eMedicine Specialties > Emergency Medicine > Rheumatology

Sarcoidosis: Follow-up

Author: Ramy Yakobi, MD, MBA, Medical Director of Emergency Department, Beth Israel/Kings Highway Division; Lecturer, Physician Assistant School, Cornell School of Medicine; Lecturer, Pre-hospital Management of Patient, Cornell/New York Presbyterian Hospital; Director of Emergency Department, New York Community Hospital
Contributor Information and Disclosures

Updated: Jul 28, 2009

Follow-up

Further Inpatient Care

• Patients with severe pulmonary and/or extrapulmonary symptoms must be admitted to the hospital.
• Prednisone treatment is instituted as discussed above. If symptoms are refractory to those treatments listed above, if multiorgan involvement occurs because of concomitant failure, or if infection is present, placing the patient in an intensive care unit (ICU) is prudent.
• In the hospital, patients may benefit from pulmonary physical therapy (PT). This decision must be made in conjunction with the PT department. PT can achieve energy conservation, incentive spirometry, segmental breathing exercises, demonstration of positions to relieve breathlessness, and interval exercise training to improve aerobic capacity.

Further Outpatient Care

• As discussed above, once prednisone therapy is started, it is continued for 4 weeks to 6 months before tapering the dosage.
• Use the following as routine parameters to ensure a favorable outcome:
  • Obtain a chest radiograph.
  • Perform pulmonary function testing.
  • Occasionally, a gallium 67 scan is performed to assess involvement of extrapulmonary organs and to distinguish between inflammatory and fibrotic processes.
  • Serial BAL fluid studies for measurement of cytokines and CD cell ratio, although not predictive of prognosis and severity of disease, are used in certain institutions to monitor the response to therapy.
  • ACE levels (as BAL) have no prognostic value, but serial results can help to assess the therapeutic response.
  • Carefully monitor medication adverse effects with serum chemistries to measure calcium, blood urea nitrogen, and creatinine levels and with liver function tests; perform periodic slit lamp examinations.
• Outpatient physical therapy may improve the patient's quality of life.

Inpatient & Outpatient Medications

• Prednisone is an oral steroid and the mainstay of medical treatment. Base dosage on the patient's symptoms.
• When failure to steroid treatment and progression of disease is evident, other empiric treatments, such as cytotoxics (eg, methotrexate) are tried. Lung transplant is an option when the patient is refractory to medical therapy and end-stage pulmonary failure is present. Cases of new-onset sarcoidosis in transplanted lungs have been documented.

Transfer

• Consider transfer to a specialized center for patients who are not responding to standard therapy or if lung transplant is entertained.
• Before transfer, ensure that the patient has stable vital signs and a good or acceptable oxygen saturation level, ECG finding, and mental status.

Deterrence/Prevention

• Although it is intuitive that smoking may worsen pulmonary sarcoidosis, many studies indicate the contrary.
• Consider measures to protect patients from pulmonary diseases, such as influenza (with flu vaccine), bronchitis, or PCP. (Patients may benefit from low-dose prophylactic trimethoprim/sulfamethoxazole treatment if taking long-term steroid therapy.)

Complications

• Complications are organ specific and variable, as follows:
  • Pulmonary - Infections, pulmonary hypertension, pulmonary fibrosis, and death
  • Cardiac - Arrhythmias and CHF
  • Ocular - Uveitis and conjunctivitis
  • Liver - Commonly involved but rarely clinically significant
  • Lymphatic - Evidence of hypersplenism

Prognosis

• Two thirds of cases resolve spontaneously, one third are long-term, and 5% result in fatality.
• Although no markers exist to accurately predict outcome, certain traits and features can suggest a prognosis, as follows:
  
  
  • Acute presentation, erythema nodosum, and stage I radiographic manifestations are considered favorable prognostic features, and remission may occur in 2-5 years.
  • Stage III-IV, chronic iritis, lupus pernio, tracheal involvement, and extrapulmonary manifestations are associated with less favorable prognosis.
  • Genetics also may have a role. Black persons have a less favorable prognosis than white persons. Patients with HLA-B13 also have a less favorable prognosis than patients with HLA-B8. The correlation between advanced cases of sarcoidosis and glucose-6-phosphate dehydrogenase (G-6-PD) deficiency are described.³

Patient Education

• Discuss compliance with medications and medical follow-up.
• Educate about extrapulmonary complications (eg, uveitis, arrhythmias) and the importance of seeking immediate medical attention.

Miscellaneous

Medicolegal Pitfalls

• Failure to consider other entities with similar presentation is a pitfall. Tuberculosis and lymphoma are particularly important in the United States.
• Consider the possibility of other life-threatening conditions (eg, pulmonary embolism) if no evidence of sarcoidosis is documented in the ED and a patient complains of dyspnea.
• Failure to recognize or ignore complaints (eg, ocular symptoms, chest pain) is a pitfall. Ignoring these complaints may indicate that the ED physician is not aware of the complications that can occur with sarcoidosis.
• To merit transfer to another hospital, all criteria must be present. In the case of sarcoidosis, ensure that vital signs, oxygen saturation levels, ECG findings, and mental status are acceptable.
• Failure to emphasize the need for follow-up treatment is a pitfall.
• Failure to explain the complications and symptoms that require immediate medical attention is a pitfall.

The authors and editors of eMedicine gratefully acknowledge the contributions of previous author, David Cheng, MD, to the development and writing of this article.

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