Podcast
Questions and Answers
Which disease is characterized by granuloma formation primarily affecting the nose and airways?
Which disease is characterized by granuloma formation primarily affecting the nose and airways?
- Granulomatosis with Polyangitis (correct)
- Giant Cell Arteritis
- Eosinophilic Granulomatosis with Polyangitis
- Takayasu Arteritis
What is the most common presentation of Granulomatosis with Polyangitis?
What is the most common presentation of Granulomatosis with Polyangitis?
- Saddle Nose Deformity
- Pulmonary Infarction
- Glomerulonephritis
- Epistaxis and Nasal Crusting (correct)
What is a significant risk factor associated with Polyarteritis Nodosa?
What is a significant risk factor associated with Polyarteritis Nodosa?
- Diabetes Mellitus
- Hepatitis-B Virus (correct)
- Smoking
- Autoimmune Diseases
What serological test is used to aid in the diagnosis of Polyarteritis Nodosa?
What serological test is used to aid in the diagnosis of Polyarteritis Nodosa?
What is a common neurological symptom present in cases of Polyarteritis Nodosa?
What is a common neurological symptom present in cases of Polyarteritis Nodosa?
Which treatment modality is considered very effective for Granulomatosis with Polyangitis?
Which treatment modality is considered very effective for Granulomatosis with Polyangitis?
When diagnosing Granulomatosis with Polyangitis, which imaging study is helpful?
When diagnosing Granulomatosis with Polyangitis, which imaging study is helpful?
What is a hallmark skin manifestation of Polyarteritis Nodosa?
What is a hallmark skin manifestation of Polyarteritis Nodosa?
Flashcards
What is vasculitis?
What is vasculitis?
VASCULITIS is a group of disorders caused by inflammation and damage to blood vessels, leading to narrowing and reduced blood flow, which can affect various organs like the skin, kidney, lung, heart, brain and GIT.
What is Granulomatosis with Polyangitis (GPA)?
What is Granulomatosis with Polyangitis (GPA)?
GPA, also known as Wegener's Granulomatosis, is a type of vasculitis that mainly affects the nose, airways, and kidneys.
What are the most common symptoms of GPA?
What are the most common symptoms of GPA?
The most common presentation of GPA is nasal problems like nose bleeds (epistaxis), crusting, and sinusitis.
What blood test is used to diagnose GPA?
What blood test is used to diagnose GPA?
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What is Polyarteritis Nodosa (PAN)?
What is Polyarteritis Nodosa (PAN)?
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What are some common symptoms of PAN?
What are some common symptoms of PAN?
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What imaging technique is used to diagnose PAN?
What imaging technique is used to diagnose PAN?
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How is PAN treated?
How is PAN treated?
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Study Notes
Vasculitis Overview
- Vasculitis is a group of disorders causing inflammation and necrosis of blood vessels.
- This leads to vessel damage and narrowing, causing ischemia and affecting various organs (skin, kidneys, lungs, heart, brain, gastrointestinal tract).
- Different types exist with varying clinical presentations and organ involvement.
Types of Vasculitis
- Granulomatosis with Polyangiitis (GPA; formerly Wegener's Granulomatosis): Characterized by granuloma formation, predominantly affecting the nose, airways, and kidneys.
- Eosinophilic Granulomatosis with Polyangiitis (EGPA; formerly Churg-Strauss Syndrome)
- Takayasu Arteritis
- Kawasaki Disease
- Polyarteritis Nodosa
- Giant Cell Arteritis
- Henoch–Schönlein Purpura
- Behçet's disease
- Cryoglobulinaemic Vasculitis
- IgG4 Disease
Granulomatosis with Polyangiitis (GPA)
- Common Presentations: Epistaxis (nosebleeds), nasal crusting, and sinusitis.
- Lung Involvement: Cough, shortness of breath (dyspnea), coughing up blood (hemoptysis), lung nodules (common in most patients).
- Kidney Involvement: Glomerulonephritis (less common).
- Other Possible Symptoms: Hearing loss (deafness), nasal septum damage, saddle nose deformity.
- Diagnosis:
- C-ANCA (anti-neutrophil cytoplasmic antibody) serology.
- Chest X-ray/HRCT scan (high-resolution CT scan) that might show multiple cavities or masses in the lungs, plus potential lung biopsy.
- Elevated ESR (erythrocyte sedimentation rate) and CRP (C-reactive protein).
- Leukocytosis (high white blood cell count).
- Treatment: Glucocorticoids (steroids) and immunosuppressive therapies (like methotrexate or azathioprine). In some cases, rituximab combined with corticosteroids is very effective.
Polyarteritis Nodosa
- Age of Onset: Typically between ages 40 and 50.
- Gender Prevalence: Males are affected more often than females (2:1 ratio).
- Cause: Unknown; however, hepatitis B virus association is a significant risk factor.
- Skin Manifestations: Palpable purpura, ulcers, infarctions (tissue death), and livedo reticularis (a skin rash).
- Neurological Signs: Sensory and motor neuropathy (nerve damage) occurs in about 70% of patients.
- Kidney Involvement: Severe high blood pressure (HTN) and/or kidney impairment, possibly due to multiple kidney infarctions. Glomerulonephritis is less common.
- Diagnosis:
- P-ANCA (anti-neutrophil cytoplasmic antibody) serology.
- MRI angiography: helps identify multiple aneurysms (bulges) in the blood vessels.
- Muscle or sural nerve biopsies may be needed.
- Treatment: High-dose glucocorticoids and cyclophosphamide.
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