Sarcoidosis pulmonary

Schipperijn AJM. Flare-up of toxoplasmosis due to corticosteroid therapy in pulmonary sarcoidosis. Ned T Geneesk 1970 114 1710. 

The early impression that interferon alfa, alone or in combination with ribavirin, could reactivate or cause new subcutaneous sarcoid nodules and pulmonary or generalized sarcoidosis, has been confirmed by several reports, with prompt recovery after interferon alfa withdrawal (SED-13, 1097) (SEDA-20, 330) (SEDA-22, 404). The incidence may have been underestimated in one series, 3 patients out of 60 who received interferon alfa alone or combined with ribavirin developed pulmonary sarcoidosis (351). In a review of 27 cases, the time to onset was 15 days to 30 months, and there were dermatological signs in 50% (352). Five patients had also taken ribavirin, but an enhanced T cell immune reaction from the combination of interferon alfa plus ribavirin is speculative. However, the association of cutaneous or systemic sarcoidosis with interferon alfa, alone or in association with ribavirin, has been exemplified by various reports (353,354), including one patient whose sarcoidosis resolved with prednisone despite continued interferon alfa treatment (355). 

Abdi EA, Nguyen GK, Ludwig RN, Dickout WJ. Pulmonary sarcoidosis following interferon therapy for advanced renal cell carcinoma. Cancer 1987 59(5) 896-900. 

Hance, A.J., Douches, S., Winchester, R.J., Ferrans, J. and Crystal, R.J. (1985). Characterisation of mononuclear phagocyte subpopulations in the human lung by using monoclonal antibodies changes in alveolar macrophage phenotype associated with pulmonary sarcoidosis. J. Immunol. 134, 284-292. 

Car, B.D., Meloni, F., Luisetti, M. etal. (1994). Elevated IL-8 and MCP-1 in the bronchoalveolar lavage fluid of patients with idiopathic pulmonary fibrosis and pulmonary sarcoidosis. Am. J. Respir. Crit. Care Med. 149, 655-659. 

Moller DR, Forman JD, Liu MC, et al. Enhanced expression of IL-12 associated with Thl cytokine profiles in active pulmonary sarcoidosis. J Immuno 1996 156 4952-60. 

Pilatte Y, Tisserand EM, Greffard A, Bignon J, Lambr6 CR (1990) Anticarbohydrate autoantibodies to sialidase-treated erythrocytes and thymocytes in serum from patients with pulmonary sarcoidosis. Am J Med, 88 486-492. 

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. 

Ansell J, Hirsch J, Dahlen J, Bussey H, Anderson D, Poller L, Jacobson A, Deykin D, Matchar D (2001) Managing oral anticoagulant therapy. Chest 119(SuppI 1) 22S-38S Berlin M, Fogdell-Hahn A, Olerup O, EkIund A, Grunewald J (1997) HLA-DR predicts the prognosis in Scandinavian patients with pulmonary sarcoidosis. Am J Respir Crit Care Med 156 1601-1605... 

Girgis RE, Basha MA, Maliarik M, Popovich J, lannuzzi MC. Cytokines in the bronchoalveolar lavage fluid of patients with active pulmonary sarcoidosis. Am J Respir Crit Care Med 1995 152 71-75. 

Petrek M, Drabek J, Kolek V, et al. CC chemokine receptor gene polymorphisms in Czech patients with pulmonary sarcoidosis. Am J Respir Crit Care Med 2000 162 1000-1003. 

In vitro, blood monocytes of patients suffering from sarcoidosis incorporate more 3H-cytidine into their nuclei than do monocytes from healthy persons with a positive tuberculin test (M.Schmidt et al. 1970). In pulmonary sarcoidosis, the patent cap-... 

As the chnical course of pulmonary sarcoidosis has been correlated with patients individual capacities for spontaneous tumour necrosis factor production by alveolar macrophages (Muller-Quernheim et al. 1992, Zheng et al. 1995), modulation of TNF in a more specific way than corticoids... 

Osteopontin, a negatively charged, glycosylated sialoprotein, absent in normal lung or granulation tissue and all isotype controls, was extensively expressed in the macrophages in biopsies from patients with pulmonary sarcoidosis (Chupp et al. 1996). 

Akira M, Kozuka T et al. (2005) Long-term follow-up CT scan evaluation in patients with pulmonary sarcoidosis. Chest 127 185-191... 

Murata K, Itoh H et al. (1986) Centrilobular lesions of the lung demonstration by high-resolution CT and pathologic correlation. Radiology 161 641-645 Murdoch J, Muller NL (1992) Pulmonary sarcoidosis changes on follow-up CT examination. AJR Am J Roentgenol 159 473-477... 

Nakatsu M, Hatabu H et al. (2002) Large coalescent parenchymal nodules in pulmonary sarcoidosis sarcoid galaxy sign. AJR Am J Roentgenol 178 1389 1393 Nishimura K, Itoh H etal. (1993) Pulmonary sarcoidosis correlation of CT and histopathologic findings. Radiology 189 105 109... 

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 ). 

Figure 12 HRCT demonstrating bilateral parenchymal nodularity with a predominate juxta bronchial distribution (A) and bulky hilar adenopathy (B) in this patient with pulmonary sarcoidosis. Abbreviation HRCT, high-resolution computed tomography.

Akira et al. examined initial and follow-up CT scans in 40 patients with pulmonary sarcoidosis (95). Predominantly nodular or multiple large nodules disappeared or decreased in size at follow-up. A conglomeration pattern shrank and evolved into bronchial distortion and decline in FEVi/FVC ratio. Interestingly, GGO and consolidation evolved into honeycombing in some patients. Because sarcoidosis has the potential to evolve over time, initial CT features have limited prognostic value (76). However, certain CT features have predictive value. Focal nodules, alveolar opacities, consolidation, or GGO suggest an active inflammatory component that may reverse with therapy (76,85,96). In contrast, distortion of lung parenchyma, coarse or hnear bands, bronchiectasis, cystic radiolucencies, and bullae reflect irreversible fibrosis (78,79,85,96). 

Brauner MW, Grenier P, Mompoint D, et al. Pulmonary sarcoidosis evaluation with high-resolution CT. Radiology 1989 172(2) 467-471. 

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. 

Tetasaki H, Fujimoto K, Muller NL, et al. Pulmonary sarcoidosis comparison of findings of inspiratory and expiratory high-resolution CT and pulmonary function tests between smokers and nonsmokers. AJR Am J Roentgenol 2005 185(2) 333-338. 

Hansell DM, Milne DG, Wilsher ML, et al. Pulmonary sarcoidosis morphologic associations of airflow obstruction at thin-section CT. Radiology 1998 209(3) 697-704. 

Magkanas E, Voloudaki A, Bouros D, et al. Pulmonary sarcoidosis. Correlation of expiratory high-resolution CT findings with inspiratory patterns and pulmonary function tests. Acta Radiol 2001 42(5) 494—501. 

Swider C, Schnittger L, Bogunia-Kubik K, et al. TNF-alpha and HLA-DR genotyping as potential prognostic markers in pulmonary sarcoidosis. Eur Cytokine Netw 1999 10(2) 143-146. 

Sharma SK, Balamurugan A, Pandey RM, et al. Human leukocyte antigen-DR alleles influence the clinical course of pulmonary sarcoidosis in Asian Indians. Am J Respir Cell Mol Biol 2003 29(2) 225-231. 

The dose of glucocorticoids varies widely. Most studies have used initial doses of 20 to 40 mg/day of prednisone or its equivalent and then decrease the dose as tolerated. For pulmonary sarcoidosis, there is no clear-cut evidence for any particular initial dose of prednisone (21). In cardiac sarcoidosis, survival rates did not differ among patients treated with initial dose of >30-mg prednisone daily compared with lower doses (33). 

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. 

The use of AZA for IPF was first reported in an open-label trial by Winterbauer et al. (144). A subsequent double-blind, randomized trial demonstrated benefit versus placebo in approximately half of the patients treated (145). Demerits et al. demonstrated a higher response rate when AZA was given concomitantly with A-acetyl cysteine compared with AZA alone (146). AZA has also been used in the treatment of scleroderma-associated pulmonary fibrosis (147). Although not all investigators have reported benefit with the drug (148), the drug has also been useful in treating chronic pulmonary sarcoidosis (7). 

Thalidomide can be effective for treating cutaneous sarcoidosis (310-312). Because the drug has many actions (313,314), including the suppression of TNF-ot (313,315,316), it is a possible agent to treat pulmonary sarcoidosis (317). 

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). 

Young RL, Harkekoad LE, Lorden RE, et al. Pulmonary sarcoidosis a prospective evaluation of glucocorticoid therapy. Ann Intern Med 1970 73 207-212. 

Pietinalho A, Lindhohn A, Haahtela T, et al. Inhaled budesonide for treatment of pulmonary sarcoidosis. Results of a double-blind, placebo-controlled, multicentre study. Eur Respir J 1996 9(suppl 23) 406s. 

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