Mixed Connective Tissue Disease — MCQs
A 34-year-old woman has Raynaud's phenomenon associated with systemic sclerosis (scleroderma). Which of the following is the most appropriate management for this condition?
Match the following: A) Caplan syndrome- 1) Found first in coal worker B) Asbestosis- 2) Upper lobe predominance C) Mesothelioma- 3) Involves lower lobe D) Sarcoidosis- 4) Pleural effusion is seen
Autoimmune diseases associated with Sjögren syndrome are all except?
A young patient presents to the clinic with erythematous lesions over the exposed areas of the skin like hands, arms, chest, etc. she also complaints of arthralgia and breathlessness. Which among the following antibodies will be useful in diagnosing this condition?
A plant prick can produce sporotrichosis. Which of the following statements about sporotrichosis is false?
A 40 year old male patient complains of severe acute abdominal pain. Radiologic examination reveals multiple gall stones. On histologic examination, foci of necrosis containing shadowy outlines of necrotic cells surrounded by basophilic calcium deposits were seen. Which type of necrosis is seen in this patient?
A male patient with purple striae, thin skin, non-healing wound, and pedunculated abdomen, most probable cause?
Which of the following is the correct combination?
All of the following statements about Lupus Anticoagulant are true, EXCEPT:
Which of the following statements about Prions is true:
Mixed Connective Tissue Disease Indian Medical PG Practice Questions and MCQs
Question 1: A 34-year-old woman has Raynaud's phenomenon associated with systemic sclerosis (scleroderma). Which of the following is the most appropriate management for this condition?
- A. ergotamine
- B. calcium channel blockers (nifedipine)
- C. beta-blocking drugs
- D. warm clothing (Correct Answer)
Explanation: ***Warm clothing*** - Maintaining **core body temperature** and direct protection of extremities from **cold exposure** is the primary non-pharmacological management for Raynaud's phenomenon. - This helps prevent the **vasospasm** triggered by cold, reducing the frequency and severity of attacks. *ergotamine* - **Ergotamine** is a powerful **vasoconstrictor** and would worsen Raynaud's phenomenon by further narrowing blood vessels. - It is primarily used for **migraine treatment** and is contraindicated in conditions involving vasoconstriction. *calcium channel blockers (nifedipine)* - While **calcium channel blockers** like nifedipine are often used as **second-line pharmacological treatment** for Raynaud's, warm clothing represents a more fundamental and universally applicable management strategy. [1] - Nifedipine works by causing **vasodilation**, which can reduce the severity and frequency of attacks when non-pharmacological methods are insufficient. [1] *beta-blocking drugs* - **Beta-blockers** can worsen Raynaud's phenomenon by causing **vasoconstriction** due to unopposed alpha-adrenergic activity. [1] - These drugs are generally **contraindicated** in patients with Raynaud's syndrome. [1]
Question 2: Match the following: A) Caplan syndrome- 1) Found first in coal worker B) Asbestosis- 2) Upper lobe predominance C) Mesothelioma- 3) Involves lower lobe D) Sarcoidosis- 4) Pleural effusion is seen
- A. A-3, B-4, C-2, D-1
- B. A-1, B-4, C-3, D-2 (Correct Answer)
- C. A-4, B-2, C-3, D-1
- D. A-2, B-4, C-3, D-1
Explanation: **A-1, B-4, C-3, D-2** - **Caplan syndrome** was first described in **coal workers** with **rheumatoid arthritis** and progressive massive fibrosis. - **Asbestosis** is often associated with **pleural effusion**, which can be benign or malignant. - **Mesothelioma** typically involves the **lower lobes** of the lungs, specifically the pleura, and is strongly linked to asbestos exposure. - **Sarcoidosis** is characterized by **non-caseating granulomas**, which have a predilection for the **upper lobes** of the lungs. *A-3, B-4, C-2, D-1* - This option incorrectly states that Caplan syndrome involves the lower lobe; **Caplan syndrome** is defined by the presence of large nodules in the lungs of coal workers with rheumatoid arthritis, and their specific lobar distribution is not a defining characteristic. - This option incorrectly states that Mesothelioma has an upper lobe predominance; **Mesothelioma** is a pleural malignancy and typically involves the **lower lobes**, extending along the pleura. *A-4, B-2, C-3, D-1* - This option incorrectly associates Caplan syndrome with pleural effusion; **Caplan syndrome** manifests as rheumatoid nodules in the lungs, not primarily pleural effusion. - This option incorrectly states that Asbestosis has an upper lobe predominance; **Asbestosis** predominantly affects the **lower lobes** of the lungs, causing interstitial fibrosis. *A-2, B-4, C-3, D-1* - This option incorrectly states that Caplan syndrome has an upper lobe predominance; the defining feature of **Caplan syndrome** is the combination of rheumatoid arthritis and pneumoconiosis, not specific lobar involvement. - This option correctly identifies pleural effusion with asbestosis and lower lobe involvement with mesothelioma, but **Caplan syndrome** is not characterized by upper lobe predominance.
Question 3: Autoimmune diseases associated with Sjögren syndrome are all except?
- A. RA
- B. SLE
- C. Mixed connective tissue disorder
- D. Addison's disease (Correct Answer)
Explanation: ***Addison's disease*** - While an **autoimmune disorder** itself (involving the adrenal glands), Addison's disease is generally **not directly associated** as a co-occurring autoimmune condition with Sjögren's syndrome. - Sjögren's syndrome is primarily characterized by **exocrine gland dysfunction**, specifically affecting lacrimal and salivary glands. *RA* - **Rheumatoid arthritis (RA)** is one of the most common autoimmune diseases found in association with **secondary Sjögren's syndrome**. - Sjögren's syndrome can occur in approximately **10-15% of patients with RA**, highlighting a strong pathogenic link. *SLE* - **Systemic lupus erythematosus (SLE)** frequently coexists with Sjögren's syndrome, often making it challenging to differentiate primary from secondary Sjögren's. - Both conditions share common **autoimmune mechanisms** and autoantibody profiles (e.g., anti-Ro/SSA and anti-La/SSB antibodies). *Mixed connective tissue disorder* - **Mixed connective tissue disease (MCTD)** is characterized by overlapping features of SLE, systemic sclerosis, and polymyositis, and also frequently includes features of **Sjögren's syndrome**. - The presence of **anti-U1-RNP antibodies** is a hallmark of MCTD, and these patients often experience sicca symptoms.
Question 4: A young patient presents to the clinic with erythematous lesions over the exposed areas of the skin like hands, arms, chest, etc. she also complaints of arthralgia and breathlessness. Which among the following antibodies will be useful in diagnosing this condition?
- A. Anti-centromere antibodies
- B. Anti-RNP antibodies
- C. Anti-dsDNA antibodies (Correct Answer)
- D. Anti-Scl 70 antibodies
Explanation: Anti-dsDNA antibodies - The clinical presentation with erythematous lesions in sun-exposed areas (consistent with photosensitivity [1] or malar rash), arthralgia, and breathlessness (suggesting serositis [4] or pulmonary involvement) is highly suggestive of Systemic Lupus Erythematosus (SLE) [3]. - Anti-dsDNA antibodies are highly specific for SLE and are included in the diagnostic criteria [4]. Their levels often correlate with disease activity, especially in lupus nephritis. *Anti-centromere antibodies* - These antibodies are typically associated with CREST syndrome (a limited form of systemic sclerosis), characterized by Calcinosis, Raynaud's phenomenon, Esophageal dysmotility, Sclerodactyly, and Telangiectasias. - This clinical picture does not align with the patient's symptoms, which point more towards an inflammatory multi-systemic disease. *Anti-RNP antibodies* - Anti-ribonucleoprotein (RNP) antibodies are characteristic of Mixed Connective Tissue Disease (MCTD) [2], which features overlapping symptoms of SLE, systemic sclerosis, and polymyositis [3]. - While some features like arthralgia might overlap, the classic erythematous lesions and breathlessness are more directly linked to SLE in this presentation, and anti-dsDNA is more specific for distinguishing SLE. *Anti-Scl 70 antibodies* - Also known as anti-topoisomerase I antibodies, these are highly specific for diffuse cutaneous systemic sclerosis. - This condition is characterized by widespread skin thickening, interstitial lung disease, and other visceral organ involvement, which is not fully supported by the patient's primary symptoms of prominent erythematous rashes and isolated arthralgia.
Question 5: A plant prick can produce sporotrichosis. Which of the following statements about sporotrichosis is false?
- A. Enlarged lymph nodes extending centripetally as a beaded chain are a characteristic finding
- B. Most cases are acquired via cutaneous inoculation
- C. It is an occupational disease of butchers, doctors (Correct Answer)
- D. Is a chronic mycotic disease that typically involves skin, subcutaneous tissue and regional lymphatics
Explanation: ***It is an occupational disease of butchers, doctors*** - Sporotrichosis is an **occupational hazard for gardeners, florists, and agricultural workers** due to exposure to decaying plant matter, not typically for butchers or doctors. - The disease is caused by **direct inoculation** of the fungus *Sporothrix schenckii* into the skin, often through a thorn prick or minor trauma. *Most cases are acquired via cutaneous inoculation* - This statement is **true** as sporotrichosis is primarily caused by **traumatic implantation** of fungal spores into the skin. - Common sources include **thorns, splinters, sphagnum moss**, and other plant materials. *Enlarged lymph nodes extending centripetally as a beaded chain are a characteristic finding* - This statement is **true** and describes the classic **lymphocutaneous sporotrichosis**, where lesions and **nodular lymphangitis** track along lymphatic channels. - The "beaded chain" appearance refers to the multiple subcutaneous nodules formed along the lymphatic vessels. *Is a chronic mycotic disease that typically involves skin, subcutaneous tissue and regional lymphatics* - This statement is **true** because sporotrichosis is a **slow-progressing fungal infection** that primarily affects the skin, the tissue just beneath the skin, and the lymphatics draining the infected area. - While systemic dissemination can occur in immunocompromised individuals, the **cutaneous and lymphocutaneous forms** are most common.
Question 6: A 40 year old male patient complains of severe acute abdominal pain. Radiologic examination reveals multiple gall stones. On histologic examination, foci of necrosis containing shadowy outlines of necrotic cells surrounded by basophilic calcium deposits were seen. Which type of necrosis is seen in this patient?
- A. Liquefactive
- B. Fibrinoid
- C. Coagulative
- D. Fat (Correct Answer)
Explanation: ***Fat*** - The description of **foci of necrosis** containing **shadowy outlines of necrotic cells** surrounded by **basophilic calcium deposits** is characteristic of **fat necrosis**, specifically enzymatic fat necrosis [1]. - This typically occurs in the **pancreas** during acute pancreatitis, where activated **lipases** released from damaged pancreatic cells digest fat, leading to the formation of **calcium soaps** [1], [2]. *Liquefactive* - This type of necrosis is characterized by the **dissolution of dead cells** into a viscous liquid mass, often seen in **brain infarcts** or **abscesses**. - It does not present with the shadowy outlines of necrotic cells or calcium deposits as described. *Fibrinoid* - **Fibrinoid necrosis** involves immune-mediated damage to blood vessel walls, where **fibrin** and plasma proteins deposit within the vessel wall. - It is typically seen in conditions like **vasculitis** or **malignant hypertension** and does not involve the saponification of fat. *Coagulative* - **Coagulative necrosis** is characterized by the preservation of the **architectural outline** of dead cells for several days, typically due to **ischemia** in solid organs (e.g., heart, kidney) [1]. - While cells are necrotic, there is no mention of **calcium deposits** or the specific fat saponification associated with the described findings. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Cellular Responses to Stress and Toxic Insults: Adaptation, Injury, and Death, pp. 53-55. [2] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Pancreas, pp. 889-890.
Question 7: A male patient with purple striae, thin skin, non-healing wound, and pedunculated abdomen, most probable cause?
- A. Insulin resistance
- B. Hypercortisolism (Correct Answer)
- C. Hypothyroidism
- D. Genetic connective tissue disorder
Explanation: Hypercortisolism - **Purple striae** are characteristic due to the breakdown of collagen and elastic fibers from excessive **cortisol**. - **Thin skin**, **non-healing wounds**, and a **pedunculated abdomen** (central obesity) are all classic signs of chronic high cortisol levels, as seen in **Cushing's syndrome** [1]. *Insulin resistance* - While insulin resistance can lead to conditions like **acanthosis nigricans** and **obesity**, it typically does not cause purple striae or thin skin directly. - It's often associated with **type 2 diabetes**, polycystic ovary syndrome, but not the specific dermatological features presented. *Hypothyroidism* - Hypothyroidism symptoms include **dry skin**, **coarse hair**, **fatigue**, and **weight gain**, but not typically purple striae or thin skin. - It can cause **non-pitting edema** (myxedema), which is distinct from the described skin changes. *Genetic connective tissue disorder* - Genetic connective tissue disorders like **Ehlers-Danlos syndrome** can cause thin, fragile skin and poor wound healing. - However, they do not typically present with the characteristic **purple striae** or **pedunculated abdomen** that point specifically to hypercortisolism.
Question 8: Which of the following is the correct combination?
- A. Cane fibre-Farmers lung
- B. Cold-Trench foot (Correct Answer)
- C. Light-Miner's nystagmus
- D. Pressure-Caisson's disease
Explanation: ***Correct Option: Cold-Trench foot*** - **Trench foot** is an occupational cold injury caused by prolonged exposure to **cold and wet conditions** - Commonly seen in soldiers, agricultural workers, and those working in cold, damp environments - Results in **non-freezing cold injury** with tissue damage from vasoconstriction and ischemia - This represents a valid occupational exposure-disease combination *Incorrect: Cane fibre-Farmers lung* - **Cane fibre (bagasse)** causes **Bagassosis**, NOT Farmer's lung - **Bagassosis**: Hypersensitivity pneumonitis from exposure to moldy sugarcane bagasse - **Farmer's lung**: Hypersensitivity pneumonitis from exposure to moldy hay or grain - While both are forms of extrinsic allergic alveolitis, they are distinct conditions with different causative agents *Incorrect: Light-Miner's nystagmus* - **Miner's nystagmus** is associated with working in **darkness or poor lighting**, not light exposure - Caused by prolonged work in dim illumination in underground mines - Involves involuntary oscillatory eye movements due to disruption of the oculomotor system - This combination is backwards - the exposure is lack of light, not light itself *Incorrect: Pressure-Caisson's disease* - **Caisson disease** (decompression sickness) is caused by **rapid reduction in ambient pressure**, not pressure exposure itself - Occurs when dissolved inert gases (nitrogen) form bubbles during rapid decompression - Seen in divers, tunnel workers, and caisson workers - The exposure is more accurately described as "pressure changes" or "decompression" rather than simply "pressure"
Question 9: All of the following statements about Lupus Anticoagulant are true, EXCEPT:
- A. May present with Recurrent Abortions
- B. May occur with minimal clinical manifestations
- C. Thrombotic spells can be followed by severe life threatening hemorrhage (Correct Answer)
- D. May present with an isolated prolongation of APTT
Explanation: Self-Correction: None of the provided 5 references directly address Lupus Anticoagulant or its specific diagnostic criteria and clinical manifestations such as placental thrombosis or isolated APTT prolongation. Reference discusses Antithrombin and Protein C/S deficiencies. Reference discusses von Willebrand Factor. Reference discusses Monitoring Anticoagulant Therapy generally. Reference discusses Disseminated Intravascular Coagulation (DIC). Reference discusses Systemic Lupus Erythematosus (SLE) skin manifestations and mentions livedo reticularis as a feature of antiphospholipid syndrome (APS) [1], but provides no detail regarding the specific claims about Lupus Anticoagulant tests or clinical exclusions asked in the question. Consequently, none of the references meet the required relevance score of 7 or higher for citation. ***Thrombotic spells can be followed by severe life threatening hemorrhage*** - While lupus anticoagulant (LA) is associated with both **thrombosis** and, less commonly, **bleeding**, severe, life-threatening hemorrhage as a direct follow-up to a thrombotic spell is not a typical pattern. LA primarily predisposes to **thrombosis**. - In the rare instances where bleeding occurs, it is usually due to acquired **factor deficiencies** or **thrombocytopenia**, conditions that can sometimes co-exist with antiphospholipid antibody syndrome (APS) but are not direct consequences of the thrombotic event itself. *May present with Recurrent Abortions* - **Recurrent abortions** (miscarriages) are a classic and well-recognized clinical manifestation of **antiphospholipid syndrome (APS)**, of which lupus anticoagulant is a key diagnostic criterion. - The presence of lupus anticoagulant indicates an increased risk for **placental thrombosis**, which can lead to fetal loss. *May occur with minimal clinical manifestations* - Some individuals test positive for lupus anticoagulant antibodies without experiencing any significant **thrombotic events** or other clinical symptoms of antiphospholipid syndrome for extended periods. - This condition is often referred to as **asymptomatic antiphospholipid antibody positivity**, highlighting that the presence of the antibody does not always immediately translate into severe clinical disease. *May present with an isolated prolongation of APTT* - Lupus anticoagulant is an in-vitro phenomenon that interferes with **phospholipid-dependent coagulation tests**, such as the **activated partial thromboplastin time (APTT)**, causing it to be prolonged. - This prolongation occurs because the antibody binds to phospholipids used in the assay, mimicking an **anticoagulant effect** in the test tube, even though the patient is actually hypercoagulable in vivo.
Question 10: Which of the following statements about Prions is true:
- A. They can be cultured in cell free media
- B. They are infectious proteins (Correct Answer)
- C. They have rich nuclear material
- D. They are made up of bacteria and virus
Explanation: ***They are infectious proteins*** - Prions are uniquely characterized as **infectious proteinaceous particles** that lack nucleic acids. - Their mechanism of pathogenicity involves inducing conformational changes in normal host proteins, leading to disease. *They can be cultured in cell free media* - Prions cannot be cultured in cell-free media because they are **not living organisms** and require host cells to propagate by converting normal proteins. - Their replication depends on the presence of the **normal prion protein (PrPC)** in the host. *They have rich nuclear material* - Prions are fundamentally **devoid of nucleic acids** (DNA or RNA), which is a defining characteristic distinguishing them from viruses, bacteria, and other microorganisms. - Their infectious nature is solely based on their **aberrant protein structure**. *They are made up of bacteria and virus* - Prions are distinct biological entities that are **neither bacteria nor viruses**; they are misfolded proteins. - They are much simpler in structure than even the smallest viruses, lacking the complex genetic material and cellular machinery found in bacteria and viruses.
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