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Inborn Errors of Immunity

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37 Intervention/management Strength of recommendation Certainty of evidence Use diagnostic skin biopsy with cultures as guidance for treatment Strong Very low PCR/immunohistochemistry for attenuated vaccine strain rubella virus Strong Moderate Topical corticosteroids for noninfectious rash Strong Moderate TNF-α inhibitors to treat progressive or refractory disease Conditional Very low Imaging for extracutaneous granulomatous disease Strong Very low Biologic/targeted molecular therapy for refractory disease Strong Moderate Adjunct measures to reduce colonization with S. aureus Conditional Moderate Topical cytotoxic therapies (e.g., cryoablation, salicylic acid or 5-flurouracil) and topical immunostimulants (e.g., imiquimod) Conditional Very low Mavorixafor (for WHIM) Strong Moderate Intervention/management Strength of recommendation Certainty of evidence Vitamin D replacement, if needed, adequate calcium dietary intake and/or supplementation, bisphosphonate therapy Conditional Moderate Consultation with dentist for extraction of retained incisors and molars Strong Moderate Standard treatment of SLE Conditional Moderate Nonsteroidal anti-inflammatory drugs (NSAIDs), corticosteroids, methotrexate, cyclophosphamide, azathioprine, rituximab Strong Low High-dose intravenous immunoglobulin (IVIG) Conditional Low NSAIDs, corticosteroids, and steroid-sparing agents such as methotrexate, sulfasalazine, cyclosporine, or biological agents Strong Moderate Physical therapy and exercise therapy Strong Moderate

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