What are the clinical manifestations and treatment options for Adult Immunoglobulin A (IgA) Vasculitis?

From the Guidelines

Adult IgA vasculitis should be treated with glucocorticoids, such as oral prednisone or pulsed intravenous methylprednisolone, for patients with mild or moderate IgAV nephropathy, while those with severe disease and renal involvement may require additional immunosuppressive therapy, including cyclophosphamide or rituximab, as well as supportive care measures like angiotensin-converting enzyme inhibitors for proteinuria. The clinical manifestations of Adult Immunoglobulin A (IgA) Vasculitis include palpable purpura, arthralgia, gastrointestinal symptoms, and potential kidney involvement, with a higher risk of chronic kidney disease compared to childhood cases 1.

Disease Assessment and Treatment

• Adult patients with IgAV should be assessed for secondary causes and malignancy with age- and sex-appropriate screening tests 1.
• Supportive care measures, such as NSAIDs like ibuprofen for pain and inflammation, may be sufficient for mild cases with primarily skin and joint manifestations.
• For moderate disease with significant gastrointestinal symptoms, prednisone at 0.5-1mg/kg/day for 1-2 weeks followed by a gradual taper over 2-4 weeks is recommended, similar to the treatment approach for IgAN 1.
• Severe cases with renal involvement, such as those with rapidly progressive glomerulonephritis (RPGN), may require more aggressive therapy, including high-dose methylprednisolone and possibly cyclophosphamide or rituximab, as well as plasma exchange to accelerate recovery in patients with life- or organ-threatening extrarenal complications of IgAV 1.

Renal Involvement and Management

• The majority of IgAV patients who will develop nephritis will do so within 3 months of presentation, highlighting the need for regular monitoring of blood pressure, urinalysis, and kidney function 1.
• Angiotensin-converting enzyme inhibitors like enalapril or angiotensin receptor blockers may be added for proteinuria, as part of the supportive care measures for patients with IgAV nephropathy 1.
• The treatment approach for IgAV nephropathy with RPGN is similar to that for rapidly progressive IgAN, emphasizing the importance of prompt and aggressive treatment to prevent long-term kidney damage 1.

From the Research

Clinical Manifestations of Adult Immunoglobulin A (IgA) Vasculitis

• The clinical course of IgA vasculitis (IgAV) in adults appears to be different from pediatric IgAV, especially due to its higher risk of evolving into end-stage renal disease 2
• The disease is noted for its heterogeneous presentation with varying severity, and its main manifestations are cutaneous purpura, arthralgias or arthritis, acute enteritis, and glomerulonephritis 3
• Poor outcomes are often due to the high frequency of glomerulonephritis in IgAV, also known as IgA vasculitis-associated nephritis (IgAVN) 4

Treatment Options for Adult Immunoglobulin A (IgA) Vasculitis

• Glucocorticoids are the first-line therapy for IgAV, especially in adults with severe manifestations 5, 3
• Colchicine, dapsone, and methotrexate can be useful for controlling minor manifestations 5
• Several immunomodulatory agents, such as cyclosporine A, tacrolimus, and mycophenolate mofetil, have shown favorable results as glucocorticoid-sparing agents 5
• Rituximab has demonstrated efficacy in reducing relapse frequency, lowering the cumulative glucocorticoid burden, and achieving long-term remission of the disease in children and adults with IgAV 6, 5, 3
• Immunoglobulins and plasma exchange therapy can also be useful in difficult and life-threatening situations 5
• Other potential therapies with encouraging results include TRF-budesonide, B-cell-directed therapy, B-cell-depleting agents, sodium-glucose cotransporter-2 inhibitors, endothelin receptor antagonists, and complement pathway inhibitors 5

Controversies and Future Directions in Treatment

• There is still controversy over the role of glucocorticoid (GC) and different immunosuppressive therapies such as cyclophosphamide, rituximab, and mycophenolate mofetil for more severe IgAV 2
• High-quality evidence or guidelines in the treatment of severe IgAV are lacking, and there is still a great need for controlled trials 2, 6, 3
• Emerging and promising novel therapies in IgAVN/IgAN, such as leflunomide, require further study 5, 4