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ANSWERS WITH EXPLANATION

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Question 1: A 48-year-old woman with a 10-year history of rheumatoid arthritis controlled with methotrexate presents with a 2-week history of increasing fatigue, pallor, and glossitis. Her MCV is elevated. What is the most likely cause of her symptoms?

Answer: The most likely cause of this patient’s symptoms is folate deficiency.

Explanation:
Methotrexate, which the patient has been on for rheumatoid arthritis, inhibits dihydrofolate reductase, leading to decreased folate levels. Folate is crucial for DNA synthesis, particularly in rapidly dividing cells like those in the bone marrow.
Symptoms such as increasing fatigue, pallor, and glossitis (inflammation of the tongue) are consistent with megaloblastic anemia, which can result from folate deficiency.
Her elevated MCV (mean corpuscular volume) indicates macrocytic anemia, which is often caused by deficiencies in folate or vitamin B12. Given the history of methotrexate use, folate deficiency is more likely.

Question 2: A 65-year-old man presents with a 3-month history of pain and stiffness in his shoulders and hips, especially in the morning. He also reports low-grade fever and fatigue. His ESR and CRP are elevated. What is the most likely diagnosis?

The most likely diagnosis for this patient is polymyalgia rheumatica (PMR).

Explanation:
Polymyalgia rheumatica is a common inflammatory condition in older adults, typically over 50 years of age, characterized by pain and stiffness in the shoulders, hips, and neck, especially in the morning.
Low-grade fever and fatigue are common systemic symptoms of PMR.
Elevated ESR (erythrocyte sedimentation rate) and CRP (C-reactive protein) indicate an inflammatory process, both of which are typically elevated in PMR.

 

Question 3 : A 40-year-old woman with systemic sclerosis presents with severe hypertension and headache. Laboratory tests reveal proteinuria and elevated creatinine levels. What is the most likely cause of her symptoms?

The most likely cause of this patient’s symptoms is scleroderma renal crisis (SRC).

Explanation:
Systemic sclerosis (scleroderma) can cause a serious complication known as scleroderma renal crisis, which is characterized by severe hypertension, proteinuria, and acute kidney injury (elevated creatinine).
Severe hypertension and headache are common presenting symptoms of scleroderma renal crisis, which is caused by renal vascular damage leading to ischemia and activation of the renin-angiotensin system.
Proteinuria and elevated creatinine levels are indicative of kidney involvement, consistent with scleroderma renal crisis.

Question 7 Incorrect about Behcet’s syndrome is:

The incorrect statement about Behçet’s syndrome is:

A. There is a strong association with HLA-B7.

Explanation:
Behçet’s syndrome is strongly associated with HLA-B51, not HLA-B7. This genetic marker is more commonly linked to the disease, particularly in populations from the Middle East and Asia.

Skin hyperactivity to minor injury (e.g., venipuncture) is correct. This is known as the pathergy reaction, where a minor trauma can lead to exaggerated skin inflammation.

Behçet’s syndrome often involves an inflammatory reaction around large blood vessels (vasculitis), which can affect both arteries and veins.

Corticosteroid therapy is indeed of value in controlling the inflammatory symptoms of Behçet’s syndrome, particularly during acute flares.

 

 

1. A 2-year-old boy comes to you with a 1-week history of high-grade fever, conjunctivitis of both eyes without discharge, and fissuring of the lips. On examination, he has a generalized erythematous rash and tender lymph nodes. What would be the most appropriate management for this child?

Answer: a. IVIG with aspirin

Explanation:
This presentation is characteristic of Kawasaki disease, a pediatric vasculitis. The treatment involves Intravenous Immunoglobulin (IVIG) and aspirin to reduce the risk of coronary artery aneurysms and other complications. Paracetamol alone would not be sufficient, and corticosteroids or plasma exchange are not first-line treatments for Kawasaki disease.


2. A biopsy of erythema nodosum (EN) lesions of acute Lofgren sarcoidosis will show:

Answer: a. Lobular panniculitis without vasculitis

Explanation:
In Lofgren syndrome, a type of acute sarcoidosis, biopsy findings of erythema nodosum (EN) lobular panniculitis with or without vasculitis are characteristic of sarcoidosis.


3. A 25-year-old lady, who is a case of Rheumatoid arthritis on treatment with oral methotrexate for the last 3 years, wishes to conceive. Her disease had been well controlled on oral methotrexate 15mg/week for the last 2 years. What would be the best treatment strategy for safe conception and pregnancy?

Answer: c. To stop oral methotrexate 6 months prior to planned conception and transition her to alternate DMARDs and observe for disease activity before allowing her for pregnancy.

Explanation:
Methotrexate is teratogenic, and it is recommended to stop methotrexate at least 3-6 months before conception. This allows the drug to clear from the system and reduces the risk of fetal abnormalities. Transitioning to other safer DMARDs like hydroxychloroquine can be considered during this period.


4. A 35-year-old lady presents with flaccid quadriparesis, facial weakness, and breathing difficulty of a few days’ duration. Her bystanders deny any similar previous episodes. Which of the following may be true?

Answer: d. She has Guillain-Barré Syndrome (GBS) and needs IVIG and ventilation

Explanation:
This patient’s presentation suggests Guillain-Barré Syndrome (GBS), which can cause flaccid paralysis and respiratory failure. Treatment with IVIG or plasmapheresis is crucial, and she may require mechanical ventilation due to respiratory muscle weakness. Myasthenic crisis and other neuromuscular disorders should be ruled out, but GBS is the most likely given the acute onset and distribution.


5. A 54-year-old female with RA is treated with infliximab for refractory disease. All the following are potential side effects of this treatment except:

Answer: d. Pulmonary fibrosis

Explanation:
Infliximab, a TNF inhibitor, can cause side effects like demyelinating disorders, exacerbation of congestive heart failure (CCF), and increased risk of infections, including disseminated tuberculosis. Pulmonary fibrosis is not a commonly reported side effect of infliximab.


6. Which of the following joints are typically spared in osteoarthritis (OA)?

Answer: a. Ankle

Explanation:
Osteoarthritis typically affects weight-bearing joints like the knees, hips, and hands (especially the DIP joints). The ankle joint is usually spared in primary OA unless there has been previous trauma or injury.


7. A positive pathergy test is classically seen in:

Answer: a. Behcet’s Disease

Explanation:
A positive pathergy test is a skin hypersensitivity reaction that is classically associated with Behcet’s disease, a systemic vasculitis. It involves exaggerated skin injury in response to minor trauma.


8. In patients with Chronic kidney disease, which is the most important contributor to renal osteodystrophy?

Answer: a. Impaired renal production of 1,25-dihydroxyvitamin D3

Explanation:
Renal osteodystrophy results from impaired kidney function, leading to decreased production of 1,25-dihydroxyvitamin D3, which in turn leads to secondary hyperparathyroidism, calcium imbalance, and bone abnormalities. Hypocalcemia and phosphate retention are secondary effects, but the primary issue is the lack of vitamin D activation.


9. All of the following are characteristics of tumor lysis syndrome except:

Answer: b. Hypercalcemia

Explanation:
Tumor lysis syndrome is a metabolic emergency caused by the rapid breakdown of tumor cells, leading to hyperkalemia, hyperphosphatemia, hypocalcemia, hyperuricemia, and sometimes lactic acidosis. Hypercalcemia is not a feature; instead, hypocalcemia is common due to the binding of released phosphate to calcium.

10. Which of the following cytokines is signaled via the JAK-STAT pathway?
Answer: b. IL-6

Explanation:
The JAK-STAT (Janus kinase-signal transducer and activator of transcription) pathway is primarily associated with cytokines that signal via specific receptors, such as IL-6. IL-6 signals through a receptor that activates JAK, leading to downstream activation of the STAT transcription factors. IL-1 and TNF signal through different pathways (such as NF-κB and MAPK), while IL-17 signals through the IL-17 receptor leading to activation of other pathways like NF-κB, not JAK-STAT.


11. All of the following are disease activity indices for Rheumatoid arthritis except:
Answer: b. BASDAI

Explanation:
DAS-28 (Disease Activity Score), CDAI (Clinical Disease Activity Index), and SDAI (Simplified Disease Activity Index) are all commonly used to assess the disease activity of rheumatoid arthritis. BASDAI (Bath Ankylosing Spondylitis Disease Activity Index) is used to assess the activity of ankylosing spondylitis, not rheumatoid arthritis.


12. Which of the following drugs does not increase the risk of osteoporosis?
Answer: b. Oral contraceptives

Explanation:
Heparin, tacrolimus, and omeprazole are associated with an increased risk of osteoporosis. Heparin can lead to bone loss with prolonged use. Tacrolimus, an immunosuppressive drug, and omeprazole, a proton pump inhibitor, have both been linked to reduced bone density. Oral contraceptives, particularly those containing estrogen, can actually help maintain bone density and are not typically associated with an increased risk of osteoporosis.


13. A patient complains of numbness in his neck. Over months, the numbness has become more pronounced and involves a dense area bilaterally from the sternal notch to the area behind the ear. On examination, scalp sensation, cranial nerve function, and upper extremity motor examination are normal. The patient has decreased pain and temperature sensation in the distribution of C4. Vibration sense is normal. Cranial and caudal to the affected area, sensation is intact. Bladder and anal sphincter function are also normal. What is the most likely cause of this patient’s neurologic disorder?
Answer: c. Intramedullary tumor

Explanation:
The patient’s symptoms of localized loss of pain and temperature sensation in the distribution of C4 with preserved motor function and vibration sense suggest involvement of the spinothalamic tract, which is responsible for pain and temperature sensation. This is typical of an intramedullary spinal cord tumor, which can cause segmental dysfunction without affecting motor or bladder functions early in its course. The other options, such as ALS and neurosyphilis, typically present differently (ALS with motor involvement, neurosyphilis with other systemic signs).


14. A 34-year-old female complains of lower extremity weakness for the last 3 days. She has noted progressive weakness in the lower extremities with loss of sensation “below the belly button” and incontinence. She has had some low-grade fevers for the last week. She denies recent travel. Past medical history is unremarkable. Physical examination is notable for a sensory level at the level of the umbilicus. The lower extremities show +3/5 strength bilaterally proximally and distally. Reflexes, cerebellar examination, and mental status are normal. All of the following are appropriate steps in evaluating this patient except:
Answer: a. Electromyography

Explanation:
This patient’s presentation is concerning for transverse myelitis or an inflammatory spinal cord syndrome, given her sensory level and lower extremity weakness with fever. The immediate steps in evaluating her would include imaging (MRI of the spine) to assess for spinal cord compression or inflammation, lumbar puncture (LP) to analyze cerebrospinal fluid for infection or inflammation, and viral serologies. Electromyography (EMG) is not appropriate as an initial step because it is used to assess nerve or muscle disorders and would not directly help in diagnosing a spinal cord issue.


15. Which of the following is not a myositis-specific antibody?
Answer: a. Anti-Ro

Explanation:
Anti-Ro antibodies are associated with Sjögren’s syndrome and systemic lupus erythematosus (SLE), not myositis. Myositis-specific antibodies include Mi-2, SRP (Signal Recognition Particle), and Jo-1, which are associated with conditions like dermatomyositis and polymyositis.

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