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Vasculitis

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In one line

·The immune system mistakenly attacks the walls of blood vessels, causing swelling, blockage, and bleeding that can damage any organ those vessels feed.

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Normal physiology

·Blood vessels are living tubes with three-layer walls. The inner layer (endothelium) is slick so blood flows smoothly. The middle layer has muscle that can tighten or relax to control blood pressure. The outer layer is tough connective tissue that holds the vessel in place. Together, these layers keep blood highways open, strong, and flexible.

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What goes wrong

·The immune system attacks the walls of blood vessels as if they were germs. White blood cells swarm the vessel wall, release chemicals that cause swelling (inflammation), and sometimes form clumps called granulomas (balls of angry immune cells). This attack thickens and narrows the vessel, slowing or blocking blood flow. It also weakens the wall so it can leak blood or burst. Which vessels get hit—big, medium, or small—determines which organs suffer.

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Hallmark signs

·Fever that won't go away
·Weight loss you didn't try for
·Purple or red spots on the skin (purpura)
·Numbness, tingling, or weakness in hands or feet
·Blood in the urine (hematuria)
·Jaw pain when chewing (jaw claudication)
·New, severe headache (often at the temples)
·Sudden vision loss or double vision

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Red flags · escalate now

·Sudden vision loss or new severe headache in someone over 50 (possible giant cell arteritis — needs urgent steroids to save sight)
·Blood in the urine plus swelling or high blood pressure (kidney damage can spiral quickly)
·Coughing up blood or fast-worsening shortness of breath (lungs may be bleeding or severely inflamed)
·Numbness, weakness, or stroke-like symptoms (brain blood vessels may be involved)

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Workup

·Red blood cell (Erythrocyte) sedimentation rate (ESR) and C-reactive protein (CRP)
·ANCA (antineutrophil cytoplasmic antibodies): PR3-ANCA and MPO-ANCA
·Urinalysis with microscopy
·Complete blood count (CBC) with differential
·Serum creatinine and estimated glomerular filtration rate (eGFR)
·Tissue biopsy (kidney, skin, lung, sural nerve, or temporal artery depending on the presentation)
·Hepatitis B and C serology, cryoglobulins, complement levels (C3, C4), antinuclear antibodies (ANA)
·Chest X-ray or high-resolution CT chest (if lung symptoms or suspicion of GPA/MPA/EGPA)

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Treatment

·High-dose corticosteroids (e.g., prednisone 1 mg/kg/day, usually 60–80 mg daily, or IV methylprednisolone 500–1000 mg daily for severe organ-threatening disease)
·Cyclophosphamide (IV or oral) or rituximab (anti-CD20 monoclonal antibody) for severe ANCA-associated vasculitis (GPA, MPA, EGPA with organ threat)
·Plasma exchange (plasmapheresis, PLEX) for severe pulmonary bleeding (hemorrhage) or rapidly progressive glomerulonephritis with dialysis-level kidney failure
·Low-dose aspirin (75–100 mg daily) for giant cell arteritis
·Tocilizumab (IL-6 receptor blocker, subcutaneous injection weekly or every other week) for giant cell arteritis
·Azathioprine, methotrexate, or mycophenolate mofetil for maintenance therapy after remission induction
·Prevention (Prophylaxis) against Pneumocystis jirovecii pneumonia (PCP) with trimethoprim-sulfamethoxazole during intense immunosuppression

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NCLEX trap

·The red or purple spots in vasculitis come from tiny blood vessels breaking and leaking blood into the skin, not from histamine release. You need steroids (drugs that calm down the whole immune system) and immunosuppressive medicines (drugs that stop immune cells from attacking) to halt the immune attack on the vessel walls—antihistamines do nothing here.
·Blood in the urine from vasculitis means the immune system is attacking the tiny filters in the kidneys right now. You must start high-dose steroids and immunosuppression immediately or the kidney filters will scar and stop working forever. This is a medical emergency—never wait.
·Fever in vasculitis comes from the immune system releasing inflammatory signals (cytokines) as it attacks blood vessel walls, not from bacteria or viruses. Antibiotics do not help. You need steroids to turn off the immune attack.
·Vasculitis hits many organs at once because blood vessels run everywhere. Always check kidneys (urine test for blood and protein), lungs (listen for crackles and get a chest x-ray), and nerves (test strength and sensation in hands and feet) even if you only see a skin rash. Missing organ damage kills people.
·Different types of vasculitis need different drugs. A patient with giant cell arteritis (inflammation of big arteries in the head) needs aspirin to prevent stroke, but a patient with ANCA-positive vasculitis (like granulomatosis with polyangiitis) needs cyclophosphamide or rituximab (strong immune-blocking drugs) along with steroids. Know the type first so you pick the right treatment.
·Vasculitis takes months to treat. The immune attack can still simmer even when you feel fine. Stopping steroids too fast causes a flare—the inflammation roars back and damages organs all over again. Keep treating until blood tests and urine tests show the inflammation is truly gone, usually 3 to 6 months or longer.

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