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We describe a patient with localized chronic fibrosing vasculitis who presented with a rhinophymalike eruption of the nose. To our knowledge, the manifestation of this condition on the nose has not been previously reported. The differential diagnosis also is examined.
The content of blood plasma corticosterone, testosterone and estradiol, and receptors to corticosteroids, androgens and estrogens in pulmonary cytosol of normal male rats and those with fibrosing process in the lungs were experimentally studied by means of radioimmunoassay. With pulmonary fibrosis, a decrease in the concentration of blood plasma corticosterone and testosterone, and lower content of their pulmonary cytosol receptors to corticosteroids, androgens and estrogens are observed. As testosterone propionate was administered in the course of this experiment, there was a normalization of blood plasma corticosterone levels and those of the pulmonary cytosol receptors to androgens. The content of blood plasma testosterone and the pulmonary cytosol receptors to corticosteroids and estrogens was partially normalized. However, it was significantly different from the indices in the control group.
A 42-year-old woman with a known history of paraquat ingestion was admitted to our hospital on the 10th day after ingestion complaining of dyspnea and general fatigue. Steroid therapy and low dose oxygen therapy were administered. She recovered and was discharged on the 190th day after paraquat ingestion. A chest X-ray film on admission disclosed a bilateral ground-glass shadow. As time passed, a reticular shadow with a loss of lung volume leaving a peripherally clear zone appeared on chest X-rays and shrank gradually toward the peri-hilar area during the course. CT of the lung clearly demonstrated progress of the pulmonary fibrosing process. In the early phase of paraquat poisoning, CT revealed diffuse high density areas dominant in the posterior part of the lungs with a subpleural intact area. The high density area was sharply demarcated from the subpleural intact area. As time passed, the high density area shrank toward the central zone, causing bullous changes in the subpleural area. The dominant fibrotic change in the intermediate and central zones seems to be one of the characteristic findings in paraquat induced pulmonary fibrosis.
The authors describe the results of examination of 67 patients with exogenous allergic and idiopathic fibrosing alveolitis. Of these, 42 patients underwent plasmocytapheresis in addition to corticosteroid therapy. The clinical improvement, positive x-ray dynamics of pulmonary lesions, normalization of the altered laboratory indicators were noted in 39 out of the 42 patients treated with the use of plasmocytopheresis and in 15 out of 25 who received pharmacotherapy alone. The use of plasmocytapheresis at the inpatient stage of the treatment enabled the dose of corticosteroids to be reduced by 26% of the initial on the average in 3/4 of the patients and subsequent hospitalization requirement to be noticeably decreased. Plasmocytapheresis did not produce any complications.
A case is described of a syndrome of pains in the lower extremities in a young man. After a number of diagnostic procedures the more common disease entities were excluded and "fibrositis" was accepted as the cause. The diagnosis was confirmed by EMG investigation, histological examination of dermomuscular specimens, positive effect of treatment and a 6-month follow-up.
Data are reported of a rare disease of idiopathic fibrosing alveolitis in association with an adenoma of the thyroid gland in a 68 years old woman. The authors emphasize difficulties in the clinical roentgenological and morphological diagnosis in this kind of combined pathology.
The main criteria for the morphologic diagnosis of fibrosing lung disease are the type of inflammation, the localization of the lesion within the lung, and the microtopographic pattern of inflammation and fibrosis. Among the microtopographic patterns six types (perilobular, intraalveolar, alveoloseptal, bronchiolitic, vasculitic, bronchiolectatic), all corresponding to pathogenetic mechanisms can be recognized. Mainly one of these basic patterns is realized in sarcoidosis, histiocytosis X and shock lung. In other diseases a combination of two or more patterns may occur. The microtopographic pattern can be shown best in open biopsies after unfolding of the lung tissue. The findings in bronchoalveolar lavage do not reflect the real composition of the inflammatory infiltration of the lung tissue, and especially T-lymphocytes are usually overrepresented.
Inhalation of fibrogenic mineral dust may enhance fibronectin (Fn) production by alveolar macrophages and increase fibroblast growth activity (FGA) in lung lavage fluid. To investigate the relationship of these changes to fibre retention and the development of asbestosis, we exposed 15 sheep to 100 mg Canadian chrysotile fibres in 100 ml saline, and ten sheep to 100 ml saline only, at ten day intervals. The animals were studied at 3 month intervals. At month 18, ten sheep had abnormal chest radiograph (category greater than or equal to 1) (group B) and five had normal radiograph (category 0) (group A). Pulmonary function data indicated restrictive patterns of abnormalities in both groups, more severe in group B. Sheep in group A had bronchoalveolar lavage (BAL) cellularity and biochemistry comparable to controls; sheep in group B had significant increases in total BAL cells (x2), macrophages (x2), neutrophils (x4) and eosinophils (x3), increased BAL lactate dehydrogenase and Fn, but FGA and procollagen 3 comparable to controls. Fibre retention was significantly increased in all exposed sheep and 2.5 x higher in group B vs group A despite similar exposures (70 intratracheal, 100 mg chrysotile infusions). Enhanced fibre retention in group B preceded the appearance of disease. This study confirms our earlier observation linking individual susceptibility to development of alveolitis to individual dust burden and provides evidence that the excess of fibre retention can be observed before detectable disease. In addition, we report a chrysotile-induced early alveolitis with incompletely expressed fibrosing activity at the time of initial radiographic detection. The intensity of the alveolitis is related to the degree of fibre retention.
Many chronic inflammations of various origin are characterized by an elevated metabolic activity of connective tissue, causing an increased tissue proliferation. The authors first review the more recent methods of analyzing the connective tissue metabolism. Details of changes of this metabolism are described with reference to liver fibrosis and rheumatoid arthritis. First steps of a pathobiochemical diagnosis of activity in organ fibroses are discussed. It seems possible to judge the activity of liver fibrosis by estimating the serum activity of lysosomal glycosidases and serum levels of terminal collagen peptides. Elevated antiprotease serum levels in rheumatoid patients may indicate an inflammatory activity.
Thirty-five individuals with fibromyalgia (fibrositis), 22 of their physicians, and 49 rheumatologists on an Arthritis Foundation referral list rated the importance of 24 aspects of fibromyalgia treatment. These encompassed symptom control, psychosocial factors, information, and physical therapy. Respondents with fibromyalgia rated their satisfaction with the way each aspect of treatment had been managed by their physician, and each completed a health status questionnaire. Fibromyalgia patients viewed 8 of the 24 aspects of treatment as significantly more important than did their own physician, and 18 of the 24 as significantly more important than did area rheumatologists. Satisfaction with the way treatment had been managed was generally low. Some evidence suggested that patients' health status was affected positively by the extent to which their physician viewed certain aspects of treatment as important. The results are expected to be useful in the design of fibromyalgia education programs for both lay and health professional audiences.
The incidence of pleural mesothelioma (PM), chronic fibrosing pleurisy (CFP) and calcified pleural plaques (CPP) has been studied in Anatolia (Turkey) in relation with environmental pollution by mineral fibers: 1) In central Anatolia, where asbestos deposits have been described, the frequency of CFP and CPP was in the range 2-25%; several cases of PM have been encountered. 2) One area (Cappadoce) is free of asbestos, but contains another kind of mineral fiber: zeolite. In this area, an outbreak of PM has been described in some villages. Among the 600 inhabitants of Karain, 28 cases of PM occurred for the period January 1975-June 1979.
Cytological and immunological investigations of bronchoalveolar lavage fluid (BLF) have been performed in 87, 27, 32 and 94 patients with fibrosing alveolitis (FA), exogenous allergic alveolitis (EAA), histiocytosis and pulmonary sarcoidosis (PS), respectively. The diseases appeared to demonstrate significant differences in BLF cell composition and relations between T-lymphocyte theophyllin-resistent and theophyllin-sensitive subpopulations. The findings can be applied in differential diagnosis of disseminated processes in the lungs. The number of neutrophils and the subpopulations correlation in FA and EAA may serve criteria of the disease activity and adequacy of the treatment administered.
Alterations that occurred to the hemispheric structure as a result of experimentally induced meningeal fibrosis are reported in this paper. The rinsing function of cerebrospinal fluid was unilaterally stopped in the subarachnoidal space of domestic pigs by means of a viscous medium which contained soluble fatty acid salts. The animals were sacrificed 35 or 95 d after the experiments. Series of frontal sections of both cerebral hemispheres were planimetrically measured to establish differences between hemispheric cross-sections. Left-side deficits between 6 and 29% were recordable from all cases. Microscopic findings included large meningeal fibroses without visible changes to the cortex and others with destruction of cortical regions. Early alterations to cortical cytoarchitecture were investigated by means of automatic image analysis. Increased cell volume densities were recorded from the third and fifth cortical layers in cortex parts with no grossly visible alterations. The authors feel that the above alterations were primarily attributable to gradual occlusion of the lesser meningeal and cortical veins.
Thirty-six patients with fibrositis received low dose amitriptyline and placebo in a randomized double blind crossover study lasting 10 weeks. Amitriptyline was associated with significant changes on the outcome measures of pain, tender point sensitivity and patient assessment of well being. Clinically significant improvements for pain and tender point sensitivity and a statistically significant improvement in generalized pain responsiveness were found between patients who reported subjective improvement on amitriptyline and those who felt no change.
Sera from patients with autoimmune lung and connective tissue diseases were investigated for antibodies to topoisomerase II. Anti-topoisomerase II antibodies were detected by ELISA in 37% of sera from patients with cryptogenic fibrosing alveolitis (CFA), in one (8%) case of sarcoidosis and in 31% of sera from systemic lupus erythematosus (SLE) patients. Sera from rheumatoid arthritis, juvenile rheumatoid arthritis, progressive systemic sclerosis, Sjögren's syndrome and undifferentiated connective tissue disease were negative. CFA and sarcoidosis sera strongly reacted in immunoblotting with a 170 kD protein, also recognized by rabbit antiserum to recombinant topoisomerase II, while SLE sera presented a weak reaction. Pre-adsorption with dsDNA dramatically decreased the topoisomerase II binding in ELISA by the most positive SLE serum, but did not affect the binding by the most positive CFA serum, thus indicating that anti-topoisomerase II reactivity of SLE sera is probably due either to cross-reacting antibodies or, in part, to minor DNA contamination of our enzyme preparation. The determination of DNA topoisomerase II relaxation activity, performed after incubation with antibody-positive sera, showed that only CFA sera precipitate enzymatic activity. The finding that CFA presents antibodies to an enzyme essential to cell survival stresses the role of autoimmunity in the pathogenesis of idiopathic pulmonary fibrosis. This may offer further insight into the cause of autoimmune disease and prove a valuable tool in the study of enzyme molecular biology.
A rare case of fibrosing mediastinitis, possibly of tuberculous etiology, causing superior vena caval obstruction is presented. The diagnosis was based on clinical features of superior vena caval obstruction, chest radiography, phlebography and mediastinal calcification in absence of definite mass lesion on CT-scan of thorax. The disease followed a relatively benign non-progressive course over next nineteen months of follow-up.
Chronic diffuse myalgia, localized areas of tenderness, fatigue, and unrefreshing sleep are related to a physiologic arousal disorder within sleep, that is, the alpha EEG NREM sleep anomaly. This sleep physiologic disorder, nonrestorative sleep, and symptoms of fibrositis syndrome are shown to occur with psychologic, environmental, and physiologic distress conditions. Pathogenic mechanisms that link nonrestorative sleep physiology to pain and fatigue may involve metabolic dysfunction of the brain with sleep-related alteration in immunologic and neurotransmitter functions (serotonin, substance P, endorphins). These sleep-related mechanisms have important implications for the understanding and treatment of fibrositis/fibromyalgia syndrome.
This review summarizes the physiologic and clinical evidence that shows nonrestorative sleep to be associated with chronic fatigue and diffuse myalgia after a flulike illness. Such a febrile illness may trigger alteration in sleep-wake brain and immune functions in patients with fibrositis or chronic fatigue syndromes.