eMedicine Specialties > Emergency Medicine > Rheumatology
Sarcoidosis: Treatment & Medication
Updated: Jul 28, 2009
- Overview
- Differential Diagnoses & Workup
- Treatment & Medication
- Follow-up
- Multimedia
Treatment
Prehospital Care
Focus prehospital care on immediate or potential life threats. Initial assessment of the patient always begins with attention to the ABCs. Focus on respiratory support and ensuring adequacy of oxygenation because patients generally present with respiratory complaints.
- Provide supplemental oxygen to all patients. Patients with signs of impending respiratory failure should have ventilatory assistance with bag-valve-mask and high-flow oxygen. Consider intubation if a patient's condition continues to deteriorate. Perform continuous pulse oximetry if available.
- Obtain intravenous access for all patients except those who have minimal complaints. Provide judicious fluid boluses to patients with evidence of dehydration.
- Monitor cardiac function of all patients with a history of sarcoidosis because the potential for cardiac involvement exists.
- Because many patients with sarcoidosis are treated with steroids, consider the possibility of altered glucose metabolism and perform blood glucose determinations.
Emergency Department Care
Perform a primary evaluation of the ABCs. Because pulmonary complaints predominate, pay attention to respiratory effort, monitor oxygenation, and evaluate for evidence of respiratory failure.
Provide supplemental oxygen for all patients. Patients with impending or obvious respiratory failure should have ventilatory assistance with bag-valve-mask and high-flow oxygen. Patients with respiratory failure should be intubated and placed on ventilator control.
Perform pulse oximetry tests. Hypoxemic patients and those with evidence of respiratory failure should have arterial blood gas determination. With the possibility of cardiac involvement, cardiac monitoring is generally indicated. Consider intravenous access and administration of fluid boluses to patients with evidence of volume depletion.
Because patients with sarcoidosis may have taken long courses of steroids, consider alteration in glucose metabolism and the possibility of secondary adrenal insufficiency. Blood glucose determination, urinalysis, and serum chemistries may be indicated.
Sarcoidosis generally is treated with steroids, but this is not endorsed by all physicians. The dosage and duration of treatment varies among institutions and studies because the prognosis is difficult to determine with the course of the disease varying from one individual to another; however, a consensus exists among most physicians to treat symptomatic patients and patients who show signs of deterioration from baseline. These guidelines are discussed below and are arbitrarily classified into pulmonary and extrapulmonary.
- Pulmonary
- Stage I-III: If the patient is asymptomatic and PFT results are normal, the patient is monitored for a few weeks with radiography and PFTs. If symptoms worsen, steroids are begun.
- Patients in stage IV are symptomatic and treated with steroids. Even though stage IV stands for pulmonary fibrosis, patients taking steroids show improvement probably because the steroids act on ongoing inflammation.
- Other agents, mainly cytotoxic medications, also have been used in the treatment of sarcoidosis; however, success has been variable.
- Consider a lung transplant if the disease does not respond to standard therapy or the disease is in the end stage.
- Pulmonary symptoms may vary from dyspnea on exertion to severe respiratory failure. Patients require supplemental oxygen, pulse oximetry, ABGs, chest radiography, and intubation when indicated. Note that endotracheal disease is present in 40% of cases in stage I and in 70% of stage II and III. Nevertheless, significant stenosis is uncommon and intubation should not be a problem.
- Review of the patient's medication is mandatory. If the patient is not taking any medications or if the patient's medication dosage was recently changed, starting or restarting the patient on medications is reasonable.
- In most cases, when symptoms are severe, 60 mg of prednisone daily is prescribed. In moderate cases, 40-60 mg of prednisone every other day is sufficient and tapered by 5 mg per month after 6 months. Many patients are treated with a maintenance dose of 20 mg.
- Ruling out other pulmonary pathologies, such as pneumonia (eg, bacterial, fungal, Pneumocystis carinii pneumonia [PCP]), CHF, or pulmonary hypertension, is mandatory.
- Extrapulmonary
- The heart can manifest with mechanical and/or conduction defects. Standard treatment is indicated. Holter monitoring is indicated in the outpatient setting. CNS or peripheral neuropathy is treated with systemic steroids.
- Patients may present with CHF (diuretics and/or inotropics are the mainstays of therapy) or arrhythmias (eg, bundle-branch blocks, atrioventricular [AV] blocks). ECG, chest radiography, and telemetry are indicated. Cardiac manifestations are treated in the usual fashion with diuretics and inotropics, as indicated. Recently, steroids and other immunosuppressive treatments showed an improvement in 87% of patients and a cure in 54% of patients from a clinical and laboratory point of view.
- The eye is commonly involved in sarcoidosis. Topical steroids may be sufficient. Ocular manifestations can present with uveitis or conjunctivitis. A slit lamp examination and/or ophthalmologic consultation is mandatory. Treatment is with local or systemic steroids.
- CNS manifestations may present with cranial nerve symptoms (eg, VII, IX, X), with signs of optic chiasm involvement (eg, papilledema, blurred vision), meningeal symptoms caused by aseptic meningitis (requires a lumbar puncture [LP] for confirmation), symptoms of stroke/transient ischemic attack, decreased DTR, and paresthesias (caused by peripheral neuropathies).
- CT scan of the head, MRI, and neurologic consultation are appropriate. Once it is established that neurologic symptoms are secondary to sarcoidosis, systemic steroids are started.
- Hypercalcemia is generally responsive to intravenous hydration. If this is not sufficient or ECG manifestations are present, systemic steroids are indicated. In the outpatient setting, a decrease in sunlight exposure and sometimes ketoconazole (decreases 1,25-dihydroxyvitamin D) are helpful in minimizing hypercalcemia and hypercalciuria.
- Skin manifestations are treated with topical or systemic steroids. Methotrexate and retinoids are used occasionally. A dermatologic consultation is suggested.
- Arthritis generally is treated with nonsteroidal anti-inflammatory drugs (NSAIDs). Steroids and occasionally colchicine are reserved for severe cases.
- Asymptomatic elevation of LFTs does not require treatment.
Consultations
Consider consultations with other services, such as pulmonology, neurology, dermatology, ophthalmology, GI, and rheumatology, for all patients, except those with minimal findings. Patients considered for discharge should meet with a specialist in managing sarcoidosis for appropriate follow-up treatment.
Medication
The goal of treatment is to minimize the inflammatory process. Generally, all medications are started when the patient is symptomatic or worsening. Two major categories in the treatment of this disease are steroids and cytotoxics.
Glucocorticoids
These agents minimize the activity of inflammatory cells and the formation of granulomas. They are used in symptomatic patients, and they commonly provide symptomatic improvement. Glucocorticoids administered through inhalation or intravenous infusions do not show significant benefit over oral dosage forms.
Prednisone (Deltasone, Orasone, Sterapred)
Used in the treatment of various allergic and inflammatory diseases. Decreases inflammation by reversing increased capillary permeability and suppressing PMN activity. Treatment should be followed with radiography and PFTs before deciding when to taper the dose. Some patients are treated with long-term steroid therapy.
When a satisfactory response is attained, taper by 5 mg/mo until a dose of 20 mg qd or qod is achieved.
Adult
0.05-2 mg/kg/d PO divided bid/qid for 4 wk
Pediatric
4-5 mg/m2/d PO; alternatively, 1-2 mg/kg PO qd; taper as in adults
Coadministration with estrogens may decrease clearance; concurrent use with digoxin may cause digitalis toxicity secondary to hypokalemia; phenobarbital, phenytoin, and rifampin may increase metabolism of glucocorticoids (consider increasing maintenance dose); monitor for hypokalemia with coadministration of diuretics
Documented hypersensitivity; viral, fungal, tubercular skin, or connective tissue infections; peptic ulcer disease; hepatic dysfunction; GI disease
Pregnancy
B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals
Precautions
Abrupt discontinuation of glucocorticoids may cause adrenal crisis; hyperglycemia, edema, osteonecrosis, myopathy, peptic ulcer disease, hypokalemia, osteoporosis, euphoria, psychosis, myasthenia gravis, growth suppression, and infections may occur with glucocorticoid use
Cytotoxic agents
Antineoplastic (cell cycle and phase specific) and may inhibit T-cell production, which is how these agents are effective in sarcoidosis.
Methotrexate (Folex, Rheumatrex)
Antimetabolite that inhibits DNA synthesis and cell reproduction in malignant cells and may suppress the immune system.
Adult
10 mg PO qwk for 30 mo
Pediatric
5-15 mg/m2/wk PO/IM as single dose or as 3 divided doses given 12 h apart
Oral aminoglycosides may decrease absorption and blood levels of concurrent oral methotrexate (MTX); charcoal lowers levels; coadministration with etretinate may increase hepatotoxicity; folic acid or its derivatives contained in some vitamins may decrease response to MTX; coadministration with NSAIDs may be fatal; indomethacin and phenylbutazone can increase plasma levels; may decrease phenytoin serum levels; probenecid, salicylates, procarbazine, and sulfonamides, including TMP-SMZ, may increase effects and toxicity; may increase plasma levels of thiopurines
Documented hypersensitivity; alcoholism; hepatic insufficiency; documented immunodeficiency syndromes; preexisting blood dyscrasias (eg, bone marrow hypoplasia, leukopenia, thrombocytopenia, significant anemia)
Pregnancy
X - Contraindicated; benefit does not outweigh risk
Precautions
Monitor CBCs monthly, and liver and renal function q1-3mo during therapy (monitor more frequently during initial dosing, dose adjustments, or when risk of elevated MTX levels [eg, dehydration] exists); has toxic effects on hematologic, renal, GI, pulmonary, and neurologic systems; discontinue if significant drop in blood counts; aspirin, NSAIDs, or low-dose steroids may be administered concomitantly with MTX (possibility of increased toxicity with NSAIDs, including salicylates, has not been tested)
More on Sarcoidosis |
| Overview: Sarcoidosis |
| Differential Diagnoses & Workup: Sarcoidosis |
 Treatment & Medication: Sarcoidosis |
| Follow-up: Sarcoidosis |
| Multimedia: Sarcoidosis |
| References |
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References
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Further Reading
Keywords
sarcoidosis, multiorgan disease, granulomatous disease, granulomas, noncaseating granulomas, lung disease, erythema nodosum, Kveim-Stilzbach test, bronchoalveolar lavage, pulmonary function test, PFT, tuberculosis, pneumonia, hypercalcemia, immunoglobulin M, IgM, hyperglobulinemia
