Cryptogenic fibrosing alveolitis predating ulcerative colitis.
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A diffuse interstitial pulmonary fibrosis occurred in a 74-year-old woman treated for ten years with labetalol for systemic hypertension. Bronchoalveolar lavage (BAL) revealed lymphocytic and neutrophilic alveolitis. The lack of another etiologic factor and the spontaneously favorable course after withdrawal of the drug support the hypothesis of a labetalol induced pulmonary fibrosis.
It is important to know the diagnostic procedure and therapy of polymyositis (PM) inspite of its rare incidence. We describe a case with PM, interstitial lung disease, high titers of anti-Jo-1-antibodies and affection of the right hand, that could be followed over 11 years. The differentiation of PM from neurologic diseases and toxic myopathies is mentioned. The patient was successfully treated with cyclosporin over a period of 20 months, but he is now in remission while receiving steroids alone. We emphasize the importance of early treatment of PM with high-dose steroids and the addition of immunosuppressive drugs (azathioprin, methotrexate, cyclophosphamid) in cases with high disease activity or resistance to steroids. In the treatment of PM/DM immunoglobulins play an increasingly important role. The problems of long-term treatment with steroids are of special interest.
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BACKGROUND: High resolution computed tomography (HRCT) revealed new findings as regards changes associated with cryptogenic fibrotizing alveolitis. So far views on changes suggesting activity of the disease are not uniform and they were not yet confronted with examinations of fluid obtained by bronchoalveolar lavage (BALT). The objective of the present work is to evaluate the contribution of HRCT to the assessment of the activity of the disease and compare the findings with the examination of BALT. METHODS AND RESULTS: The authors evaluated the HRCT examination made in 52 patients with histologically confirmed cryptogenic fibrotizing alveolitis and in 39 of them the finding was confronted with the BALT examination. Signs of active inflammatory disease according to HRCT comprised a ground-glass increase of the density in 80.8%, centrilobular micronodules in 17.3% and a prominence of centrilobular structures in 32.7%. The suggested extent of affection of the pulmonary parenchyma assessed according to HRCT correlated well with the degree of dyspnoea, restrictive and obstructive ventilation disorder, pulmonary diffusion and with hypoxaemia. The authors confirmed also an inverse correlation between the grade assessed by HRCT and the percentage of lymphocytes in BALT. CONCLUSIONS: The investigation confirmed the contribution of HRCT in the diagnosis of patients with cryptogenic fibrotizing alveolitis, it confirmed the complementary importance of HRCT and BALT examination when assessing the activity of the disease and its asset when monitoring patients during treatment.
We describe an experimental model of the fasciitis-panniculitis syndrome, which includes eosinophilic fasciitis and its related disorders. Rats were given a single or repeated subcutaneous injections of saline or a 10% lipase solution. The injection sites were studied histologically and histomorphometrically. With few exceptions, the subcutaneous-fascial unit of the saline-injected rats was normal. An acute necrotizing panniculitis with inflammatory involvement of the adjacent fascia was found 2 days after a single injection of lipase. Three to 6 weeks after a single injection of lipase, the subcutaneous fat tissue and fascia were fibrotically thickened and chronically inflamed. Similar but more advanced alterations had developed in animals killed 3 weeks after three weekly injections of lipase. Progressive fibrotic thickening of the subcutaneous-fascial unit was observed following an increasing number of weekly lipase injections. The acute-phase reaction expresses a pattern of steatonecrotic tissue damage. In the ohronic phase, the fibrotically thickened subcutaneous layers typify a reaction pattern analogous to that of the human fasciitis-panniculitis syndrome. This experimental model permits better appreciation of the disorder's nonspecific pathogenesis and may help in the search for alternative therapeutic modalities.
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To evaluate the possibility that TGF-beta and TNF-alpha are involved in fibrosis induced in mouse lung by irradiation, the proportion of cells immunoreactive for each was compared in two strains of mice. C3HeB/FeJ mice develop only classical pneumonitis during the early phase, whereas C57L/J mice develop small, tightly packed areas of inflammation which undergo fibrosis during the latent period, and exhibit progressive fibrosis of large regions of intense inflammation during the early phase. Very few cells were immunoreactive for an antibody to the latency-associated peptide (LAP) of TGF-beta during the latent period in C3HeB/FeJ mice, and no cells were positive during the early phase. In contrast, between 0.7 and 10% of cells were positive in C57L/J mice in lesions without fibrosis and in lesions in the early stages of fibrosis. Fibroblasts positive for LAP were seen only in lesions containing fibrosis. A similar pattern of immunoreactivity was seen in C57L/J mice using an antibody which recognizes active TGF-beta, with the exception that positive fibroblasts were observed within areas of inflammation without fibrosis. Thus the association of active TGF-beta with fibroblasts might be a characteristic of the initiation of fibrosis in this model. TNF-alpha was detected in macrophages in all classes of lesions, and minor differences between the strains did not appear to be biologically meaningful.
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The prognostic features of the disease are summed up. The leading symptom of the disease is the dyspnoe followed by a dry hacking cough, loss of weight and fever. Clinically we find rales, cyanosis clubbing and signs of right heart insufficiency. Lung function data point to a decreased vital capacity and diffusing capacity; residual volume and functional residual capacity are about normal. Corticosteroid monotherapy is not an efficient treatment. The combination of prednisolon + azathioprine improves vital capacity but not working capacity. Prednisolone and d-penicillamine improve all parameters including working capacity. This can be enhanced by still adding azathioprine or cyclophosphamide to prednisolone and d-penicillamine.
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