muramidase and Scleroderma--Systemic

A 65-year-old man was diagnosed with systemic sclerosis on the basis of skin thickening and positivity of anti-Scl-70 antibodies. Because myogenic enzymes, such as creatinine phosphokinase and aldorase, were also elevated, myopathy or myositis associated with systemic sclerosis was considered. Muscle magnetic resonance imaging and gallium scintigraphy did not show abnormalities. Findings of muscle biopsy demonstrated presence of noncaseating granulomas with multinucleated giant cells. In addition, serum angiotensin-converting enzyme and lysozyme were elevated, and therefore a diagnosis of sarcoid myopathy was made. Further, renal sarcoidosis was revealed with renal biopsy. Prednisolone (40 mg/day) improved both the myopathy and nephritis. Sarcoid myopathy is a rare condition, but it should be considered when myogenic enzymes are elevated in the patient with systemic sclerosis. Further, muscle biopsy may be essential to make an accurate diagnosis in such condition.

Topics: Aged; Biopsy; Giant Cells; Granuloma; Humans; Kidney Diseases; Male; Muramidase; Muscle, Skeletal; Muscular Diseases; Prednisolone; Renin; Sarcoidosis; Scleroderma, Systemic; Treatment Outcome

Interstitial lung diseases are characterised by the recruitment of mononuclear cells to disease sites where maturation occurs and activation products, including lysozyme (LZM), are released. Analysis of in vitro cell culture supernatants for activation products masks the functional heterogeneity of cell populations. It is therefore necessary to examine the secretion of activation products by single cells to assess whether the activation of newly recruited mononuclear phagocytes at the sites of disease in the lung is uniform and controlled by the local microenvironment.. The reverse haemolytic plaque assay was used to evaluate, at a single cell level, the ability of bronchoalveolar lavage (BAL) fluid from seven patients with sarcoidosis to activate Ficoll-Hypaque-separated peripheral blood mononuclear cells by comparison with BAL fluid from six normal volunteers and nine patients with systemic sclerosis. Monolayers of peripheral blood mononuclear cells and sheep red blood cells were cultured either alone or in the presence of 20% (v/v) BAL fluid with a polyclonal anti-LZM antibody. LZM/anti-LZM complexes bound to red blood cells surrounding the secreting cells were disclosed following complement lysis of red blood cells and quantification of plaque dimensions using microscopy and image analysis.. Bronchoalveolar lavage fluid from all the patients with sarcoidosis increased LZM secretion by peripheral blood mononuclear cells compared with unstimulated mononuclear cells. By contrast, BAL fluid from the other individuals had no effect on LZM secretion.. Single cells activated by BAL fluid can be evaluated by the reverse haemolytic plaque assay. BAL fluid from patients with sarcoidosis, but not from patients with systemic sclerosis or normal individuals, contains components capable of activating mononuclear phagocytes to secrete lysozyme.

Topics: Adult; Bronchoalveolar Lavage Fluid; Epithelium; Female; Hemolytic Plaque Technique; Humans; Immunoenzyme Techniques; Male; Middle Aged; Monocytes; Muramidase; Sarcoidosis, Pulmonary; Scleroderma, Systemic