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Sarcoidosis is a multisystemic disorder of unknown etiology that most commonly affects adults between 20 and 40 years of age. Patients with sarcoidosis frequently present with bilateral hilar lymphadenopathy and pulmonary infiltration, and often with ocular and skin lesions. The diagnosis is established when clinical and radiographic findings are supported by histologic evidence of non-caseating epithelioid cell granulomas found on tissue biopsy. Diagnosis of sarcoidosis requires exclusion of other causes of granuloma formation. Sarcoidosis is also characterized by sarcoid

Sarcoidosis is a multisystemic disease of unknown etiology that may affect any organ or system in the body. It most commonly affects adults of both sexes between the ages of 20 and 40, but it may occur at any age. Two thirds of patients with sarcoidosis are less than 40 years old at the time of diagnosis. The prevalence of sarcoidosis is slightly higher in females than in males, but these rates vary widely in different countries and populations. Sarcoidosis was formerly thought to be 10 times more

In the United States, the incidence of sarcoidosis has been reported to be from one in 2,500 to one in 10,000. It is a very common disorder in the black population of the Caribbean region, particularly when migration to Europe occurs. However, throughout the world, 79 percent of patients with sarcoidosis are white. Sarcoidosis occurs frequently in the white population of Scandinavia; in Sweden, the incidence of sarcoidosis is 64 per 100,000 persons. It is also frequently diagnosed in, sarcodosis, sercoidosis

It has been hypothesized that sarcoidosis is the result of various antigens causing T-cell activation in a genetically predisposed host. The increased number of "helper" T cells attracts monocytes, and granuloma formation occurs. B-cell activation also

Sarcoidosis with pulmonary involvement is primarily a restrictive lung disease with abnormal gas exchange but may occasionally

Clinical Manifestations

Symptoms of sarcoidosis may be caused by a number of factors, including the "mass effect" of the granuloma(s); immune complex vasculitis (as occurs in erythema nodosum); metabolically active granulomas; and fibrotic distortion lasting even

The lungs are the primary target of this disease. About 88 percent of patients with sar-coidosis have lung involvement. It is customary to stage intrathoracic sarcoidosis by comparing current chest radiographs with the chest radiograph taken on initial presentation. Intrathoracic sarcoidosis is divided into four stages. Approximately 8 percent of patients with sarcoidosis present at

Twenty-nine percent of patients with sarcoidosis present at stage 2. During this stage, chest radiographs show hilar lymphadenopathy associated with diffuse pulmonary infiltration (Figure 4). The signs and symptoms are usually mild in relation to the severity of the abnormalities shown on radiograph. Multiple pulmonary nodules or infiltrates may also be present. Results of

The patient presenting with stage 3 sarcoidosis may have minimal symptoms, (ie, cough, dyspnea, mild weight loss) or significant problems, including pulmonary hypertension, cor pulmonale and respiratory failure. Many patients in stage 3 have intrinsic restrictive

Table 1. Stages of Sarcoidosis


Patients presenting at this stage (%)

Findings on chest radiograph

Results of pulmonary function testing

Signs and symptoms

Patients expected to go into remission (%)


8 to 10

Normal (but with

multisystem involvement)


Varies with

system affected

Most remit




Bilateral hilar

lymphadenopathy with or without enlarged right paratracheal nodes

Normal, except

for decreased diffusing capacity; normal mechanics


asymptomatic or with nonpulmonary complaints

70 to 75% remit

within two years; 10 to 15% progress to stage 2



Hilar lymphadenopathy

with diffuse pulmonary infiltration; pulmonary nodules may be seen

Usually restrictive

changes with decreased diffusing capacity; obstructive changes may be present

Usually mild in

relation to the severity of the radiographic findings


spontaneously remit; 25 to 30% persist at stage 2 or progress to stage 3



Diffuse pulmonary

infiltration, but without hilar lymphadenopathy; fibrosis; small lung volumes; elevated diaphragms effusions; calcifications; "honeycombing"


restrictive changes, but with obstructive changes due to bronchial involvement; changes may be severe

Varies: may be

minimal (cough, dyspnea, weight loss) to severe (cor pulmonale, pulmonary hypertension; may progress to respiratory failure)


spontaneously remit within two years


A large number of patients (40 to 50 percent) with chronic sarcoidosis have extrapulmonary involvement that


The diagnosis of sarcoidosis requires three components: the presence of clinical and radiographic findings consistent with a diagnosis of sarcoidosis; non-caseating (non-necrotizing) granulomas found


The goals of treatment for sarcoidosis include resolving inflammatory lesions that are interfering with organ function, preventing pulmonary fibrosis and diminishing symptoms. If the patient presents with

Corticosteroids continue to be the mainstay of therapy, although they have not been proved to prolong life. Several different protocols exist. To induce disease regression, treatment with prednisone may

Inhaled steroids have been used in patients with sarcoidosis for relief of symptoms, but it has not been proved that this therapy reduces disease progression. Inhaled and oral bronchodilators, supplemental oxygen and synthetic "liquid" tears have also been used to reduce symptoms. Topical ophthalmic steroids have been used to reduce ocular manifestations of sarcoidosis. If symptoms of erythema nodosum and arthritis are present in patients with stage 2 disease, a nonsteroidal anti-inflammatory drug such as indomethacin (Indocin), in a dosage of 25 mg three times daily, may be used.

Newer therapies have been reported. Hydroxychloroquine (Plaquenil), given in a dosage of 200 mg every other day for nine months, may be useful in the treatment of cutaneous sarcoidosis but can

Hydroxychloroquine has also been found to be helpful in the management of hypercalcemia Methotrexate (Rheumatrex), in a low dosage of 7.5 to 15 mg once per week, has 

Other treatments are available, but few controlled trials have been performed: chlorambucil (Leukeran), cyclophosphamide (Cytoxan) and azathioprine (Imuran). Rarely, lung transplantation has been performed in patients with severe, refractory disease, with varying results. Several patients had a recurrence of


The prognosis of patients with sarcoidosis is generally good, with spontaneous resolution of disease being the "rule" Mortality rates vary from 5 to 8 percent. The prognosis is usually better in women, in patients with less