Neonatal and Pediatric Lupus Erythematosus Medication
- Author: Jeffrey P Callen, MD; Chief Editor: Lawrence K Jung, MD more...
Medication Summary
Neonatal lupus erythematosus (NLE) does not require specific therapy. Some research suggests that infants from subsequent pregnancies are less likely to be affected by cardiac neonatal lupus erythematosus if the mother is treated with prednisolone, dexamethasone, or betamethasone.
Sunscreens are also useful in the treatment of cutaneous lupus erythematosus in children and adults. Sunscreens do not block all wavelengths of light; therefore, consider the preparation and its characteristics, rather than suggesting that any sunscreen is effective. In adult studies, the wavelengths of light responsible for induction of cutaneous lupus erythematosus are within the ultraviolet (UV)-B and UV-A range. Unfortunately, many sunscreens are labeled for UV-B protection only with the sun-protection factor (SPF). Patients and parents should be advised to apply sunscreen well before exposure and to use a sunscreen with a high SPF that provides a broad-spectrum (UV-A) coverage and is water resistant. Reapplication after several hours is necessary. Encourage behavior modification of UV avoidance.
A general management plan of pregnancy in mothers with systemic lupus erythematosus includes treatment of disease flares using drugs that are effective but also safe for the fetus.[24] Such an approach may diminish or reduce the prevalence of complete heart block associated with neonatal lupus erythematosus. Corticosteroids and some immunosuppressive drugs are sometimes used, but long-term outcome data in children exposed to immunosuppressive drugs in utero is lacking.
Topical corticosteroids
Class Summary
Topical corticosteroid agents are used to control cutaneous lesions. Select a specific agent based on the treatment site and type of lesion. Facial skin is more prone to atrophy than skin of the scalp or hands; therefore, use a weaker topical corticosteroid on the face. Thick lesions may require more potent agents. In addition, treat hair-bearing areas with a lotion, gel, or foam instead of a cream or ointment.
Hydrocortisone topical (Ala-Cort, Beta-HC, Westcort)
Topical hydrocortisones are lower potency topical steroids (0.5%, 1%, 2.5%) that are useful on the face and intertriginous areas. These agents have mineralocorticoid and glucocorticoid effects that result in anti-inflammatory activity.
Triamcinolone topical (Oralone, Zyloptic, Triderm, Kenalog)
Topical triamcinolone decreases inflammation by suppressing migration of polymorphonuclear lymphocytes (PMNs) and reversing capillary permeability. This agent is a moderate-potency topical steroid available in ointment (0.1%) and cream (0.1%, 0.5%) formulations.
Clobetasol propionate (Temovate, Blobex, Olux)
Clobetasol is a superpotent topical steroid that decreases inflammation by suppressing migration of polymorphonuclear lymphocytes (PMNs) and reversing capillary permeability.
Betamethasone dipropionate (Celestone, Luxiq, Diprolene)
Betamethasone is a superpotent topical steroid that decreases inflammation by suppressing migration of polymorphonuclear lymphocytes (PMNs) and reversing capillary permeability.
Immunosuppressive agents
Class Summary
Patients with immune dysregulation and autoimmunity often benefit from immunosuppression. Immunosuppressive drugs are useful in patients with skin disease that is unresponsive to topical agents and in patients with arthritis that does not respond to nonsteroidal anti-inflammatory drugs (NSAID)s. These drugs are not needed in neonatal lupus erythematosus but may be used in children with skin or joint disease of systemic lupus erythematosus (SLE).
Hydroxychloroquine (Plaquenil)
Hydroxychloroquine inhibits chemotaxis of eosinophils and locomotion of neutrophils as well as impairs complement-dependent antigen-antibody reactions.
Hydroxychloroquine sulfate 200 mg is equivalent to 155 mg hydroxychloroquine base and 250 mg chloroquine phosphate.
Corticosteroids
Class Summary
Corticosteroids are useful in adults or children with renal, central nervous system (CNS), or severe hematologic disease associated with systemic lupus erythematosus (SLE). These agents are rarely needed in neonatal lupus erythematosus, but they may be used in patients with severe hepatitis or thrombocytopenia.
Prednisone
Prednisone may decrease inflammation by reversing increased capillary permeability and suppressing polymorphonuclear lymphocyte (PMN) activity.
Non-steroid topical immunosuppressants
Class Summary
Nonsteroidal topical immunosuppressive agents may be useful in postpubertal children with cutaneous systemic lupus erythematosus (SLE). Note that prolonged use of topical corticosteroids may produce significant skin adnexal atrophy. If needed, these agents may be used intermittently and short term as second-line therapy.
Pimecrolimus (Elidel)
Pimecrolimus is a second-line agent for short-term and intermittent treatment in patients whose conditions are unresponsive to or who are intolerant of other treatments. This agent is a topical calcineurin inhibitor derived from ascomycin, a natural substance produced by the fungus Streptomyces hygroscopicus var ascomyceticus.
Pimecrolimus penetrates inflamed epidermis to inhibit T-cell activation by blocking transcription of proinflammatory cytokine genes such as interleukin (IL)-2, interferon gamma (T helper cell type 1 [Th1]), IL-4, and IL-10 (Th2-type). This agent also blocks the catalytic function of calcineurin and prevents the release of inflammatory cytokines and mediators from mast cells in vitro after stimulation by antigen/IgE (immunoglobulin E).
Pimecrolimus selectively inhibits production and release of inflammatory cytokines from activated T cells by binding to cytosolic immunophilin receptor macrophilin-12. The resulting complex inhibits phosphatase calcineurin, thus blocking T-cell activation and cytokine release. Cutaneous atrophy was not observed in clinical trials, which is a potential advantage over topical corticosteroids.
Tacrolimus (Protopic)
Tacrolimus is a second-line agent for short-term and intermittent treatment in patients whose condition is unresponsive to or who are intolerant of other treatments. This agent is a topical calcineurin inhibitor that penetrates the inflamed epidermis to inhibit T-cell activation by blocking transcription of proinflammatory cytokine genes such as interleukin (IL)-2, interferon gamma (T helper cell type 1 [Th1]), IL-4, and IL-10 (Th2-type).
Tacrolimus blocks the catalytic function of calcineurin and prevents the release of inflammatory cytokines and mediators from mast cells in vitro after stimulation by antigen/IgE (immunoglobulin E). This agent also inhibits transcription for genes that encode IL-3, IL-4, IL-5, granulocyte-macrophage colony-stimulating factor (GM-CSF), and tumor necrosis factor (TNF)-alpha, all of which are involved in the early stages of T-cell activation. Cutaneous atrophy was not observed in clinical trials, which is a potential advantage over topical corticosteroids.
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