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Lupus & Vasculitis: Connection, Types, Symptoms & Treatment
The Lupus-Vasculitis Link
Vasculitis = blood vessel inflammation. In SLE, immune complexes and autoantibodies deposit in vessel walls, activating complement and recruiting leukocytes → vessel damage. Occurs in ~10–30% of SLE patients.
Types of Lupus Vasculitis
1. Cutaneous (Small Vessel) Vasculitis
- Most common lupus vasculitis.
- Palpable purpura (lower extremities), urticarial vasculitis (lesions >24 hr, residual hyperpigmentation), livedo reticularis.
- Ulcers, digital infarcts, nailfold infarcts.
- Skin biopsy: leukocytoclastic vasculitis (neutrophils, nuclear dust, immune complexes).
2. Systemic (Medium/Large Vessel) Vasculitis
- Rare but serious.
- Polyarteritis nodosa (PAN)-like: mesenteric, renal, coronary, cerebral arteries.
- Takayasu-like: large vessel (aorta, subclavian) stenosis/aneurysm.
- CNS vasculitis: stroke, encephalopathy.
Clinical Presentation
- Skin: Purpura, ulcers, livedo, nodules.
- Constitutional: Fever, fatigue, weight loss.
- Organ-specific: Abdominal pain (mesenteric), hematuria/renal failure, mononeuritis multiplex, stroke, cardiac ischemia.
- Labs: Elevated ESR/CRP, low complement, anemia, elevated creatinine.
Diagnosis
- Clinical context + labs + imaging (CT angiography, MRA).
- Biopsy of affected tissue (skin, nerve, kidney) — gold standard.
- ANCA usually negative (distinguishes from primary AAV).
- Exclude infections, malignancy, drug reactions.
Treatment
Severity-driven:
- Mild cutaneous: Sun protection, topical steroids, hydroxychloroquine, colchicine, dapsone.
- Moderate-severe / systemic: High-dose glucocorticoids + immunosuppressant (cyclophosphamide, mycophenolate, azathioprine, rituximab).
- Refractory: IVIG, plasma exchange, belimumab, anifrolumab.
- Antiphospholipid syndrome overlap: Anticoagulation (not immunosuppression).
Prognosis
- Cutaneous: Generally good; recurrent flares manageable.
- Systemic: Guarded; depends on organ involvement, treatment response.
- Early diagnosis and aggressive immunosuppression improve outcomes.
- Long-term: Monitor for atherosclerosis (accelerated by vasculitis + steroids).
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