Pulmonary involvement in systemic sclerosis: the detection of early changes by thin section CT scan, bronchoalveolar lavage and 99mTc-DTPA clearance

Interstitial Lung Disease in Systemic Sclerosis: Diagnosis and Management

“…Increased numbers of lymphocytes and mast cells may also be seen [33]. Early studies correlated increased granulocytes in BAL with increased response to immunosuppression presumably because this represented active alveolitis [34,35]. Subsequently, BAL granulocytosis has been shown to correlate with the degree of ground glass opacity seen on HRCT [30] and with more advanced interstitial disease [36].…”

Elevated levels of leukotriene B4 and leukotriene E4 in bronchoalveolar lavage fluid from patients with scleroderma lung disease

“…Interstitial lung disease is a frequent complication of SSc and one of the main causes of death in SSc patients (1,2). Although the pathogenesis of scleroderma lung disease (SLD) is not completely understood, it has been documented that profound fibrosis of the lungs is accompanied and preceded by inflammatory processes (3,4). Cellular infiltration of the lung interstitium and elevated numbers of inflammatory cells in the pulmonary alveoli are hallmarks of inflammatory reactions in the lungs (5,6) .…”

Inflammation and Pulmonary Fibrosis

“…The pathogenesis of scleroderma lung disease is poorly understood. Early changes may include interstitial oedema and widening and inflammation of the alveolar walls with collections of mononuclear cells and neutrophils, leading to a combination of an inflammatory reaction and concommittant fibroblast proliferation (Harrison et al, 1989;Harrison et al, 1991). Tiny cysts result from progressive thinning and rupture of the alveolar walls associated with extensive interstitial and peri-bronchial fibrosis (Hayman & Hunt, 1952).…”

“…the accumulation of immune and inflammatory cells within the alveolar structures, precedes lung injury and may be the first step of the fibrotic process for which it may be entirely responsible. The alveolitis in scleroderma is characterized by an accumulation of activated alveolar macrophages, lymphocytes, neutrophils and eosinophils (Edelson et al, 1985;Harrison et al, 1989;Konig et al, 1984;Owens et al, 1986;Pesci et al, 1986;Silver et al, 1984;Wallaert et al, 1988;Zhu et al, 2002). In scleroderma, alveolar macrophages have been shown to spontaneously release greater amounts of superoxide anion than normal alveolar macrophages (Wallaert et al, 1988).…”