Pulmonary sarcoidosis disease

Additional indications for GCs include certain types of shock, hepatic diseases (e.g., hepatic necrosis), renal diseases (e.g., idiopathic nephrotic syndrome), and certain respiratory disorders (e.g., pulmonary sarcoidosis). It should be understood that in many of the conditions GCs are palliative at best and that in some instances their use may be controversial. 

Nonspecific Interstitial Pneumonitis ), CTD-related DPLD (see chap. 17, Collagen Vascular Disease-Associated Pulmonary Fibrosis ), exposure-related DPLD (see chap. 10-13), and sarcoidosis (see chap. 7, Pulmonary Sarcoidosis ). 

Remy-Jardin M, Giraud F, Remy J, et al. Pulmonary sarcoidosis role of CT in the evaluation of disease activity and functional impairment and in prognosis assessment. Radiology 1994 191(3) 675-680. 

The use of MTX has been almost exclusively for sarcoidosis. It has been a useful steroid-sparing agent for chronic pulmonary disease (9,10) and exhibits steroidsparing effects in acute pulmonary sarcoidosis (35). While widely used to treat RA, there is no evidence to suggest that MTX prevents or treats RA-associated pulmonary fibrosis. 

However, in a dose-escalation trial, thalidomide was ineffective in a cohort of 12 patients with pulmonary symptoms (310). In a trial of pulmonary sarcoidosis, thalidomide exhibited limited steroid-sparing benefit and no improvement in pulmonary lung function (318). Thalidomide is extremely teratogenic and use of the agent requires careful monitoring in both male and female patients. Thalidomide causes somnolence, constipation, and peripheral neuropathy in a significant number of patients (310). These side effects are dose dependent and may explain why thalidomide is not as useful for extracutaneous disease (318). 

Hunninghake GW, Crystal RG. Pulmonary sarcoidosis a disorder mediated by excess helper T-lymphocyte activity at sites of disease activity. N Engl J Med 1981 305 429 32. 

COPE with Cytokines. 2006. Available at http //www.copewithcytokines.de/. Kunitake R, Kuwano K, Miyazaki H, et al. Apoptosis in the course of granulomatous inflammation in pulmonary sarcoidosis. Eur Respir J 1999 13(6) 1329-1337. Agostini C, Zambello R, Sancetta R, et al. Expression of tumor necrosis factor-receptor superfamily members by lung T lymphocytes in interstitial lung diseases. Am J Respir Crit Care Med 1996 153 1359-1367. 

Treatment of sarcoidosis remains controversial. CSs are the cornerstone of therapy for severe or progressive sarcoidosis (pulmonary or extrapulmonary), and often produce dramatic resolution of disease (28,157). The long-term benefit of CS therapy has not been established, as relapses may occur upon taper or cessation of therapy (5,22,28,157). Early prospective, randomized smdies found no long-term benefit with CSs among patients with pulmonary sarcoidosis (158-161). However, these studies included patients with normal or near normal... 

BraunerM, Grenier Pet al (1989) Pulmonary sarcoidosis Evaluation with high resolution CT. Radiology 172 467-471 Brauner M, Lenoir S et al (1992). Pulmonary sarcoidosis CT assessment of lesion reversibility. Radiology 182 349-354 Cullen MR (2005) Screening for chronic beryllium disease one hurdle down, two to go. Am J Respir Crit Care Med 171 3-4... 

Respiratory diseases Symptomatic sarcoidosis Loeffler syndrome not manageable by other means berylliosis fulminating or disseminated pulmonary tuberculosis when used concurrently with appropriate antituberculous chemotherapy aspiration pneumonitis. 

Pulmonary diseases Aspiration pneumonia, bronchial asthma, prevention of infant respiratory distress syndrome, sarcoidosis... 

Interstitial lung diseases Idiopathic pulmonary fibrosis Sarcoidosis... 

Finally, WHO group V includes diagnoses not fitting into any of the above categories, such as sarcoidosis, pulmonary histiocytosis, extrinsic compression of pulmonary artery, etc. Sarcoidosis may involve chronic lung disease, an active vasculitis, extrinsic compression, or cardiac disease. 

Sarcoidosis is a multisystem granulomatous disease of unknown origin that occurs most commonly in young adults. Pulmonary manifestations occur in more than 90% of patients. Accumulation of CD4 T cells that proliferate in situ spontaneously produces inflammatory cytokines and causes a lymphocytic alveolitis and granulomatous lesions. The disease is characterized by autoantibodies on erythrocytes (Pilatte et al., 1990), but deregulation of the immune response, in particular the cytokine response, is an alternative explanation to an autoimmune pathogenesis. 

A sarcoidosis-like pulmonary disease has been clearly associated with beryllium exposure. 

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