenomegaly [1]. - While patients with rheumatoid arthritis can develop renal complications, they are not directly linked to the definition of Felty's syndrome itself [1]. *Rheumatoid arthritis* - **Rheumatoid arthritis** is a prerequisite for diagnosing Felty's syndrome, as it represents the underlying chronic inflammatory disease [1]. - Patients typically have severe, long-standing, and often seropositive rheumatoid arthritis [1]. *Neutropenia* - **Neutropenia** (low neutrophil count) is a hallmark of Felty's syndrome, contributing to an increased risk of infections [1]. - The neutropenia is often accompanied by active rheumatoid arthritis and splenomegaly [1]. *Splenomegaly* - **Splenomegaly** (enlarged spleen) is the third essential component of the classic triad in Felty's syndrome [1]. - The enlarged spleen is thought to contribute to the neutropenia by sequestering or destroying neutrophils [1].

Q: A 30-year-old male presented with fever with chills and migratory polyarthralgias. On examination some skin lesions were noted. Aspiration was done from the painful left knee and subjected to gram-staining. The patient reported of similar infections in the past which led to frequent hospitalizations. Which of the following should be screened for in the above patient:-

Total hemolytic complement deficiency. **Total hemolytic complement deficiency** - **Recurrent bacterial infections**, particularly with *Neisseria* species, are characteristic of deficiencies in the **terminal complement pathway** (C5-C9) or **total hemolytic complement (CH50)** [1]. - The patient's presentation with **recurrent infections** and **migratory polyarthralgia** suggests a systemic inflammatory process potentially related to immune dysregulation. *Chediak-Higashi syndrome* - This is an **autosomal recessive** disorder characterized by **partial oculocutaneous albinism**, recurrent pyogenic infections, and neurological abnormalities. - While recurrent infections are present [2], the history does not mention albinism or neurological features, and the arthralgia is less typical for this syndrome. *Neutropenia* - **Neutropenia** can cause recurrent bacterial infections, but it typically presents with more severe and localized infections (e.g., abscesses, cellulitis) rather than migratory polyarthralgia. - The type of infection and the systemic presentation make a primary diagnosis of neutropenia less likely without further evidence. *Common variable immunodeficiency* - **Common variable immunodeficiency (CVID)** is characterized by **hypogammaglobulinemia** leading to recurrent sinopulmonary infections. - While recurrent infections are a feature, CVID is less commonly associated with migratory polyarthralgia and the specific skin lesions mentioned in this context.

Q: All are true about Sjogren syndrome except

Subcutaneous fibrosis. ***Subcutaneous fibrosis*** - **Subcutaneous fibrosis** is typically associated with conditions like **systemic sclerosis** or **eosinophilic fasciitis**, not Sjögren's syndrome [2]. - Sjögren's syndrome primarily affects exocrine glands and other organ systems, but **fibrosis of subcutaneous tissue** is not a characteristic manifestation. *Xerostomia* - **Xerostomia**, or **dry mouth**, is a hallmark symptom of Sjögren's syndrome due to immune-mediated destruction of salivary glands. - This symptom can lead to dental caries, dysphagia, and difficulty speaking. *Lack of tear* - **Keratoconjunctivitis sicca**, or **dry eyes** (lack of tears), is another cardinal feature of Sjögren's syndrome, resulting from inflammation of the lacrimal glands [1]. - Patients often experience grittiness, foreign body sensation, and blurred vision due to insufficient tear production. *Interstitial nephritis* - **Interstitial nephritis** can occur in Sjögren's syndrome, particularly **tubulointerstitial nephritis**, which may lead to **renal tubular acidosis**. - Renal involvement, though less common than sicca symptoms, is a recognized extraglandular manifestation.

Q: All are true about Raynaud’s phenomena except:

Atherosclerosis of vessels. ***Atherosclerosis of vessels*** - **Raynaud's phenomenon** is characterized by episodic **vasospasm**, not primarily by widespread **atherosclerosis** of the vessels. - While systemic sclerosis, a condition associated with severe Raynaud's, can lead to vascular structural changes [1], **atherosclerosis** is not the hallmark pathological process of Raynaud's itself. *More common in females* - **Primary Raynaud's phenomenon** is indeed more prevalent in **females**, especially young women, with a female-to-male ratio of approximately 9:1. - Hormonal influences and genetic predisposition are thought to contribute to this higher incidence in women. *Spasm of vessels* - The central mechanism of **Raynaud's phenomenon** is the **episodic vasospasm** of small arteries and arterioles, typically in the fingers and toes [1]. - This vasospasm leads to transient reduction in blood flow, causing characteristic color changes. *Exposure to cold aggravates* - **Exposure to cold temperatures** is the most common trigger for attacks of Raynaud's phenomenon. - Cold causes exaggerated vasoconstriction, leading to the characteristic blanching, cyanosis, and subsequent rubor of the affected digits.

Q: Vigorous treatment of nephritis in SLE is indicated in:

Class III & IV. ***Class III & IV*** - **Class III (focal) and Class IV (diffuse) lupus nephritis** involve significant **immune complex deposition** and **inflammation** leading to proliferative changes in the glomeruli [1]. - These classes carry a high risk of **progression to end-stage renal disease (ESRD)** and require aggressive immunosuppressive therapy to preserve renal function [2]. *Class IV & V* - While **Class IV lupus nephritis** certainly requires vigorous treatment, **Class V (membranous) lupus nephritis** typically has a slower progression and is often managed with less aggressive immunosuppression initially, unless it progresses to proliferative forms or is associated with nephrotic syndrome and declining renal function [1]. - The combination of only IV and V does not fully capture the most common severe proliferative forms. *Class V & VI* - **Class V (membranous) lupus nephritis** generally has a more indolent course than proliferative forms and may not always require very aggressive treatment unless there is significant proteinuria or declining renal function [1]. - **Class VI (sclerotic) lupus nephritis** represents irreversible scarring and fibrosis, where vigorous *immunosuppressive* treatment is largely ineffective as the damage is already established. *Class I & II* - **Class I (minimal mesangial) and Class II (mesangial proliferative) lupus nephritis** generally have a good prognosis and are often managed with conservative treatment. - These mild forms typically do not require the intensive immunosuppression indicated for more severe proliferative lesions.

Q: All of the following conditions are observed in Gout except

Deficiency of enzyme Xanthine oxidase. ***Deficiency of enzyme Xanthine oxidase*** - A deficiency in **xanthine oxidase** would lead to a *decrease* in uric acid production, thus *preventing* or *ameliorating* gout [3]. - Gout is caused by an *excess* of uric acid, often due to *overproduction* or *underexcretion*, not a lack of the enzyme that produces it [1][3]. *Uric acid nephrolithiasis* - Patients with gout are at an increased risk of developing **uric acid kidney stones** due to high circulating uric acid levels [2]. - The precipitation of **uric acid crystals** in the urinary tract can lead to stone formation and renal colic. *Increase in serum urate concentration* - Gout is fundamentally caused by **hyperuricemia**, defined as an abnormally high concentration of uric acid in the blood [1][3]. - This elevated urate concentration leads to the **deposition of monosodium urate crystals** in joints and other tissues [4][5]. *Renal disease involving interstitial tissues* - Chronic hyperuricemia in gout can lead to **urate crystal deposition** in the kidney's interstitial tissues, causing **interstitial nephritis** [1]. - This can result in **chronic kidney disease** and impaired renal function over time, representing a common complication of uncontrolled gout [2].

Q: Increased frequency of HLA-B 27 is seen in all the following diseases except

Myasthenia gravis. ***Myasthenia gravis*** - **Myasthenia gravis** is an autoimmune disorder characterized by muscle weakness and fatigue due to antibodies against acetylcholine receptors at the neuromuscular junction; it is not associated with **HLA-B27**. - Its pathogenesis involves **antibodies** and T-cell mediated responses against muscle proteins, distinct from the **spondyloarthropathies**. *Ankylosing spondylitis* - **Ankylosing spondylitis** is a chronic inflammatory disease primarily affecting the spine and sacroiliac joints, showing a very strong association with **HLA-B27** (over 90% of cases) [1]. - The presence of **HLA-B27** is a key diagnostic and prognostic factor in this condition [1]. *Reiters syndrome* - **Reiter's syndrome**, now more commonly known as **Reactive arthritis**, is a seronegative spondyloarthropathy often triggered by an infection and strongly associated with **HLA-B27** [1]. - It classically presents with a triad of **arthritis**, **urethritis**, and **conjunctivitis**, linked to the **HLA-B27** allele [1]. *Acute anterior uveitis* - **Acute anterior uveitis** is an inflammation of the iris and ciliary body, and it commonly occurs in association with **HLA-B27** positive spondyloarthropathies, including ankylosing spondylitis and reactive arthritis [1]. - Approximately 50% of patients with **acute anterior uveitis** are **HLA-B27** positive, even in the absence of other systemic diseases [1].

Q: Behcet's disease is characterized by all of the following Except

Aortic aneurysm is the most common vascular disorder. ***Aortic aneurysm is the most common vascular disorder*** - While **aortic involvement** can occur in Behcet's disease, it is **not the most common vascular disorder**; thrombotic events (both venous and arterial) are far more prevalent. - **Venous thrombosis** is the **most common vascular manifestation** of Behcet's disease, affecting both superficial and deep veins. *Immunosuppressive therapy is used in Mucocutaneous and eye lesions* - **Immunosuppressive therapy**, including corticosteroids and other agents, is foundational for managing Behcet's disease, particularly for **severe mucocutaneous and ocular involvement** to prevent tissue damage and preserve vision. - This statement accurately reflects a key aspect of Behcet's disease management. *Iritis associated with oral and genital mucocutaneous ulcerations* - Behcet's disease is characterized by a **triad of oral ulcerations, genital ulcerations, and inflammatory eye disease (including iritis)**. - This combination of symptoms is a hallmark of the condition and is listed in diagnostic criteria. *Venous thrombosis is managed with lifelong oral anticoagulation* - **Venous thrombosis is common** in Behcet's, but **lifelong oral anticoagulation** as a blanket treatment is **controversial and not standard practice**. - Management often focuses on immunosuppression to reduce inflammation rather than just anticoagulation, as thrombi in Behcet's are highly inflammatory and respond better to **immunosuppressive therapy**.

Q: A 35-year-old man presents to the physician with arthritic pain in both knees along with back pain. He states that the pain has been present for months. The patient is afebrile, and his slightly swollen knee joints are neither hot nor tender to palpation; however the pain does restrict his motion. The cartilage of his ears appears slightly darker than normal. No tophi are present. A urine specimen is taken for analysis of uric acid content and turns black in the laboratory while standing. What is the most likely diagnosis at this point?

Alkaptonuria. ### Alkaptonuria - The key diagnostic features are **arthritic pain in the knees and back**, **darkening of ear cartilage (ochronosis)** [1], and **urine turning black on standing** due to the oxidation of homogentisic acid. - This is an **autosomal recessive disorder** caused by a deficiency of **homogentisate 1,2-dioxygenase**, leading to the accumulation of homogentisic acid. ### Ankylosing spondylitis - Characterized by **chronic inflammatory back pain and stiffness** that improves with exercise, often associated with sacroiliitis. - While back pain is present, the absence of findings like **HLA-B27 positivity** (not mentioned) and the specific urine and cartilage findings make it less likely. ### Maple syrup urine disease - This is a metabolic disorder affecting the metabolism of **branched-chain amino acids** (leucine, isoleucine, valine), leading to a characteristic **sweet-smelling urine**. - It typically presents in infancy with severe neurological symptoms and feeding difficulties, not with arthritic pain and dark urine. ### Phenylketonuria - Caused by a deficiency of **phenylalanine hydroxylase**, leading to the accumulation of phenylalanine. - Presents with severe intellectual disability, seizures, and a musty odor in urine if untreated, without the specific arthritic or ochronotic findings seen here.

Question 1

A 58-year-old man with a diagnosis of chronic myeloid leukemia is treated with intensive chemotherapy. He goes into remission but develops pain in the left wrist. On physical examination, there is swelling and warmth on the palpation of the wrist. Polarized light microscopy of fluid aspirated from the wrist joint shows needle-shaped crystals that display negative birefringence. Which of the following processes most likely played an important role in the pathogenesis of the patient's wrist pain?

Accepted Answer

Excessive production of uric acid

Answer

Cytokine-induced synovial proliferation

Answer

Abnormal platelet function with joint hemorrhages

Answer

Chemotherapeutic toxicity to the articular cartilage

Question 2

True regarding felty's syndrome is all, except -

Accepted Answer

Nephropathy

Answer

Rheumatoid arthritis

Answer

Neutropenia

Answer

Splenomegaly

Question 3

A 30-year-old male presented with fever with chills and migratory polyarthralgias. On examination some skin lesions were noted. Aspiration was done from the painful left knee and subjected to gram-staining. The patient reported of similar infections in the past which led to frequent hospitalizations. Which of the following should be screened for in the above patient:-

Accepted Answer

Total hemolytic complement deficiency

Answer

Chediak-Higashi syndrome

Answer

Neutropenia

Answer

Common variable immunodeficiency

Question 4

All are true about Sjogren syndrome except

Accepted Answer

Subcutaneous fibrosis

Answer

Xerostomia

Answer

Lack of tear

Answer

Interstitial nephritis

Question 5

All are true about Raynaud’s phenomena except:

Accepted Answer

Atherosclerosis of vessels

Answer

More common in females

Answer

Spasm of vessels

Answer

Exposure to cold aggravates

Question 6

Vigorous treatment of nephritis in SLE is indicated in:

Accepted Answer

Class III & IV

Answer

Class IV & V

Answer

Class V & VI

Answer

Class I & II

Question 7

All of the following conditions are observed in Gout except

Accepted Answer

Deficiency of enzyme Xanthine oxidase

Answer

Uric acid nephrolithiasis

Answer

Increase in serum urate concentration

Answer

Renal disease involving interstitial tissues

Question 8

Increased frequency of HLA-B 27 is seen in all the following diseases except

Accepted Answer

Myasthenia gravis

Answer

Ankylosing spondylitis

Answer

Reiters syndrome

Answer

Acute anterior uveitis

Question 9

Behcet's disease is characterized by all of the following Except

Accepted Answer

Aortic aneurysm is the most common vascular disorder

Answer

Immunosuppressive therapy is used in Mucocutaneous and eye lesions

Answer

Iritis associated with oral and genital mucocutaneous ulcerations

Answer

Venous thrombosis is managed with lifelong oral anticoagulation

Question 10

A 35-year-old man presents to the physician with arthritic pain in both knees along with back pain. He states that the pain has been present for months. The patient is afebrile, and his slightly swollen knee joints are neither hot nor tender to palpation; however the pain does restrict his motion. The cartilage of his ears appears slightly darker than normal. No tophi are present. A urine specimen is taken for analysis of uric acid content and turns black in the laboratory while standing. What is the most likely diagnosis at this point?

Accepted Answer

Alkaptonuria

Answer

Ankylosing spondylitis

Answer

Maple syrup urine disease

Answer

Phenylketonuria

Rheumatology and Immunology — MCQs

Rheumatology and Immunology — MCQs

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