Vasculitides classification US Medical PG Practice Questions and MCQs
Practice US Medical PG questions for Vasculitides classification. These multiple choice questions (MCQs) cover important concepts and help you prepare for your exams.
Vasculitides classification US Medical PG Question 1: A 6-year-old boy presents to his pediatrician accompanied by his mother for evaluation of a rash. The rash appeared a little over a week ago, and since that time the boy has felt tired. He is less interested in playing outside, preferring to remain indoors because his knees and stomach hurt. His past medical history is significant for an upper respiratory infection that resolved uneventfully without treatment 2 weeks ago. Temperature is 99.5°F (37.5°C), blood pressure is 115/70 mmHg, pulse is 90/min, and respirations are 18/min. Physical exam shows scattered maroon macules and papules on the lower extremities. The abdomen is diffusely tender to palpation. There is no cervical lymphadenopathy or conjunctival injection. Which of the following will most likely be found in this patient?
- A. Coronary artery aneurysms
- B. Leukocytoclastic vasculitis (Correct Answer)
- C. Thrombocytopenia
- D. Mitral regurgitation
- E. Occult malignancy
Vasculitides classification Explanation: ***Leukocytoclastic vasculitis***
- This patient presents with symptoms highly suggestive of **Henoch-Schönlein Purpura (HSP)**, including a preceding URI, fatigue, low-grade fever, **abdominal pain**, and a **palpable purpura** primarily on the lower extremities.
- HSP is characterized by IgA-mediated **small-vessel vasculitis**, which histologically presents as leukocytoclastic vasculitis with IgA deposition on immunofluorescence.
*Coronary artery aneurysms*
- **Coronary artery aneurysms** are a classic complication of **Kawasaki disease**, not Henoch-Schönlein Purpura.
- Kawasaki disease presents with different clinical features, including prolonged fever, conjunctival injection, oral changes, and cervical lymphadenopathy.
*Thrombocytopenia*
- **Thrombocytopenia** is characterized by a low platelet count and often presents with petechiae, purpura, and bleeding, but the rash in HSP is due to inflammation and extravasation of red blood cells, not low platelets.
- Platelet counts in HSP are typically **normal** or can be slightly elevated as an acute phase reactant.
*Mitral regurgitation*
- **Mitral regurgitation** is a common manifestation of **rheumatic fever**, particularly after recurrent episodes, caused by valvular damage.
- Rheumatic fever is also preceded by infection (Group A Strep) but involves different symptoms like migratory polyarthritis, carditis, chorea, erythema marginatum, and subcutaneous nodules, which are not described here.
*Occult malignancy*
- While an **occult malignancy** can cause paraneoplastic syndromes or constitutional symptoms, the specific constellation of symptoms, including the migratory rash, abdominal pain, and preceding URI, points much more strongly to **HSP** in a 6-year-old.
- The presentation is more consistent with an acute, inflammatory process rather than a chronic, insidious malignant one.
Vasculitides classification US Medical PG Question 2: A 4-year-old boy is brought to the emergency department by his parents after 10 days of fever, varying from 38.0–40.0°C (100.4–104.0°F). On physical examination, the child is ill-looking with an extensive rash over his trunk with patchy desquamation. His hands are swollen, and he also shows signs of a bilateral conjunctivitis. The laboratory test results are as follows:
Hemoglobin 12.9 g/dL
Hematocrit 37.7%
Mean corpuscular volume 82.2 μm3
Leukocyte count 10,500/mm3
Neutrophils 65%
Lymphocytes 30%
Monocytes 5%
Platelet count 290,000/mm3
Erythrocyte sedimentation rate (ESR) 35 mm/h
What is the next best step in the management of this patient’s condition?
- A. High-dose aspirin (Correct Answer)
- B. Influenza vaccine
- C. Echocardiography
- D. Low-dose aspirin
- E. Corticosteroids
Vasculitides classification Explanation: ***High-dose aspirin***
- This patient presents with classic **Kawasaki disease**: prolonged fever (10 days), bilateral conjunctivitis, rash with desquamation, and extremity changes (swollen hands).
- Standard treatment for Kawasaki disease includes **IVIG (2 g/kg) plus high-dose aspirin (80-100 mg/kg/day)**. While both should be given together, **high-dose aspirin** is the best *therapeutic* option among those listed.
- High-dose aspirin provides anti-inflammatory effects during the acute phase and helps reduce fever and systemic inflammation.
- Treatment should be initiated promptly (ideally within 10 days of fever onset) to reduce the risk of **coronary artery aneurysms**.
*Influenza vaccine*
- The influenza vaccine is not a treatment for acute illness and has no role in managing Kawasaki disease.
- Vaccination would not address the ongoing systemic inflammation or prevent cardiac complications.
*Echocardiography*
- **Echocardiography should be performed** in all cases of Kawasaki disease to assess for coronary artery abnormalities, both at diagnosis and during follow-up.
- However, it is a **diagnostic/monitoring tool**, not a therapeutic intervention. Medical treatment to reduce inflammation takes priority over imaging.
- The question asks for the "next best step in management," which implies therapeutic action rather than diagnostic testing.
*Low-dose aspirin*
- Low-dose aspirin (3-5 mg/kg/day) is used during the **convalescent phase** for its antiplatelet effects, typically after fever resolution.
- It is transitioned to after the acute inflammatory phase is controlled with high-dose aspirin.
- Not appropriate for initial acute management where anti-inflammatory dosing is needed.
*Corticosteroids*
- Corticosteroids are reserved for **IVIG-refractory cases** or patients with severe coronary artery involvement.
- They are not part of initial first-line therapy and should not be used before IVIG administration.
- Their use is indicated only when standard therapy fails.
Vasculitides classification US Medical PG Question 3: A 53-year-old man comes to the emergency department because of a 1-month history of cough productive of small amounts of blood-tinged sputum. During this time, he has also developed fatigue, myalgia, and shortness of breath on exertion. He has had a 4-lb (2-kg) weight loss over the past 2 months. He has no personal history of serious illness. His mother has systemic lupus erythematosus. His temperature is 37.2°C (99.0 °F), pulse is 98/min, respirations are 22/min, and blood pressure is 152/98 mm Hg. Diffuse rhonchi are heard on auscultation of the chest bilaterally. There are multiple palpable, erythematous, nonblanching lesions on the lower extremities bilaterally. Laboratory studies show:
Leukocyte count 12,300 cells/mm3
Platelet count 400,000 cells/mm3
Erythrocyte sedimentation rate 83 mm/hr
Serum
Creatinine 2.1 mg/dL
Antinuclear antibody 1:40
Urine
Protein 3+
Blood 2+
RBC casts numerous
A biopsy specimen of the skin shows inflammation of the arterioles and capillaries without granuloma formation. Further evaluation of this patient is most likely to show which of the following findings?
- A. Hepatitis B surface antigen
- B. Increased serum cryoglobulins
- C. Myeloperoxidase antineutrophil cytoplasmic antibody (Correct Answer)
- D. Anti-glomerular basement membrane antibodies
- E. Anti-double stranded DNA antibodies
Vasculitides classification Explanation: ***Myeloperoxidase antineutrophil cytoplasmic antibody***
- The patient presents with **pulmonary-renal syndrome** and vasculitic skin lesions.
- The skin biopsy shows **inflammation of arterioles and capillaries without granuloma formation**, pointing towards a small vessel vasculitis such as **microscopic polyangiitis**, which is commonly associated with **MPO-ANCA (p-ANCA)** positivity.
*Hepatitis B surface antigen*
- **Polyarteritis nodosa** is a medium-sized vessel vasculitis often associated with **hepatitis B virus infection**.
- However, the skin biopsy showing **small vessel vasculitis** (arterioles and capillaries) makes polyarteritis nodosa less likely.
*Increased serum cryoglobulins*
- **Cryoglobulinemic vasculitis** is associated with **hepatitis C virus infection** and often presents with palpable purpura, arthralgias, and renal involvement.
- While the patient has palpable purpura and renal involvement, the absence of **hepatitis C risk factors** and the specific biopsy findings make this less probable than microscopic polyangiitis.
*Anti-glomerular basement membrane antibodies*
- **Goodpasture syndrome** is characterized by rapidly progressive glomerulonephritis and pulmonary hemorrhage due to **anti-GBM antibodies**.
- While the patient has both pulmonary and renal involvement, a skin vasculitis is not typical for Goodpasture syndrome, and the biopsy would show **linear IgG deposition** along the GBM, not inflammation of arterioles and capillaries.
*Anti-double stranded DNA antibodies*
- **Anti-dsDNA antibodies** are highly specific for **systemic lupus erythematosus (SLE)**, which can cause vasculitis and renal disease.
- While the patient's mother has SLE, his clinical presentation, particularly the lung involvement and the specific type of skin vasculitis, is more classic for an **ANCA-associated vasculitis** than for SLE.
Vasculitides classification US Medical PG Question 4: A 72-year-old man comes to the physician because of a 6-month history of intermittent dull abdominal pain that radiates to the back. He has smoked one pack of cigarettes daily for 50 years. His blood pressure is 145/80 mm Hg. Abdominal examination shows generalized tenderness and a pulsatile mass in the periumbilical region on deep palpation. Further evaluation of the affected blood vessel is most likely to show which of the following?
- A. Obliterative inflammation of the vasa vasorum
- B. Formation of giant cells in the tunica media
- C. Necrotizing inflammation of the entire vessel wall
- D. Fragmentation of elastic tissue in the tunica media (Correct Answer)
- E. Accumulation of foam cells in the tunica intima
Vasculitides classification Explanation: ***Fragmentation of elastic tissue in the tunica media***
- This patient's presentation with **intermittent dull abdominal pain radiating to the back**, a **pulsatile periumbilical mass**, and a history of **heavy smoking** is highly suggestive of an **abdominal aortic aneurysm (AAA)**.
- The pathological hallmark of AAA is **degradation and fragmentation of elastic tissue in the tunica media**, caused by chronic inflammation and increased activity of **matrix metalloproteinases (MMPs)**.
- This medial degeneration leads to **weakening of the vessel wall** and progressive **dilation**, ultimately forming an aneurysm.
- While atherosclerosis initiates the process, the actual aneurysm formation is characterized by this elastic tissue destruction in the media.
*Accumulation of foam cells in the tunica intima*
- This describes the **early lesion of atherosclerosis**, which is a **risk factor** for AAA development.
- However, when examining an **established AAA**, the predominant finding is not intimal foam cells but rather **medial degeneration** with elastic tissue fragmentation.
- Atherosclerosis is the underlying cause, but the question asks about findings in the affected vessel (the aneurysm itself).
*Obliterative inflammation of the vasa vasorum*
- This is characteristic of **syphilitic aortitis** (tertiary syphilis), which typically affects the **ascending thoracic aorta**.
- While syphilis can cause aneurysms, the patient's presentation and demographics are more consistent with atherosclerotic AAA.
*Formation of giant cells in the tunica media*
- This finding is associated with **giant cell arteritis** (temporal arteritis), which affects large and medium-sized arteries, particularly the temporal and ophthalmic arteries.
- It presents with headache, jaw claudication, and visual disturbances—features absent in this case.
*Necrotizing inflammation of the entire vessel wall*
- This describes **necrotizing vasculitis** such as **polyarteritis nodosa**, which affects medium-sized muscular arteries.
- While vasculitis can cause aneurysms, the clinical picture of AAA in an elderly smoker with atherosclerotic risk factors points to atherosclerotic pathogenesis, not primary vasculitis.
Vasculitides classification US Medical PG Question 5: A 75-year-old woman comes to the physician because of generalized weakness for 6 months. During this period, she has also had a 4-kg (8.8-lb) weight loss and frequent headaches. She has been avoiding eating solids because of severe jaw pain. She has hypertension and osteoporosis. She underwent a total left-sided knee arthroplasty 2 years ago because of osteoarthritis. The patient does not smoke or drink alcohol. Her current medications include enalapril, metoprolol, low-dose aspirin, and a multivitamin. She appears pale. Her temperature is 37.5°C (99.5°F), pulse is 82/min, and blood pressure is 135/80 mm Hg. Physical examination shows no abnormalities. Laboratory studies show:
Hemoglobin 10 g/dL
Mean corpuscular volume 87 μm3
Leukocyte count 8,500/mm3
Platelet count 450,000/mm3
Erythrocyte sedimentation rate 90 mm/h
Which of the following is the most appropriate next step in management?
- A. Temporal artery biopsy only
- B. Intravenous methylprednisolone only
- C. Intravenous methylprednisolone and temporal artery biopsy
- D. Oral prednisone and temporal artery biopsy (Correct Answer)
- E. Oral prednisone only
Vasculitides classification Explanation: ***Oral prednisone and temporal artery biopsy***
- The patient's symptoms (generalized weakness, weight loss, headaches, **jaw claudication**) and elevated **ESR** are highly suggestive of **giant cell arteritis**. Prompt initiation of high-dose oral corticosteroids (prednisone) is crucial to prevent irreversible vision loss.
- A **temporal artery biopsy** is necessary to confirm the diagnosis, but treatment should not be delayed while awaiting the biopsy results.
*Temporal artery biopsy only*
- While a **temporal artery biopsy** is essential for diagnosis, delaying treatment until after the biopsy significantly increases the risk of permanent complications, particularly **vision loss**.
- **Giant cell arteritis** is a medical emergency requiring immediate corticosteroid therapy.
*Intravenous methylprednisolone only*
- **Intravenous methylprednisolone** is typically reserved for cases with **severe vision loss** or other critical ischemic complications, which are not described here.
- While treatment should be initiated immediately, an **oral corticosteroid** generally suffices for initial management in the absence of severe symptoms, and it still requires a follow-up **biopsy**.
*Intravenous methylprednisolone and temporal artery biopsy*
- As mentioned, **IV methylprednisolone** is usually for more severe, vision-threatening cases. For this patient's presentation, **oral prednisone** is the appropriate initial corticosteroid.
- While both elements are part of management, the *route* of corticosteroid administration is typically oral for uncomplicated cases.
*Oral prednisone only*
- Initiating **oral prednisone** is appropriate to prevent complications like vision loss, but a definitive diagnosis requires a **temporal artery biopsy**.
- Without a biopsy, long-term corticosteroid therapy without histologic confirmation could lead to unnecessary side effects or mask an alternative diagnosis.
Vasculitides classification US Medical PG Question 6: A 45-year-old man comes to the physician because of fatigue and joint pain for 8 months. He has pain in both knees, both elbows, and diffuse muscle pain. He does not have dyspnea. He also had several episodes of a nonpruritic rash on his lower extremities. Eight years ago, the patient was diagnosed with hepatitis C. His temperature is 37.9°C (100.2°F), pulse is 90/min, and blood pressure is 140/90 mm Hg. Examination of the lower extremities shows raised purple papules that do not blanch when pressure is applied. Cardiopulmonary examination shows no abnormalities. Laboratory studies show:
Hemoglobin 13.9 g/dL
Leukocyte count 8,500/mm3
Platelets 160,000/mm3
Serum
Creatinine 1.1 mg/dL
ALT 123 U/L
AST 113 U/L
Further evaluation of this patient is most likely to show which of the following findings?
- A. Elevated IgA in serum
- B. Granulomatous inflammation of vessels
- C. Hypocomplementemia (Correct Answer)
- D. Elevated perinuclear anti-neutrophil cytoplasmic antibodies
- E. Positive pathergy test
Vasculitides classification Explanation: ***Hypocomplementemia***
- The patient's history of **hepatitis C** infection, along with **fatigue, polyarthralgia, elevated liver enzymes, and palpable purpura**, is classic for **HCV-associated mixed cryoglobulinemia syndrome**.
- **Mixed cryoglobulinemia** (types II and III) involves immune complex deposition, which activates and consumes complement, leading to **low C3 and C4 levels** (hypocomplementemia).
- **Hypocomplementemia** is a hallmark laboratory finding and helps distinguish cryoglobulinemic vasculitis from other small vessel vasculitides.
*Elevated IgA in serum*
- Elevated IgA levels are characteristic of **IgA vasculitis (Henoch-Schönlein purpura)**, which typically affects children and presents with palpable purpura, abdominal pain, and glomerulonephritis.
- While IgA vasculitis can occur in adults, the strong association with **hepatitis C infection** and the typical adult presentation point toward cryoglobulinemia rather than IgA vasculitis.
*Granulomatous inflammation of vessels*
- **Granulomatous inflammation of vessels** is a hallmark of **Granulomatosis with Polyangiitis (GPA)** or **Eosinophilic Granulomatosis with Polyangiitis (EGPA)**.
- These conditions typically present with upper/lower respiratory tract involvement, renal disease, and **ANCA positivity**, not the pattern seen here.
*Elevated perinuclear anti-neutrophil cytoplasmic antibodies*
- **p-ANCA** (perinuclear anti-neutrophil cytoplasmic antibodies) are primarily associated with **microscopic polyangiitis** and **eosinophilic granulomatosis with polyangiitis (EGPA)**.
- The clinical picture of **HCV-associated mixed cryoglobulinemia** does not typically involve ANCA positivity; instead, **rheumatoid factor** and **cryoglobulins** would be the relevant serologic markers.
*Positive pathergy test*
- A **positive pathergy test** is characteristic of **Behçet's disease**, an inflammatory disorder causing recurrent oral and genital ulcers, skin lesions, and uveitis.
- This condition does not align with the patient's presentation of palpable purpura, polyarthralgia, and HCV-associated systemic symptoms.
Vasculitides classification US Medical PG Question 7: A 40-year-old woman comes to the emergency department because of difficulty walking for the past 4 hours. She first noticed her symptoms after getting up this morning and her foot dragging while walking. She feels tired. She has a history of chronic sinusitis. Six months ago, she was diagnosed with asthma. Current medications include an albuterol inhaler and inhaled corticosteroids. Her temperature is 38.9°C (102°F), pulse is 80/min, and her blood pressure is 140/90 mm Hg. Auscultation of her lungs shows diffuse wheezing over bilateral lung fields. Physical examination shows tender subcutaneous nodules on the extensor surfaces of the elbows. There are palpable, non-blanching erythematous lesions on both shins. Dorsiflexion of the right foot is impaired. Sensation to pinprick, light touch, and vibration is decreased over the ulnar aspect of the left forearm. Laboratory studies show:
Hemoglobin 11.3 g/dL
Leukocyte count 24,500
Segmented neutrophils 48%
Eosinophils 29%
Lymphocytes 19%
Monocytes 4%
Platelet count 290,000/mm3
Serum
Urea nitrogen 32 mg/dL
Creatinine 1.85 mg/dL
Urine
Blood 2+
Protein 3+
Which of the following is the most likely diagnosis in this patient?
- A. Granulomatosis with polyangiitis
- B. Excessive glucocorticoid use
- C. Goodpasture syndrome
- D. Henoch-Schönlein purpura
- E. Eosinophilic granulomatosis with polyangiitis (Correct Answer)
Vasculitides classification Explanation: ***Eosinophilic granulomatosis with polyangiitis (EGPA)***
- This patient presents with a classic triad: **asthma**, **eosinophilia** (29%), and **multisystem vasculitis** as evidenced by mononeuropathy, skin lesions (nodules and palpable purpura), and kidney involvement.
- The history of chronic sinusitis, new-onset foot drop (mononeuropathy), **palpable purpura**, and elevated creatinine with proteinuria strongly point towards EGPA.
*Granulomatosis with polyangiitis (GPA)*
- While GPA can cause sinusitis, kidney disease, and neuropathy, it typically presents with **neutrophilic inflammation** and **c-ANCA** positivity, not prominent eosinophilia or severe asthma.
- Granulomatosis with polyangiitis typically involves the **upper and lower respiratory tracts** and kidneys but lacks the pronounced eosinophilia and severe asthma seen here.
*Excessive glucocorticoid use*
- This condition is associated with Cushingoid features, **osteoporosis**, and immunosuppression, none of which fully explain the patient's acute neurological deficits, eosinophilia, or vasculitic manifestations.
- Although the patient has asthma, her symptoms are not consistent with the side effects of inhaled corticosteroids or chronic systemic glucocorticoid use.
*Goodpasture syndrome*
- Goodpasture syndrome is characterized by **recurrent pulmonary hemorrhage** and rapidly progressive **glomerulonephritis** due to anti-GBM antibodies.
- It does not explain the prominent eosinophilia, asthma, or the presence of subcutaneous nodules and palpable purpura.
*Henoch-Schönlein purpura (HSP)*
- HSP typically presents in children with **palpable purpura** on the buttocks and lower extremities, **arthralgias**, abdominal pain, and **IgA nephropathy**.
- It does not involve significant eosinophilia, severe asthma, or mononeuropathy as seen in this adult patient.
Vasculitides classification US Medical PG Question 8: A 27-year-old man presents to his primary care physician with worsening cough and asthma. The patient reports that he was in his usual state of health until 1 month ago, when he developed a cold. Since then his cold has improved, but he continues to have a cough and worsening asthma symptoms. He says that he has been using his rescue inhaler 3 times a day with little improvement. He is studying for an accounting exam and states that his asthma is keeping him up at night and making it hard for him to focus during the day. The patient admits to smoking tobacco. His smoking has increased from a half pack per day since he was 17 years old to 1 pack per day during the past month to cope with the stress of his exam. The patient's temperature is 99°F (37.2°C), blood pressure is 110/74 mmHg, pulse is 75/min, and respirations are 15/min with an oxygen saturation of 97% on room air. Physical examination is notable for mild expiratory wheezes bilaterally. Labs are obtained, as shown below:
Serum:
Na+: 144 mEq/L
Cl-: 95 mEq/L
K+: 4.3 mEq/L
HCO3-: 23 mEq/L
Urea nitrogen: 24 mg/dL
Glucose: 100 mg/dL
Creatinine: 1.6 mg/dL
Leukocyte count and differential:
Leukocyte count: 13,000/mm^3
Segmented neutrophils: 63%
Eosinophils: 15%
Basophils: < 1%
Lymphocytes: 20%
Monocytes: 1.3%
Hemoglobin: 13.5 g/dL
Hematocrit: 50%
Platelets: 200,000/mm^3
Urinalysis reveals proteinuria and microscopic hematuria. Which of the following is associated with the patient's most likely diagnosis?
- A. IgA deposits
- B. Smoking
- C. c-ANCA levels
- D. Hepatitis B surface antigen
- E. p-ANCA levels (Correct Answer)
Vasculitides classification Explanation: ***p-ANCA levels***
- The patient presents with asthma, sinusitis-like symptoms (prior cold followed by worsening cough/asthma), eosinophilia (15%), and renal involvement (proteinuria, hematuria, elevated creatinine). This constellation of symptoms is highly suggestive of **Eosinophilic Granulomatosis with Polyangiitis (EGPA)**, formerly known as Churg-Strauss Syndrome.
- Approximately 30-40% of EGPA patients are positive for **p-ANCA (anti-myeloperoxidase antibodies)**, which are associated with the vasculitic phase and renal involvement.
*IgA deposits*
- **IgA deposits** are characteristic of **IgA nephropathy (Berger's disease)** or **Henoch-Schönlein purpura** (now IgA vasculitis), which typically present with hematuria and proteinuria, sometimes after an upper respiratory infection.
- However, these conditions do not typically cause severe asthma, significant eosinophilia, or a systemic vasculitis picture with pulmonary involvement as seen in this patient.
*Smoking*
- While the patient is a smoker and smoking can exacerbate asthma and contribute to chronic lung disease, it is not an *associated factor* with the underlying diagnosis of EGPA itself.
- Smoking is a risk factor for many respiratory illnesses but doesn't specifically point to EGPA in the context of the given clinical and laboratory findings.
*c-ANCA levels*
- **c-ANCA (anti-proteinase 3 antibodies)** are primarily associated with **Granulomatosis with Polyangiitis (GPA)**, formerly Wegener's granulomatosis.
- While GPA can present with kidney involvement and pulmonary symptoms, it typically involves the upper airways (sinusitis, otitis), lungs, and kidneys, but is usually *not* associated with severe asthma or prominent eosinophilia, which are key features in this patient.
*Hepatitis B surface antigen*
- **Hepatitis B surface antigen** positivity is associated with **polyarteritis nodosa (PAN)** due to immune complex deposition.
- PAN is a necrotizing vasculitis that can affect multiple organs but typically spare the lungs and is not associated with asthma or eosinophilia.
Vasculitides classification US Medical PG Question 9: A 62-year-old man presents to the emergency department with hematuria and hemoptysis that started in the morning. He notes that he has had frequent lung infections throughout his adult life, the most recent being 2 weeks ago. He also mentions that he has had hematuria twice before but never as severe as he is having currently. His medical history is otherwise non-significant, and his only medication is acetaminophen as needed. His blood pressure is 136/92 mm Hg, heart rate is 86/min, respiratory rate is 16/min, and temperature is 37.0°C (98.6°F). Chest radiography shows a resolving right middle lobe airspace opacity. His initial laboratory tests are notable for elevated erythrocyte sedimentation rate and C-reactive protein level. While in the examination room, the patient develops a spontaneous nosebleed. What is the most likely diagnosis?
- A. Goodpasture syndrome
- B. IgA nephropathy
- C. Minimal change disease
- D. Post-streptococcal glomerulonephritis
- E. Granulomatosis with polyangiitis (Correct Answer)
Vasculitides classification Explanation: ***Granulomatosis with polyangiitis***
- This patient presents with a **triad of upper airway (nosebleed), lower airway (hemoptysis, recurrent lung infections), and renal involvement (hematuria)**, which is classic for granulomatosis with polyangiitis (GPA), a form of ANCA-associated vasculitis.
- The elevated **ESR and CRP** indicate systemic inflammation, which is common in vasculitic conditions.
*Goodpasture syndrome*
- Characterized by **glomerulonephritis and pulmonary hemorrhage (hemoptysis)**, but typically does not involve the upper airways (e.g., nosebleeds).
- Diagnosis is confirmed by the presence of **anti-glomerular basement membrane antibodies**, which often presents more acutely.
*IgA nephropathy*
- Often presents with **recurrent episodes of gross hematuria**, frequently following an upper respiratory tract infection.
- While it involves the kidneys, it **does not typically cause pulmonary or upper airway symptoms** such as hemoptysis or recurrent lung opacities.
*Minimal change disease*
- Characterized by **nephrotic syndrome (proteinuria, hypoalbuminemia, edema)** and rarely presents with hematuria.
- **Does not cause pulmonary or upper airway manifestations** like hemoptysis or nosebleeds.
*Post-streptococcal glomerulonephritis*
- Typically occurs **1-3 weeks after a streptococcal infection** and presents with acute nephritic syndrome (hematuria, proteinuria, edema, hypertension).
- **Does not involve recurrent lung infections or hemoptysis** and is less likely in an adult with recurrent hematuria episodes.
Vasculitides classification US Medical PG Question 10: A 50-year-old woman comes to the emergency department because of fever and productive cough with blood in the sputum for 1 day. She also reports a sharp pain under her ribs that is worsened on taking deep breaths. Over the past 2 years, she has had repeated episodes of sinusitis, for which she used over the counter medication. She has recently started a new job at a wire-mesh factory. Her temperature is 38.3°C (100.9 °F), pulse is 72/min, respirations are 16/min, and blood pressure is 120/80 mm Hg. Physical examination shows palpable nonblanching skin lesions over her hands and feet. Examination of the nasal cavity shows ulcerations of the nasopharyngeal mucosa and a small septal perforation. Pulmonary examination shows stridor on inspiration. Laboratory studies show:
Hemoglobin 13.2 g/dL
Leukocyte count 10,300/mm3
Platelet count 205,000/mm3
Serum
Urea nitrogen 24 mg/dL
Creatinine 2.4 mg/dL
Urine
Protein 2+
RBC 70/hpf
RBC casts numerous
WBC 1–2/hpf
A chest x-ray shows multiple cavitating, nodular lesions bilaterally. Which of the following additional findings is most likely to be present in this patient?
- A. Increased p-ANCA titers
- B. Increased anti-Smith titers
- C. Increased anti-GBM titers
- D. Increased c-ANCA titers (Correct Answer)
- E. Decreased ADAMTS13 activity
Vasculitides classification Explanation: ### ***Increased c-ANCA titers***
- The patient's presentation with **sinusitis**, **nasal ulcerations**, **pulmonary cavitating nodules** with hemoptysis, and **rapidly progressive glomerulonephritis** (elevated creatinine, proteinuria, RBC casts) is highly indicative of **Granulomatosis with Polyangiitis (GPA)**.
- **c-ANCA (cytoplasmic antineutrophil cytoplasmic antibodies)**, primarily targeting **proteinase 3 (PR3)**, are serological markers commonly elevated in GPA and help confirm the diagnosis.
### *Increased p-ANCA titers*
- **p-ANCA (perinuclear antineutrophil cytoplasmic antibodies)**, typically targeting **myeloperoxidase (MPO)**, are more commonly associated with **microscopic polyangiitis** or **eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome)**.
- While both GPA and microscopic polyangiitis can present with kidney and lung involvement, the prominent upper airway disease (sinusitis, nasal ulcerations, septal perforation) in this patient points more strongly towards GPA and c-ANCA positivity.
### *Increased anti-Smith titers*
- **Anti-Smith antibodies** are highly specific for **Systemic Lupus Erythematosus (SLE)**.
- The clinical presentation of **upper airway destruction**, **cavitating lung lesions**, and **glomerulonephritis with RBC casts** is not typical for SLE, which more commonly presents with malar rash, arthritis, serositis, and other systemic symptoms.
### *Increased anti-GBM titers*
- **Anti-GBM (anti-glomerular basement membrane) antibodies** are characteristic of **Goodpasture syndrome**, which causes a **rapidly progressive glomerulonephritis** and often **pulmonary hemorrhage**.
- However, Goodpasture syndrome does not typically involve **upper airway disease** (sinusitis, nasal ulcerations, septal perforation) or **palpable skin lesions**, which are prominent features in this patient.
### *Decreased ADAMTS13 activity*
- **Decreased ADAMTS13 activity** is diagnostic of **Thrombotic Thrombocytopenic Purpura (TTP)**.
- TTP is characterized by the pentad of **thrombocytopenia**, **microangiopathic hemolytic anemia**, **neurological symptoms**, **renal insufficiency**, and **fever**, but it does not involve the prominent **destructive upper airway disease** or **cavitating lung lesions** seen in this patient.
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