Management of SLE-Induced Angioedema

Acute Management

For severe or organ-threatening manifestations, including airway involvement, administer pulses of intravenous methylprednisolone (250-1000 mg per day for 1-3 days) to provide immediate therapeutic effect, as recommended by the European League Against Rheumatism 1
Use glucocorticoids at doses appropriate to the severity of organ involvement, with initial oral prednisone at higher doses (0.5-1 mg/kg/day) with subsequent tapering, according to the European League Against Rheumatism 2

After acute control, transition to maintenance therapy with prednisone at <7.5 mg/day (or equivalent) with the goal of eventual withdrawal, as recommended by the European League Against Rheumatism 4
Prompt initiation of immunomodulatory/immunosuppressive agents, such as azathioprine, mycophenolate, or methotrexate, to expedite steroid tapering, according to the European League Against Rheumatism 1, 3

Consider add-on belimumab for persistently active or flaring disease, as recommended by the European League Against Rheumatism 5
Rituximab may be considered in organ-threatening, refractory disease, according to the European League Against Rheumatism 3
Cyclophosphamide can be used for severe organ-threatening disease or as "rescue" therapy, as recommended by the European League Against Rheumatism 4

Regular monitoring of disease activity using validated indices, such as the British Isles Lupus Assessment Group (BILAG) index, is recommended by the European League Against Rheumatism 6
Monitor complement levels (C3, C4) and anti-dsDNA antibodies to assess disease activity, as recommended by the European League Against Rheumatism 6
Screen for infections, which are a common complication of immunosuppressive therapy, according to the European League Against Rheumatism 7

Achieve remission or low disease activity in all organ systems, as recommended by the European League Against Rheumatism 1
Prevent disease flares and recurrence of angioedema, according to the European League Against Rheumatism 1
Minimize glucocorticoid exposure to reduce adverse effects, as recommended by the European League Against Rheumatism 4
Improve long-term survival and quality of life, according to the European League Against Rheumatism 5