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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