Rheumatoid Vasculitis

Background

• Rheumatoid vasculitis is a rare, potentially serious complication of rheumatoid arthritis (RA), particularly in longstanding disease, that is characterized by inflammation of primarily small-and medium-sized blood vessels.
  • It is the most serious extra-articular manifestation of RA, associated with high morbidity and mortality.
  • The skin and peripheral nervous system are most commonly involved, although any organ system can be affected.
• Typically occurs in older patients with longstanding, erosive, deforming disease.
  • Reported to be more common in men.
  • Reported to occur in about 1%-5% of patients with rheumatoid disease.
• The precise cause and pathogenesis of rheumatoid vasculitis is not well understood, but appears to involve both immune complex-mediated and cytotoxic processes, leading to vessel wall inflammation, injury, necrosis, thrombus formation, and tissue ischemia.

Evaluation

• Suspect rheumatoid vasculitis in patients with seropositive rheumatoid arthritis (RA), who have developed skin lesions or neuropathy.
• Diagnosis of rheumatoid vasculitis is typically based on a combination of
  • clinical presentation
  • blood tests
  • imaging studies (if indicated depending on organ involvement)
  • tissue biopsy demonstrating histological evidence of small-to-medium-vessel vasculitis in the affected organ (necessary for definitive diagnosis)
  • exclusion of other causes of vasculitic involvement
• Scott and Bacon criteria for the diagnosis of rheumatoid vasculitis includes the presence of ≥ 1 of the following in a patient with RA:
  • mononeuritis multiplex
  • peripheral gangrene
  • biopsy evidence of acute necrotizing arteritis plus systemic illness (such as fever, weight loss)
  • deep cutaneous ulcers or extra-articular disease (such as pleurisy, pericarditis, scleritis) if associated with typical digital infarcts or biopsy evidence of vasculitis
• Early, acute diagnosis of rheumatoid vasculitis is crucial in order to initiate appropriate treatment to control symptoms, and minimize morbidity and mortality associated with the disease.
• For classifying mild-to-moderate disease vs. severe disease:
  • characteristics of mild-to-moderate disease typically include
    • isolated nail fold infarcts (limited cutaneous disease)
    • pericarditis
  • characteristics of severe disease typically include
    • necrotizing arteritis on histopathology
    • vasculitic neuropathy
    • major end organ involvement, such as
      • digital or extremity ischemia
      • scleritis
      • peripheral ulcerative keratitis
      • mononeuritis multiplex
      • involvement of heart (excluding pericarditis), lungs, kidneys, central nervous system, and/or gastrointestinal tract

Management

• General management considerations:
  • If biopsy is not feasible, initiation of treatment should be considered in the setting of a strong clinical suspicion of rheumatoid vasculitis (following exclusion of other causes).
  • No standard has been established for the management of rheumatoid vasculitis.
    • A comprehensive assessment is imperative in determining an optimal treatment strategy, and should be performed prior to making definitive treatment decisions.
    • Treatment is typically guided by
      • disease severity
      • extent of involvement
      • chronicity of vasculitic manifestations
      • presence of other extra-articular manifestations
  • Management of rheumatoid vasculitis typically requires aggressive pharmacologic therapies; however, adjunctive measures may include
    • smoking cessation
    • control of blood pressure
    • localized treatment of skin ulcers
• In patients with mild-to-moderate disease, a less aggressive management approach using oral disease-modifying anti-rheumatic drug (DMARD) therapy may be appropriate.
• In patients with severe disease:
  • Management typically involves use of
    • high-dose corticosteroids
    • aggressive immunosuppressive therapy, commonly cyclophosphamide
  • Use of biologic therapy may also be considered.