What is the treatment for abnormal autoimmune issues?

Treatment of Abnormal Autoimmune Issues

The treatment of abnormal autoimmune issues should be tailored to the specific autoimmune condition, with corticosteroids being the first-line therapy for most autoimmune disorders, followed by disease-specific immunosuppressive agents based on the organ system involved and severity of disease. 1, 2

First-Line Treatment Options

Corticosteroids

• Prednisone 1-2 mg/kg/day is the standard first-line treatment for most autoimmune conditions, with an expected response rate of 70-80% in conditions like autoimmune hemolytic anemia 1
• For moderate disease, oral prednisone 0.5-1 mg/kg/day is recommended; for severe disease, intravenous methylprednisolone 1-2 mg/kg/day is preferred 1
• Corticosteroid tapering should be gradual over at least 1 month after improvement to prevent relapse 2

Disease-Specific First-Line Treatments

• For systemic autoimmune rheumatic diseases with interstitial lung disease (SARD-ILD), mycophenolate is conditionally recommended as the preferred first-line therapy 2
• For autoimmune encephalitis, after ruling out infection, high-dose corticosteroids are recommended as first-line treatment, with IVIG or plasma exchange (PLEX) as alternatives if steroids are contraindicated 2
• For immune checkpoint inhibitor-related autoimmune issues, treatment depends on severity, with temporary suspension of immunotherapy and corticosteroids for moderate to severe cases 2

Second-Line Treatment Options

For Refractory Disease

• For autoimmune hemolytic anemia not responding to corticosteroids, consider immunosuppressive agents such as cyclosporine, mycophenolate mofetil, or azathioprine 1
• For autoimmune encephalitis without improvement after initial therapy, add IVIG or PLEX; consider rituximab for antibody-mediated autoimmunity or cyclophosphamide for cell-mediated autoimmunity 2
• For SARD-ILD with disease progression despite first-line therapy, options include rituximab, nintedanib, tocilizumab, or cyclophosphamide 2

Biologic Therapies

• TNF inhibitors like etanercept may be considered for certain autoimmune conditions, but should be used with caution due to potential side effects including serious infections, malignancies, and paradoxical autoimmunity 3
• For rapidly progressive interstitial lung disease (RP-ILD) in systemic autoimmune rheumatic diseases, rituximab, cyclophosphamide, IVIG, mycophenolate, calcineurin inhibitors, and JAK inhibitors are conditionally recommended as first-line options 2

Treatment Based on Specific Autoimmune Conditions

Autoimmune Hematologic Disorders

• For autoimmune cytopenias, monitor complete blood counts regularly with frequency based on severity 4
• Folic acid supplementation of 1 mg daily should be administered to support increased erythropoiesis in autoimmune hemolytic anemia 1
• For severe lymphopenia (<250 lymphocytes/μL), initiate Pneumocystis jirovecii prophylaxis and consider CMV screening 4

Autoimmune Neurological Disorders

• For autoimmune encephalitis with severe initial presentation, consider combination therapy with steroids/IVIG or steroids/PLEX from the beginning rather than sequential therapy 2
• If no improvement with conventional second-line therapies, consider novel approaches such as tocilizumab or bortezomib 2

Autoimmune Rheumatic Disorders with Lung Involvement

• For systemic sclerosis-associated ILD, strongly avoid glucocorticoids as first-line treatment due to risk of scleroderma renal crisis 2
• For rapidly progressive ILD, consider upfront combination therapy (triple therapy for those with confirmed or suspected MDA-5 and double or triple therapy for those without) over monotherapy 2
• Early referral for lung transplantation is conditionally recommended over later referral after progression on optimal medical management 2

Special Considerations

Patients with History of Autoimmune Disease

• Patients with a history of autoimmune diseases were often excluded from clinical trials of newer immunotherapies 2
• Treatment decisions should be based on individual potential benefits and expected risks 2
• Patients with vitiligo or endocrine deficiencies adequately controlled with substitutive treatment can be prescribed immune checkpoint inhibitors with close monitoring 2

Pregnancy and Breastfeeding

• Most immunosuppressive medications should not be used during pregnancy unless the clinical benefit outweighs the potential risk 2
• Women of childbearing potential should use effective contraception during treatment and for at least 6 months after the last dose of immunosuppressive therapy 2

Elderly Patients

• No overall differences in safety were reported in elderly patients (≥65 years old) across different approved immune checkpoint inhibitors 2
• No dose adjustment is recommended based on age alone 2

Monitoring and Follow-up

• Regular monitoring of disease activity markers and medication side effects is essential 4
• Patients should be under close monitoring as immune-related adverse events may occur at any time: at the beginning, during, or even after treatment discontinuation 2
• Surveillance should continue for 1 year after immunotherapy discontinuation 2
• For patients on long-term steroids, monitor for steroid-related complications, including hyperglycemia, hypertension, mood changes, insomnia, and fluid retention 1

Important Pitfalls to Avoid

• Delaying treatment in severe cases can increase morbidity and mortality 1
• Distinguishing between disease flare, medication effect, and new-onset infection can be challenging 4
• A too rapid decrease in corticosteroid dose may cause a relapse or worsening of adverse effects 2
• Patients on immunosuppression should receive antibiotic prophylaxis to prevent opportunistic infections when appropriate 2
• Avoid live vaccines during immunosuppressive therapy 3