A 49-year-old female diagnosed with sarcoidosis benefitted from an outpatient pulmonary rehabilitation program.

J0i00924.jpg (8167 bytes)An official statement of the American Thoracic Society begins, “Sarcoidosis is a systemic granulomatous disease that primarily affects the lung and lymphatic systems of the body.”1 What does that really mean to the individual who has been diagnosed with this complicated and confusing condition? Since 1877, when it was first described as a separate diagnosis, sarcoidosis has been challenging medical practitioners, not only with its identification and diagnosis in individual patients, but also to determine its causes and the best forms of treatment for it.

Various theories have been proposed for the causes of this often insidious and debilitating condition. From work that suggested a contagion to theories of exposure to an antigen to investigations of genetics, explanations have been sought worldwide. Unfortunately, as of February 1999, when a joint statement was adopted by the American Thoracic Society, the European Respiratory Society, and the World Association of Sarcoidois and Other Granulomatous Disorders, it was concluded that “the cause of the disorder is still unknown.”1

In that statement, what was drawn from all of the research and evidence to date was, “It is likely that genetically predisposed hosts are exposed to antigens that trigger an exaggerated cellular immune response leading to granuloma formation.” Evidence supporting that idea comes from epidemiological investigation, the presence of an inflammatory response in sarcoidosis, and work concerning the T cell receptor in patients with sarcoidosis.

In essence, this means that ways must be found—in the absence of much concrete information—to minimize the physical, emotional, and social effects of a chronic and debilitating condition.

Because sarcoidosis is a multiorgan disorder, patients may present to clinicians working in many different disciplines. The presentation depends on the site and extent of organ involvement, on ethnicity, and on the duration of illness, as well as on the activity of the granulomatous process. Systems affected may include the heart, liver, kidneys, nervous system, skin, and eyes.

Nonspecific constitutional symptoms such as low-grade fever, fatigue, malaise, and weight loss may occur in about one third of patients with sarcoidosis. Fatigue, when present, can be quite disabling. Occasionally, night sweats may occur. The constitutional symptoms are more frequently seen among patients whose ancestors were from Africa or the Indian subcontinent. In the absence of a known causative agent, sarcoidosis remains a diagnosis of exclusion. There are no definitive diagnostic blood, skin, or radiological imaging tests specific for the disorder.2 According to the ATS statement, however, “the diagnosis is established when clinicoradiological findings are supported by histological evidence of noncaseating epithelioid cell granulomas.”1

In the lungs, which are affected in more than 90% of patients, sarcoidosis is an interstitial disorder involving alveoli, blood vessels, and bronchioles and producing clinical findings of dry rales, restricted lung volumes, and abnormalities of gas exchange. The airways are also susceptible to granulomatous involvement. Limitation of airflow is the most common presenting pulmonary physiologic abnormality.

Inhaled and oral corticosteroids have been used as treatment for airway involvement, with differing opinions on their effectiveness in clinical trials. Their side effects, however, can create additional complications.

Spontaneous remissions occur in nearly two thirds of patients, but the course of the disease is chronic or progressive in 10% to 30%. Fatalities occur in 1% to 5% of patients, typically owing to progressive respiratory insufficiency or to the involvement of the central nervous system or myocardium. In the United States, most deaths are due to pulmonary complications.1

Patients with sarcoidosis should be evaluated for systemic symptoms such as myalgia and fatigue. Patients with those symptoms, and those with significant respiratory insufficiency, may benefit from pulmonary rehabilitation. Hypoxemia at rest or upon exercise may call for supplemental oxygen therapy. In fact, according to pulmonary rehabilitation guidelines, “careful evaluation of the patient’s oxygen requirements as determined by arterial blood gases and/or exercise oximetry is helpful for titrating oxygen therapy during exercise as well as during activities of daily living.”3

Pulmonary rehabilitation is considered appropriate for any patient with stable disease of the respiratory system and disabling symptoms. Even patients with severe disease can benefit if they are selected appropriately and if realistic goals are set.4

The 1999 ATS statement on pulmonary rehabilitation noted, “Because pulmonary rehabilitation has traditionally dealt with patients with chronic obstructive pulmonary disease (COPD), the effectiveness of this therapy for pulmonary conditions other than COPD has received less attention.”5 It states, further, “the same principles of ameliorating secondary morbidity also apply. By necessity programs for patients without COPD may differ in educational focus and exercise prescription from traditional rehab for those with COPD. Exercise training of patients with interstitial lung disease (ILD) may require modification because of exercise induced hypoxemia.” It has been observed that “patients with ILD are younger than patients with COPD and are not aware of the course of their illness. Furthermore, lifestyle changes occur sooner for the ILD patient than for the COPD patient; therefore, the path from diagnosis to disability is often short.”3

As the 1999 ATS statement puts it, “Although anecdotal information supports its use, little scientific information is available on the effectiveness of [pulmonary rehabilitation] in disease other than COPD and asthma….Do patients with pulmonary fibrosis or chest wall disease improve with [pulmonary rehabilitation]? What special interventions are necessary for these patients?”5

In general, the principle of symptom-limited exercise may be applied to almost any population. Patients who become breathless with minimal exertion must begin with very low-level activity, such as the use of light hand weights, seated cuff weights, and hip-strengthening exercises. As muscle strength improves, other forms of light exercise can be added, closely linked to the patient’s perceived exertion. In the patient with interstitial disease, hypoxemia secondary to reduced diffusing capacity will require close monitoring, as well. Frequently, even minor exertion can produce precipitous drops in saturation. The definition of pulmonary rehabilitation stresses an individually tailored program of care; indeed, the patient with sarcoidosis will need exactly that.

Case Report
A 49-year-old African American female was hospitalized in February 1998 with acute bronchitis due to Staphylococcus aureus infection and a diagnosis of sarcoidosis with right pleural effusion secondary to lymphatic obstruction (probably chronic) from prior granulomatous lymphadenopathy. She had previously undergone diagnostic thoracoscopy, pleural biopsy, lung biopsy, and chest-tube drainage for talc sclerosis. She also had persistent hypoxemia, reactive airway disease, hypertension, insulin-dependent diabetes mellitus, urethral stricture, and hyperlipidemia.

She was hospitalized for 11 days, and her prescribed medications, upon discharge, included beclomethasone, zafirlukast, simvastatin, fluticasone, insulin, salmeterol, albuterol, ipratropium, troglitazone, amoxicillin, famotidine, prednisone, lisinopril, and tramadol. She was also discharged with a prescription for continuous supplemental oxygen delivered via nasal cannula at a flow rate of 2 L/min because resting pulse oximetry had yielded a reading of 84% while she breathed room air.

Her previous medical history included moderate to severe obstructive lung disease, and a diagnosis of sarcoidosis had been made in 1960 when she had presented with a positive tuberculin purified protein derivative test, a normal chest radiograph, and skin lesions.

Serial pulmonary function studies at 6-month intervals from 1994 to 1997 demonstrated a steady decline in forced expiratory volume in 1 second, from an initial 0.94 L (54% of the predicted vale) to 0.73 L (43% of the predicted value). Her forced vital capacity demonstrated a similar decline, from 1.22 L (62%) to 0.99 L (52%). An echocardiogram performed in 1997 demonstrated normal ventricular function without evidence of valvular disease.

A month after discharge, this patient was referred to outpatient pulmonary rehabilitation due to deconditioning and dyspnea on exertion. The patient was admitted to the program on April 6, 1998. At that time, her 6-minute walking distance was 318 m (at about 3.2 km per hour) using supplemental oxygen at a flow rate of 2 L/min. Physical findings included elevated shoulders and primary use of accessory muscles with minimal movement of the diaphragm and no rib excursion. She subjectively reported that her lung disease interfered significantly with walking more than half of a block; self-care; and basic household chores such as laundry, carrying groceries, cleaning, and meal preparation. She reported shortness of breath when moving from supine to seated positions in bed, during floor-to-stand transfers, when getting in and out of a car, and when climbing more than four steps.

She had no history of smoking or abuse of drugs or alcohol. She was married, with two teenage daughters living at home. She was employed full time as a high school counselor, and at the time of admission, she was on long-term sick leave.

During her orientation to the 8-week program, she received instruction in diaphragmatic breathing in sitting and supine positions, in exertion/exhalation coordination, and in the use of pursed-lip breathing. Since she also had a history of bronchospasm, the pursed-lip breathing was an adjunct that may not be generally useful in restrictive lung diseases.

Each week, the patient attended 1 hour of occupational therapy instruction for biofeedback, energy conservation and work simplification, and stress management/relaxation. She had some particularly stressful circumstances at home, including a chronically ill husband and conflict with her older daughter.

The program uses a symptom-limited exercise protocol and the 0-to-10 modified Borg scoring system. Patients are instructed to try to work at a level of 3 (moderate), which is not uncomfortable, and to reduce the intensity of an activity if they feel their breathlessness to be at a level of 4 (somewhat severe) or greater. Exercise sessions are normally held 3 days per week. The patient attended exercise 3 days per week during the first month; because she wanted to return to work, however, she decreased attendance to 2 days per week in the second month, with a third session to be done independently.

As part of her plan to return to work, she was able to arrange for her home care company to place an oxygen concentrator in her office. She used a portable pulsed-delivery system for times when she needed to move about the building.

During the course of the program, she was able to increase her total exercise time from 21.5 to 47 minutes. She was able to increase her treadmill walking from 7 minutes at 1.44 km per hour to 20 minutes at 3.06 km per hour.

She increased her use of the piston-style stair simulator from 4 minutes and 14 steps to 10 minutes and 60 steps. She increased her use of a rower from 4 minutes at 3.4 W to 10 minutes at 6.3 W. Her hand weights increased from 0.45 kg to 0.9 kg. She was taught arm exercises that employed a resistive training band to use as part of her home routine.

Upon completion of the program at the end of May, she had a 6-minute walking distance of 378 m (3.68 km per hour), an increase of 19% from her initial test. Her arm strength and endurance increased 186%.

She has remained relatively stable since completing the program. During follow-up care, she did report some difficulty in maintaining her exercise regimen and in following her diet for diabetes, but stated that this adherence waxed and waned with life stressors.

Carlie Ream, MA, RRT, CPFT, is coordinator of pulmonary rehabilitation, St Agnes HealthCare, Baltimore.

1. American Thoracic Society. ATS statement on sarcoidosis. Am J Respir Crit Care Med. 1999;160;736-755.
2. Newman LS, Rose CS, Maier LA. Sarcoidosis. N Engl J Med. 1997;336:1224-1234.
3. American Association of Cardiovascular and Pulmonary Rehabilitation. Guidelines for Pulmonary Rehabilitation Programs. 2nd ed. Champaign, Ill: Human Kinetics; 1998.
4. Reis AL, Carlin BW, Carrieri-Kohlman V, et al. Pulmonary rehabilitation: joint ACCP/AACVPR evidence-based guidelines. Chest. 1997;112:1363-1396.
5. American Thoracic Society. ATS statement: pulmonary rehabilitation—1999. Am J Respir Crit Care Med. 1999;159:1666-1682.