Pediatric Anti-GBM Disease (Goodpasture Syndrome) Medication

Updated: Mar 05, 2015
  • Author: Rudolph P Valentini, MD; Chief Editor: Michael R Bye, MD  more...
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Medication

Medication Summary

Medications used to treat anti–glomerular basement membrane (anti-GBM) disease (Goodpasture disease) are immunosuppressive agents and prophylactic antibiotics, such as the following:

  • Medications include prednisone, cyclophosphamide, and trimethoprim-sulfamethoxazole

  • Antihypertensives may be required if hypertension occurs

  • Calcium channel blockers (eg, extended-release nifedipine) and diuretics are useful

  • Angiotensin-converting enzyme inhibitors should be used with caution, because these agents may increase serum potassium levels and decrease renal function

  • Avoid beta-blockers as well in light of the patient's pulmonary disease

  • Clindamycin lotion can be used topically to treat steroid-induced acne

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Corticosteroids

Class Summary

Glucocorticoids have anti-inflammatory properties and cause profound and varied metabolic effects. These agents modify the body's immune response to diverse stimuli.

Methylprednisolone (Solu-Medrol, A-Methapred, Depo-Medrol, Medrol)

Methylprednisolone is used to treat pulmonary hemorrhage and rapidly progressive glomerulonephritis (RPGN). This agent decreases anti-GBM antibody production as well as decreases inflammation by suppressing migration of polymorphonuclear leukocytes (PMNs) and reversing increased capillary permeability.

Prednisone

Prednisone is initially used after pulse methylprednisolone treatment is completed. This agent decreases anti-GBM antibody production.

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Antineoplastics, Alkylating

Class Summary

Alkylating agents bind with DNA and interfere with cell growth and differentiation.

Cyclophosphamide

Cyclophosphamide is a potent immunosuppressant used as an adjunct to corticosteroids and plasma exchange. This agent interferes with the inflammatory response by decreasing bone marrow response through the interference of DNA cross-linking and decreases anti-GBM antibody production.

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Monoclonal Antibodies

Class Summary

Monoclonal antibodies are used to bind to specific antigens, thereby stimulating the immune system to target these antigens.

Rituximab (Rituxan)

Rituximab is a genetically engineered, chimeric, murine/human monoclonal antibody directed against the CD20 antigen found on the surface of normal and malignant B lymphocytes. Thus, rituximab (anti-CD-20) monoclonal antibody binds to pre-B cells and mature B cells. This results in lymphocytotoxic effects to B cells, which should result in reduced autoantibody production.

Rituximab is an immunoglobulin G type 1 (IgG1) kappa immunoglobulin containing murine light-chain and heavy-chain variable region sequences and human constant region sequences. There have been isolated reports of rituximab being used for Goodpasture disease/anti-GBM disease in adults with disease refractory to corticosteroids, alkylating agents, and plasmapheresis.

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Antibiotics

Class Summary

Antibiotics are used to prevent opportunistic infection with Pneumocystis carinii.

Trimethoprim and sulfamethoxazole (Bactrim DS, Septra DS)

Trimethoprim and sulfamethoxazole prevents or reduces incidence of P carinii pneumonia in immunosuppressed patients.

Clinamycin (Cleocin, Clindagel, Clindamax)

Clindamycin lotion can be used topically to treat steroid-induced acne. Treats serious skin and soft tissue staphylococcal infections. Also effective against aerobic and anaerobic streptococci (except enterococci). Inhibits bacterial growth, possibly by blocking dissociation of peptidyl tRNA from ribosomes, causing RNA-dependent protein synthesis to arrest. Commonly used topically, but can be given orally. Apply thin film twice daily except for the gel form. Apply gel form once daily.

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Calcium Channel Blockers

Class Summary

The treatment of hypertension should be designed to reduce the blood pressure and other risk factors of coronary heart disease. Pharmacologic therapy should be individualized based on a patient’s age, race, known pathophysiologic variables, and concurrent conditions.

Nifedipine (Adalat, Procardia, Afeditab CR, Nifediac CC, Nifedical XL)

Nifedipine reduces systemic vascular resistance through relaxation of vascular smooth muscle, thereby reducing systemic blood pressure.

Amlodipine (Norvasc)

Amlodipine is generally regarded as a dihydropyridine, although experimental evidence suggests that it also may bind to nondihydropyridine binding sites. Amlodipine blocks the postexcitation release of calcium ions into cardiac and vascular smooth muscle, thereby inhibiting the action of adenosine triphosphatase (ATPase) on myofibril contraction.

The overall effect is reduced intracellular calcium levels in cardiac and smooth-muscle cells of the coronary and peripheral vasculature, resulting in dilatation of coronary and peripheral arteries. Amlodipine also increases myocardial oxygen delivery in patients with vasospastic angina, and it may potentiate ACE inhibitor effects.

During depolarization, amlodipine inhibits calcium ions from entering slow channels and voltage-sensitive areas of vascular smooth muscle and myocardium. It benefits nonpregnant patients with systolic dysfunction, hypertension, or arrhythmias. It has a substantially longer half-life than nifedipine and diltiazem and is administered once daily.

Isradipine (DynaCirc)

Isradipine is a dihydropyridine calcium-channel blocker. It inhibits calcium from entering select voltage-sensitive areas of vascular smooth muscle and myocardium during depolarization. This causes relaxation of coronary vascular smooth muscle, which results in coronary vasodilation. Vasodilation reduces systemic resistance and blood pressure, with a small increase in resting heart rate. Isradipine also has negative inotropic effects.

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Diuretics, Thiazide

Class Summary

Thiazide diuretics inhibit the reabsorption of sodium in the distal tubules, increasing the excretion of sodium, water, and potassium and hydrogen ions. They have been effective in treating hypertension of various etiologies. Besides diminishing sodium reabsorption, they also appear to diminish the sensitivity of blood vessels to circulating vasopressor substances. In all patients treated with diuretics, electrolyte levels should be monitored. Examples of thiazide diuretics are hydrochlorothiazide and chlorthalidone.

Hydrochlorothiazide (Microzide)

Hydrochlorothiazide inhibits reabsorption of sodium in distal tubules, causing increased excretion of sodium and water as well as of potassium and hydrogen ions.

Chlorthalidone (Thalitone)

Chlorthalidone inhibits reabsorption of sodium in distal tubules, causing increased excretion of sodium and water as well as of potassium and hydrogen ions.

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Diuretics, Loop

Class Summary

Loop diuretics inhibit the reabsorption of sodium chloride in the thick ascending limb of the loop of Henle. They can be used to treat hypertension in patients with renal insufficiency; they are less effective than thiazide diuretics in patients who are hypertensive with normal renal function. Examples of loop diuretics are furosemide and bumetanide.

Furosemide (Lasix)

Furosemide is a loop diuretic that increases the excretion of water by interfering with the chloride-binding cotransport system, which, in turn, inhibits sodium and chloride reabsorption in the ascending loop of Henle and the distal renal tubule. It increases renal blood flow without increasing the filtration rate. The onset of action is generally within 1 hour. Furosemide increases potassium, sodium, calcium, and magnesium excretion.

The dose must be individualized to the patient. Depending on the response, administer furosemide at increments of 20-40 mg, no sooner than 6-8 hours after the previous dose, until the desired diuresis occurs. When treating infants, titrate with 1 mg/kg/dose increments until a satisfactory effect is achieved.

Diuretics have major clinical uses in managing disorders involving abnormal fluid retention (edema) and in treating hypertension; their diuretic action causes decreased blood volume.

Bumetanide (Bumex)

Bumetanide increases the excretion of water by interfering with the chloride-binding cotransport system, which, in turn, inhibits sodium, potassium, and chloride reabsorption in the ascending loop of Henle. These effects increase the urinary excretion of sodium, chloride, and water, resulting in profound diuresis. Renal vasodilation occurs after administration, renal vascular resistance decreases, and renal blood flow is enhanced. In terms of effect, 1 mg of bumetanide is equivalent to approximately 40 mg of furosemide.

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