Hyperglobulinemia in Sjögren's Syndrome: Prognostic Markers and Management

Prognostic Markers

Cryoglobulins and hypocomplementemia are the main prognostic markers for severe disease, according to the European League Against Rheumatism (EULAR) 1, 2
Anti-Ro/SS-A antibodies are the most specific autoantibodies in Sjögren's, as stated by the American College of Rheumatology 1
Antinuclear antibodies are the most frequently detected but less specific, as reported by the European League Against Rheumatism (EULAR) 1

The European League Against Rheumatism (EULAR) recommends not treating hyperglobulinemia itself, but rather treating the underlying systemic disease activity using ESSDAI-guided therapy 3, 4
The therapeutic approach should involve assessing disease activity systematically using ESSDAI to determine if systemic treatment is warranted, as suggested by the European League Against Rheumatism (EULAR) 3, 5
Systemic immunosuppression should be reserved for active systemic disease, not for isolated hyperglobulinemia or sicca symptoms alone, according to the American College of Rheumatology 1, 4
First-line systemic therapy should include glucocorticoids at minimum effective dose for the shortest duration, as recommended by the European League Against Rheumatism (EULAR) 3
Steroid-sparing agents such as cyclophosphamide, azathioprine, methotrexate, leflunomide, or mycophenolate may be used for maintenance, as suggested by the European League Against Rheumatism (EULAR) 3, 4
Rituximab may be used for refractory disease, particularly when hyperglobulinemia is associated with cryoglobulinemia, vasculitis, or risk of lymphoma, as recommended by the European League Against Rheumatism (EULAR) 3, 4