Is sarcoidosis fatal, can individuals die from it?

The outcome of sarcoidosis depends upon the severity of the illness. It is not common, but in a certain percentage of situations, sarcoidosis can be fatal.

What is the outcome of sarcoidosis of the lung, can it be cured?

The chest x-ray pattern has been classification into three stages of sarcoidosis of the lung. These are not necessarily sequential stages that all patients follow, but they are used to some extent for description of disease activity.

Stage I is limited to lymph node enlargement in the hilar regions. The lungs appear normal.

Stage II shows enlarged lymph nodes in the hilar regions and small nodular opacities in the lungs.

Stage III shows no enlarged lymph nodes but shows linear shadows suggestive of scarring in the upper lungs and sometimes cystic structures. This last stage probably represents a late stage and may represent an inactive process.

Stages I and II and the most common. Stage III is less common.

Outcome varies among these three x-ray patterns. For stage I, 60% to 80% of patients are cured. For stage II the percentage decreases somewhat to 50% to 60%. For stage III, it is the lowest with 30%.

Findings that are associated with a poorer prognosis include onset over age 40 years, symptoms for more than six months, involvement of three or more organs, and stage III pulmonary disease.

Computerized chest x-rays that show a fluffy or "ground glass" appearance and nodular opacities suggest a responsive lesion. Cystic air spaces and architectural distortion of the lung tissues represent irreversible findings.

Serial pulmonary function and chest radiographs are the most helpful tests for monitoring the activity of sarcoidosis of the lung. Other specialized tests are utilized for monitoring sarcoidosis of the heart, eye, kidney or other organs.

The overall outcome for sarcoidosis is fairly good, 50% of patients with sarcoidosis will eventually have complete resolution without residual effects. Among the remaining 50%, there may be permanent organ dysfunction, but it is usually of a mild degree and only detectable by detailed testing.

How is sarcoidosis of the lung treated?

There has been a change in the approach to the management of sarcoidosis for a large group of patients. In the past, patients with the diagnosis of sarcoidosis were treated. Now, patients are categorized into two groups. One group that is treated, and another group that treatment is delayed or not given.

The current emphasis is for a period of observation for patients who do not require treatment for symptoms. Studies have shown that 40% of patients will have spontaneous improvement, 40% of patients will respond to subsequent treatment, and 20% of patients will require immediate treatment.

Corticosteroid therapy remains the standard treatment usually in the form of prednisone. Immediate therapy is usually indicated for patients with stage II and stage III lung disease who have symptoms and pulmonary function test abnormalities. Immediate therapy is also recommended for patients with severe sarcoidosis of the heart, eye, neurological system, or if there is a severely increased calcium level in the blood.

A period of observation, three months or six months, is appropriate for individuals without symptoms, without pulmonary function test abnormalities, and with milder forms of the illness. If no symptoms develop and no new disease activity is detected, continued observation is recommended. A total of two years of observation and monitoring is usually sufficient to establish resolution or an inactive state. If symptoms develop before the scheduled monitoring tests or if symptoms and test findings change at the three-month or six-month interval to a severe category, corticosteroid treatment is begun.

Current treatment protocols indicate the use of 30 to 40 mg of prednisone daily for 8 to 12 weeks, with gradual decreasing of the dose to 10 to 20 mg every other day over a period of 6 to 12 months.

In special situations where sarcoidosis involves the airways and the patient is being treated with prednisone, inhaled corticosteroid therapy may be utilized as a means of decreasing the dose of the prednisone.

Does corticosteroid treatment have side effects?

Yes, the adverse effects can be numerous, most are reversible but some are not. This medication will save a person’s life, but can also cause difficulties in some individuals.

The common adverse reactions include increased appetite, weight gain, and bruising of the skin. A rounded puffy face, acne-like skin lesions, "fat pads" below the neck in the back and in the front may develop over time.

Some psychological effects, high blood pressure, diabetes, and osteoporosis (softening of the bones) may develop. Cataracts can occur. A very rare condition known as aseptic necrosis of the hips requiring hip replacement may develop.

It is important to obtain the list of effects and review them.

Some individuals have no difficulty with the medication. Others may be bothered by some of the adverse reactions but can tolerate them or they disappear. Every once in awhile, a person cannot take the medication or develops a severe reaction.

The prednisone is given at the lowest dose that is effective for the shortness length of time as possible. The every other day dosage can decrease the side effects.

What if corticosteroid therapy is not effective?

For sarcoidosis, corticosteroid treatment has appeared to improve the radiographic findings, yet whether the medication has an ultimate effect on whether a person is going to have residual scarring has not been proven. For this reason, there has been a change in the approach to treatment as noted above. In some situations, the scarring process may progress.

In these situations if the scarring is combined with a decreased oxygen value, supplemental oxygen can be beneficial. The heart has to work harder because of the increased pressure in the lungs from the fibrosis and soon fails. Oxygen has a direct benefit for the heart. Oxygen will prevent the heart from enlarging and weakening. The heart will not fail. In order to be effective, supplemental oxygen is required for at least 18 hours per day. Generally, it is recommended that the oxygen is utilized 24 hours daily. It seems excessive at first, but individuals become use to it and are able to lead an active and zestful life.

A pulmonary rehabilitation program should also supplement the oxygen program. Exercise is very useful in improved conditioning and efficiency of the muscles from the remaining good lung. The rehabilitation program is useful because an individual can be pushed to a higher level of exertion in a protected environment. The exercise program (e.g., 30 to 45 minutes of daily walking) can be continued on a home basis after the controlled, supervised program.

For patients who are not able to take prednisone or who require high doses of prednisone, methotrexate at 10 mg per week may enable patients to decrease their prednisone use after six months. Methotrexate treatment in some patients may be related to abnormal blood tests and certain infections. It is important to review and discuss possible adverse reactions with the doctor so a person can be aware of a potential reaction early.

There have been anecdotal reports of other treatments. Some of these may appear to be effective on an individual basis, yet studies of a large number of patients are needed to confirm the success of these treatments.

In life threatening, disabling disease and progressive disease that has not responded to treatment, lung transplantation may be utilized.