Intervention
Controversy exists regarding optimal treatment. More than 75% patients with sarcoidosis require only symptomatic treatment, such as with nonsteroidal anti-inflammatory drugs.42 Around 10% of patients require treatment for extrapulmonary disease, and 15% of patients with persistent lung disease may require some form of treatment.43
Drug treatment
Corticosteroids, immunosuppressives, and miscellaneous are used to treat pulmonary sarcoidosis.
Corticosteroids
Corticosteroids remain the mainstay of treatment. Generally, prednisone is given daily and then tapered over a 6-month period. This approach is considered adequate for pulmonary disease. The dose given to the patients is debated. Some patients may require low-dose long-term steroids.
Steroids can relieve symptoms and suppress inflammation and granuloma formation. The indications for steroid treatment include involvement of the central nervous system, ocular involvement, cardiac involvement, hypercalcemia, and nephrocalcinosis. The decision to treat pulmonary sarcoidosis with steroids remains a subject of debate and is based on clinical, physiologic, and radiologic findings.
The initial response to steroid treatment does not, however, preclude the progression to pulmonary fibrosis and death. Drug therapies, such as treatment with steroids and other anti-inflammatory medications, have their own risks of morbidity and mortality, and long-term monitoring is usually required. In more than 70% of patients, the disease regresses within 2 years of the initial onset regardless of treatment. In some patients, sarcoidosis progress relentlessly, and they may require long-term medication.
Corticosteroid use may be associated with increased relapse rates. High-dose inhaled corticosteroids are an option, but conclusive evidence that this treatment is effective is lacking. Corticosteroids may be beneficial for cardiac disease, but they may result in aneurysm formation.44
Immunosuppressives
Noncorticosteroid agents are being used more frequently. These agents include methotrexate or other alternatives, such as cyclosporine, pentoxifylline, and azathioprine. These may be successful alternatives to prednisone and are steroid-sparing agents.
Miscellaneous
Hydroxychloroquine may be used for cutaneous lesions, hypercalcemia, neurologic sarcoidosis, and bone lesions. Nonsteroidal anti-inflammatory drugs are indicated for the treatment of arthralgias.
Procedure
Biopsy
Tissue diagnosis is required in most patients. Biopsy specimens can be obtained from the lungs or extrapulmonary sites. Transbronchial biopsy via fiberoptic bronchoscopy has a diagnostic yield of 85% when 4 samples are obtained. Occasionally, mediastinoscopy, thoracoscopy, or thoracotomy for node or lung biopsy may be required.
Some asymptomatic patients who do not require treatment may be monitored without a biopsy. Observation may be sufficient only for patients who have a classic presentation, are asymptomatic, and can ensure close follow-up. Results may be positive, even in the setting of normal chest radiographic findings. If treatment is to be given for sarcoidosis, tissue confirmation is essential. The classic histologic finding is the presence of noncaseating granulomas with special stains negative for fungus and mycobacteria.
Massive hemoptysis, defined as the expectoration of more than 600 mL of blood in 48 hours, may complicate a mycetoma. Surgical resection of the cavity is the treatment of choice. Contraindications to surgery include bilateral advanced lung disease, large transpleural blood vessels, failure to identify the bleeding site, and continued hemoptysis after previous surgery. Bronchial artery embolization is a valuable technique in patients with persistent hemoptysis who are not surgical candidates.
Bronchial artery embolization
Before bronchial artery embolization is attempted, angiography is performed to assess the bronchial arterial anatomy, the nonbronchial systemic blood supply to the mycetoma, and the presence and extent of any contribution of blood supply from the pulmonary artery. The technique involves selective bronchial artery catheterization with a femorovisceral 5F end-hole catheter.
After preliminary bronchial angiography is performed, the catheter is securely inserted into the bronchial artery to be embolized. A coaxial catheter may be required for more selective and more distal placement. Various embolic agents can be used; the simplest material is polyvinyl alcohol (PVA), a particulate agent that is available in a variety of sizes. Because mycetomas can derive their blood supply from the intercostal arteries, the thyrocervical trunks and pulmonary arteries may also need to be selectively embolized.
Percutaneous CT or fluoroscopically guided intracavitary injection of a paste containing glycerin and amphotericin B or other fungal agents has been successful in the treatment of aspergillomas and chronic necrotizing aspergillosis. The fungal ball partially or completely resolves in 75% of patients. The intracavitary instillation of sodium or potassium iodide has also been used successfully in the treatment of hemoptysis in patients who are not surgical candidates.
Chest pain may occur, particularly after intercostal artery embolization. Rarely, mild dysphagia may occur as a result of an interruption of blood supply to the mid esophagus. This dysphagia is self-limiting and resolves spontaneously. Transverse myelitis and bronchial necrosis are the most serious complications; fortunately, these are rare. Most case reports of transverse myelitis are historical and are probably related to contrast agent toxicity. Nontarget embolization leading to infarction is also reported. This complication is less likely to occur when a superselective approach with end-hole catheter is used.
Disease course and prognosis
The course and prognosis of sarcoidosis are correlated with the mode of disease onset, the patient's race, and the presenting stage.
An acute onset of disease, so-called Löfgren syndrome, usually occurs as a self-limiting condition that resolves spontaneously, whereas an insidious onset may be followed by relentless progressive fibrosis. The disease resolves within 1-2 years in 60% of patients presenting with stage I disease, in 46% with stage II disease, and in 12% with stage III disease.
Non-white patients tend to have more extrathoracic manifestations and, therefore, a more guarded prognosis. Overall, two thirds of African American patients with sarcoidosis require treatment, compared with one third of white patients.45
Medicolegal Pitfalls
- Lymphoma may mimic sarcoidosis and vice versa.
- Failure to diagnose sarcoidosis may lead to an unnecessary delay in treatment and increase the patient's anxiety.
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Further Reading
Keywords
Boeck sarcoid, Besnier-Boeck-Schaumann, lupus pernio, benign granulomatous sarcoid, Jungling's sarcoidosis, Jungling sarcoidosis, Mortimer's sarcoidosis, Mortimer sarcoidosis, Schaumann's sarcoidosis, Schaumann sarcoidosis, non-caseating granuloma, noncaseating granuloma
