The clinical spectrum of sarcoidosis is protean, but pulmonary manifestations typically dominate. Abnormalities are present on chest radiographs in 90% to 95% of patients.132,158,164,182,196 Cough or dyspnea may be prominent in patients with significant endobronchial or pulmonary parenchymal involvement.175 Fatigue and malaise are often concomitant features.113,182,196 Thirty percent to 60% of patients with sarcoidosis are asymptomatic, however, with incidental findings on chest radiographs.132,149,164,181,182,196 The clinical course and prognosis of sarcoidosis are variable. Spontaneous remissions occur in nearly two thirds of patients but the course is chronic in 10% to 30%.69,71,132,158,164,182,196 Chronic pulmonary sarcoidosis may persist for years, resulting in inexorable loss of lung function and destruction of the alveolar architecture.71,132,158,164,182,196 In that context, chest radiographs demonstrate severe fibrotic and cystic changes, volume loss, distortion, and end-stage honeycomb lung (Fig. 1). Fatalities ascribed to sarcoidosis occur in 1% to 6% of patients.56,69,71,75,113,132,144,158,164,174,175,182,196 A retrospective review from the British Isles analyzed the course and prognosis of 818 patients with sarcoidosis (both treated and untreated).132 Forty-eight patients (5%) died, usually because of chronic respiratory failure or cor pulmonale. Chest radiographs were abnormal in 700 patients (86%) at presentation but eventually resolved (with or without treatment) in 52%.132 Remissions were achieved in 2 years in more than 80% of patients with erythema nodosum or acute arthritis, but in fewer than 20% of patients with lupus pernio (disfiguring cutaneous sarcoidosis), osseous involvement, or nephrocalcinosis. Others have confirmed a more favorable prognosis when acute inflammatory manifestations are present (e.g., erythema nodosum, polyarthritis, fever, and bilateral lymphadenopathy).11,113,132,149,174,175 Poor prognostic factors include black race, lupus pernio, and chronic involvement of bone, lungs, or nasopharynx.11,79,132,174,175 Swedish investigators followed 505 patients with sarcoidosis for up to 15 years.71 Thirty patients (6%) died but only four deaths were directly attributed to sarcoidosis (less than 1% mortality rate). A review of 210 sarcoid patients in Denmark followed for 1 to 10 years noted an excellent prognosis.158 At presentation, nearly half the patients were asymptomatic; 116 (55%) had stage I radiographs; only 10 had stage III disease. At long-term follow-up, chest radiographs had worsened in only 13%. The favorable prognosis reflects inclusion of few patients with unfavorable prognostic factors or advanced radiographic disease. Early studies in the United States181 and the British Isles164 alluded to major differences in prognosis according to the radiographic stage (discussed later). In both series, stage I sarcoidosis was never fatal and the disease usually remitted spontaneously. By contrast, morbidity and mortality were significant in patients with chronic parenchymal infiltrates (radiographic stages II, III, or IV).164,181 A study of 775 cases of sarcoidosis in Japan noted that 86% were asymptomatic at long-term follow-up; fewer than 1% died as a direct result of sarcoidosis.214 Huang75 reported a 2.8% mortality rate among 1090 sarcoid patients in Europe. In the United States,56 87% of deaths attributed to sarcoidosis were secondary to pulmonary complications. In Japan,56 myocardial sarcoid accounted for 77% of deaths. Given the widely disparate prognosis of pulmonary sarcoidosis, management is controversial. Corticosteroids or immunosuppressive agents have been used to ablate the inflammatory (granulomatous) component,11,174,175 but toxicities may be considerable.112 Indications for treatment and therapeutic options are discussed subsequently.

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HfdSelect -h $EXPLOR_AREA/Data/Main/Exploration/biblio.hfd -nk 002874 | SxmlIndent | more

{{Explor lien
   |wiki=    Sante
   |area=    ChloroquineV1
   |flux=    Main
   |étape=   Exploration
   |type=    RBID
   |clé=     ISTEX:0D074FAF6594B0B4B2F3E11577830102918F3890
   |texte=   PULMONARY SARCOIDOSIS
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