Prednisone (Deltasone, Meticorten, Orasone, Sterapred)

Immunosuppressant for treatment of autoimmune disorders. May decrease inflammation by reversing increased capillary permeability and suppressing PMN activity. Stabilizes lysosomal membranes and suppresses lymphocytes and antibody production.

0.05-2 mg/kg/d PO divided bid/qid; not to exceed 80 mg/d; taper as condition improves; single morning dose is safer for long-term use, but divided doses have more anti-inflammatory effect

4-5 mg/m²/d PO; alternatively, 0.05-2 mg/kg PO divided bid/qid; if used > 3 wk, taper over 2 wk as symptoms resolve

Documented hypersensitivity; viral infection, peptic ulcer disease, hepatic dysfunction, connective tissue infections, and fungal or tubercular skin infections

Coadministration with estrogens may decrease clearance; when used with digoxin, digitalis toxicity secondary to hypokalemia may increase; phenobarbital, phenytoin, and rifampin may increase metabolism of glucocorticoids (consider increasing maintenance dose); monitor for hypokalemia with coadministration of diuretics

C - Safety for use during pregnancy has not been established.

Abrupt discontinuation may cause adrenal crisis; hyperglycemia, edema, osteonecrosis, myopathy, peptic ulcer disease, hypokalemia, osteoporosis, euphoria, psychosis, myasthenia gravis, growth suppression, and infections may occur

Hydrocortisone (Solu-Cortef)

Decreases inflammation by suppressing migration of PMN leukocytes and reversing increased capillary permeability.

15-240 mg IV/IM q12h

1-5 mg/kg/d or 75-300 mg/m²/d PO divided q12-24h

Documented hypersensitivity; viral, fungal, or tubercular skin infections

Clearance may decrease with estrogens; may increase digitalis toxicity secondary to hypokalemia

C - Safety for use during pregnancy has not been established.

Caution in hyperthyroidism, osteoporosis, peptic ulcer, cirrhosis, nonspecific ulcerative colitis, diabetes, and myasthenia gravis

Ibuprofen (Ibuprin, Advil, Motrin)

DOC for mild to moderate pain. Inhibits inflammatory reactions and pain by decreasing prostaglandin synthesis.

400 mg PO q4-6h, 600 mg q6h, or 800 mg PO q8h while symptoms persist; not to exceed 3.2 g/d

6 months to 12 years: 4-10 mg/kg/dose PO tid/qid
>12 years: Administer as in adults

Documented hypersensitivity; avoid in peptic ulcer disease, recent GI bleeding or perforation, renal insufficiency, and high risk of bleeding

May decrease effects of loop diuretics with coadministration; coadministration with anticoagulants may increase PT (monitor and watch for signs of bleeding); may increase serum lithium levels and risk of methotrexate toxicity; probenecid may increase toxicity of NSAIDs

B - Usually safe but benefits must outweigh the risks.

Category D in third trimester of pregnancy; caution in congestive heart failure, hypertension, and decreased renal and hepatic function; caution in coagulation abnormalities or during anticoagulant therapy

Tetracycline (Sumycin)

Inhibits bacterial protein synthesis by binding with 30S and possibly 50S ribosomal subunit(s). Has anti-inflammatory activity.

Dissolve the contents of a 250-mg cap in 5 mL water or flavoured syrup and hold solution in mouth for 2 min before swallowing; repeat qid

<8 years: Not recommended
>8 years: 25-50 mg/kg/d (10-20 mg/lb) PO qid

Documented hypersensitivity; severe hepatic dysfunction

Bioavailability decreases with antacids containing aluminum, calcium, magnesium, iron, or bismuth subsalicylate; can decrease effects of oral contraceptives, causing breakthrough bleeding and increased risk of pregnancy; can increase hypoprothrombinemic effects of anticoagulants

D - Unsafe in pregnancy

Photosensitivity may occur with prolonged exposure to sunlight or tanning equipment; reduce dose in renal impairment; consider drug serum level determinations in prolonged therapy; use during tooth development (last half of pregnancy through age 8 y) can cause permanent discoloration of teeth; Fanconilike syndrome may occur with outdated tetracyclines

Amlexanox (Aphthasol)

Paste at 5% concentration. Topical mucosal anti-inflammatory agent. Exact mechanism of action remains unknown. Begin treatment as soon as patient notices symptoms.

Apply topically to each mouth ulcer qid

Not established

Documented hypersensitivity

None reported

C - Safety for use during pregnancy has not been established.

No serious reactions have been reported; less serious adverse effects of burning, transient pain, and contact dermatitis/mucositis have been reported

Sucralfate (Carafate, Sulcrate)

Forms viscous adhesive substance that protects GI lining against pepsin, peptic acid, and bile salts. Use for short-term management of ulcers.

1 g PO qid

Not established; suggested dose, 40-80 mg/kg/d PO divided q6h

Documented hypersensitivity

May decrease effects of ketoconazole, ciprofloxacin, tetracycline, phenytoin, warfarin, quinidine, theophylline, and norfloxacin

B - Usually safe but benefits must outweigh the risks.

Caution in renal failure and conditions that impair excretion of absorbed aluminum

Azathioprine (Imuran)

Antagonizes purine metabolism and inhibits synthesis of DNA, RNA, and proteins. May decrease proliferation of immune cells, which results in lower autoimmune activity.

1 mg/kg/d PO for 6-8 wk; increase by 0.5 mg/kg q4wk until response or dose reaches 2.5 mg/kg/d

Initial dose: 2-5 mg/kg/d PO/IV
Maintenance dose: 1-2 mg/kg/d PO/IV

Documented hypersensitivity, deficiency of thiopurine methyltransferase

Toxicity increases with allopurinol; concurrent use with ACE inhibitors may induce severe leukopenia; may increase levels of methotrexate metabolites and decrease effects of anticoagulants, neuromuscular blockers, and cyclosporine

D - Unsafe in pregnancy

Increases risk of neoplasia; caution in liver disease and renal impairment; hematologic toxicities may occur; monitor liver and hematologic laboratory values periodically; evaluate thiopurine methyltransferase levels prior to initiating therapy

Chlorambucil (Leukeran)

Alkylates and cross-links strands of DNA, inhibiting DNA replication and RNA transcription.

0.1-0.2 mg/kg/d PO or 3-6 mg/m²/d PO for 3-6 wk; adjust dose depending on blood counts

0.1-0.2 mg/kg/d PO for 5-15 wk

Documented hypersensitivity; previous resistance to this medication

None reported

D - Unsafe in pregnancy

Caution in seizure disorders and bone marrow suppression

Tacrolimus (Prograf, Tacrine, FK506)

Suppresses humoral immunity (T lymphocyte) activity.

0.05 mg/kg/d IV or 0.15-0.3 mg/kg/d PO divided bid

0.1 mg/kg/d IV or 0.3 mg/kg/d PO

Documented hypersensitivity

Levels may increase with diltiazem, nicardipine, clotrimazole, verapamil, erythromycin, ketoconazole, itraconazole, fluconazole, bromocriptine, grapefruit juice, metoclopramide, methylprednisolone, danazol, cyclosporine, cimetidine, or clarithromycin; levels may decrease with rifabutin, rifampin, phenobarbital, phenytoin, or carbamazepine

B - Usually safe but benefits must outweigh the risks.

Do not administer simultaneously with cyclosporine; tonic clonic seizures may occur

Cyclosporine (Sandimmune, Neoral)

Cyclic polypeptide that suppresses some humoral immunity and, to a greater extent, cell-mediated immune reactions such as delayed hypersensitivity, allograft rejection, experimental allergic encephalomyelitis, and graft versus host disease for a variety of organs. For children and adults, base dosing on ideal body weight.

2-10 mg/kg/d IV divided q8-12h

Administer as in adults

Documented hypersensitivity; uncontrolled hypertension or malignancies; do not administer concomitantly with PUVA or UVB radiation in psoriasis because may increase risk of cancer

Carbamazepine, phenytoin, isoniazid, rifampin, and phenobarbital may decrease concentrations; azithromycin, itraconazole, nicardipine, ketoconazole, fluconazole, erythromycin, verapamil, grapefruit juice, diltiazem, aminoglycosides, acyclovir, amphotericin B, and clarithromycin may increase toxicity; acute renal failure, rhabdomyolysis, myositis, and myalgias increase when taken concurrently with lovastatin

C - Safety for use during pregnancy has not been established.
 

Evaluate renal and liver function often by measuring BUN, serum creatinine, serum bilirubin, and liver enzymes; may increase risk of infection and lymphoma; reserve IV use only for those who cannot take PO; monitor blood pressure

Thalidomide (Thalomid)

Immunomodulatory agent that may suppress excessive production of TNF-alpha and may down-regulate selected cell-surface adhesion molecules involved in leukocyte migration.

100-300 mg/d PO qd with water, preferably hs and at least 1 h pc
<50 kg (110 lb): Start at low end of dose regimen

Not established

Documented hypersensitivity

May increase sedation of alcohol, barbiturates, chlorpromazine, and reserpine; due to teratogenic effects, women must use 2 additional methods of contraception or abstain from intercourse

X - Contraindicated in pregnancy

Perform pregnancy test within 24-h period prior to initiating therapy (weekly during the first month, followed by monthly tests with regular menstrual cycles or q2wk with irregular menstrual cycles); bradycardia may occur; use protective measures (eg, sunscreens, protective clothing) against exposure to sunlight or UV light (eg, tanning beds); prescribing physician must enter STEPS program established by manufacturer

Infliximab (Remicade)

Neutralizes cytokine TNF-alpha and inhibits its binding to TNF-alpha receptor. Mix in 250 mL NS for infusion over 2 h. Must use with low protein-binding filter (>1.2 µm) Has been used off label for treating BD.

5-10 mg/kg IV infusion at 0, 2, and 6 wk as induction regimen; then, 5 mg/kg q8wk for maintenance IV infusion must be administered over at least 2 h; must use infusion set with inline, sterile, nonpyrogenic, low protein-binding filter (>1.2 µm); regimen approved for psoriatic arthritis; case reports describe slightly different intervals; one reported administration day 1, day 30, and then every 8 wk for total of 20 mo and 16 mo of continuous treatment in 2 different patients

Not available

Documented hypersensitivity

None reported

B - Usually safe but benefits must outweigh the risks.
 

TNF-alpha modulates cellular immune responses; anti-TNF therapies (eg, infliximab) may adversely affect normal immune responses and allow development of superinfections; more cases of lymphoma were observed in TNF alpha-blockers compared with control groups; may increase risk of reactivation of tuberculosis in patients with particular granulomatous infections; may worsen congestive heart failure; demyelination has been reported with TNF-alpha inhibitors

Etanercept (Enbrel)

Soluble p75 TNF receptor fusion protein (sTNFR-Ig). Inhibits TNF binding to cell surface receptors, which, in turn, decreases inflammatory and immune responses. Off label use for BD.

25 mg SC 2 times/wk or 50 mg SC 2 times/wk for 3 mo; then maintenance dose of 50 mg/wk (administration is for psoriatic arthritis)

0.8 mg/kg SC; maximum single dose 25 mg

Documented hypersensitivity, sepsis, concurrent live vaccination.

None reported

B - Usually safe but benefits must outweigh the risks.

Serious infections may develop, in which case therapy should be discontinued; possible adverse effects include injection site pain, redness, swelling, and headaches; rare cases of lupuslike symptoms and heart failure have been reported (discontinue treatment if symptoms develop); in some clinical trials, increased incidence of lymphomas, other malignancies, and demyelinating disorders has been reported