Vasculitis

Disease Description (Causes and Symptoms)

• Definition: A group of diseases in which inflammation develops in the walls of blood vessels. Symptoms and prognosis vary depending on the location of the inflammation (large, medium, or small vessels). Causes include autoimmune reactions, infections, malignancies, and drug-induced reactions; in many cases, immune mechanisms are involved.
• Pathophysiology summary: When inflammation develops in the vessel wall, the vessel lumen may narrow or become blocked, and vascular leakage can cause bleeding and edema. When capillaries or small venules are involved, palpable purpura is common, and in severe cases, organ ischemia or bleeding (in the kidneys, gastrointestinal tract, lungs, nervous system, etc.) may occur.
• Main symptoms: Fever, generalized fatigue, joint pain, skin spots and petechial bleeding (purpura), abdominal pain, bloody stools, and black stools (gastrointestinal involvement), hematuria and proteinuria (kidney involvement), respiratory symptoms (cough, hemoptysis, pulmonary hemorrhage), neurological symptoms (paralysis, sensory disturbances), and orchitis (testicular pain) has been reported in children.

Treatment Methods and Precautions

• Basic principles: Treatment is determined according to the cause and severity of the inflammation and the organs involved. The goals are to relieve symptoms and prevent organ damage.
• Drug therapy (general standard)
  • Mild cases (skin lesions only, etc.): Conservative treatment (rest, topical treatment) and consideration of nonsteroidal anti-inflammatory drugs (for pain control).
  • Moderate to severe cases (abdominal pain, kidney involvement, respiratory tract bleeding, etc.): Oral or intravenous corticosteroids (e.g., prednisolone/prednisone).
  • If corticosteroids are insufficient or in cases of relapse or severe disease: Immunosuppressants (such as cyclophosphamide, azathioprine, methotrexate, and mycophenolate) may be used.
  • For specific mechanisms or diseases, biologic agents (e.g., rituximab) or IVIG may be indicated.
  • In acute severe complications (rapid deterioration of kidney function, pulmonary hemorrhage, etc.), plasmapheresis or intensive care may be necessary.
• Children (e.g., IgA vasculitis/Henoch–Schönlein purpura): The natural course is generally favorable, and conservative treatment is prioritized; corticosteroids may be considered in cases of severe abdominal pain or kidney involvement.
• Lifestyle management and preventive measures
  • Infection prevention: The risk of infection increases during immunosuppressive treatment, so wash your hands, seek prompt medical attention for acute infections, and receive vaccinations when necessary (completion of possible vaccinations before starting immunosuppression is recommended; live vaccines are contraindicated during immunosuppression).
  • Check for possible drug-induced causes: Recently taken medications (such as antibiotics, antipyretics, and anticoagulants) may be the cause, so consult a doctor about discontinuing or changing them.
• Precautions (including the risk of complications)
  • Kidney involvement (glomerulonephritis) → may progress to chronic kidney failure (regular urine and kidney function tests are necessary).
  • Severe gastrointestinal bleeding, intestinal perforation, pulmonary hemorrhage, and central nervous system involvement are emergencies requiring immediate treatment.
  • With long-term use of corticosteroids or immunosuppressants, side effects such as infections, osteoporosis, hyperglycemia, and weight gain require management (including osteoporosis prevention and monitoring of blood glucose and blood pressure).

Checklist Before an Outpatient Visit (Including Suggested Items to Bring and Questions)

• Items to bring (recommended)
  • A list of all medications currently being taken (prescription drugs, OTC medications, herbal medicines, etc.), including the time and dosage of each medication
  • Copies or summaries of recent test results (urinalysis, blood tests (CBC, creatinine, liver function tests, inflammatory markers), ANCA/immune tests, imaging materials, etc.)
  • Photos of symptoms such as skin lesions and abdominal pain (when worsened), along with records of when symptoms began and how they have progressed
  • Records of emergency symptoms such as fever, black stools, or blood during urination
• Basic items to check during an outpatient visit
  • Fever, skin lesions (location and characteristics), abdominal pain (location, frequency, and changes in stools), presence or absence of hematuria/proteinuria, respiratory symptoms, neurological symptoms, and recent infections or medication use
• Examples of questions (to prepare in advance for the medical staff)
  • Could any medications be worsening my current symptoms?
  • What additional tests should I undergo (kidney function tests, immune tests, biopsy, etc.)?
  • If kidney involvement is suspected, when will I be referred to a nephrologist (nephrology department)?

General Examples of Questions to Ask the Doctor

• What are the main side effects of this medication (corticosteroid or immunosuppressant), and how can they be prevented?
• After treatment begins, which tests will be used to monitor effectiveness and side effects (frequency and items)?
• How high is the risk of relapse, and how will treatment differ if a relapse occurs?
• I am planning a pregnancy or breastfeeding. Are there any restrictions on treatment choices?
• Which vaccinations do you recommend during immunosuppressive treatment (timing and type)?

Examples of Disease-Specific Questions

• My urinalysis (or blood in my urine) remains abnormal. What tests should I undergo, and when, to confirm kidney involvement, and what immediate treatment will I receive if positive findings are detected?
• When gastrointestinal symptoms such as abdominal pain or black stools are severe, what symptoms require a visit to the emergency room (or what signs indicate that I should seek immediate medical attention)?