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PubMed1988

Plasma substance P levels in fibrositis.

The mechanism of pain in the fibrositis syndrome is unknown. We measured plasma levels of substance P in 32 patients with fibrositis and 26 sex and age matched controls using a radioimmunoassay. The mean plasma level of substance P in the patients with fibrositis was 371 +/- 91 pg/ml and in controls 397 +/- 84 pg/ml (p = NS). We conclude that determination of plasma levels of substance P in fibrositis is of no diagnostic value. This does not exclude the possible role of substance P as a neurotransmitter in the fibrositis syndrome.

Adult↗
PubMedDate not supplied

[Comparative epicutaneous study on allergy to mild-fibrosing agents].

In connection with clinical observations and literature data about allergic manifestations in the oral cavity in the treatment of periodontal pockets with mild-fibrosing agents, we performed a comparative epicutaneous study on the preparations most often used in our country: Phytodont, maraslavin, zemisvet and preparation P. The method of Jadahsson and Block was used in patients with manifested periodontosis. Positive allergic tests with zemisvet and preparation P have not been established so far, whereas they were 5.71% with maraslavin and 8.57%--with phytodont. That provided grounds to recommend the fibrosing therapy in the single courses to be performed with various mild-fibrosing agents so as to reduce the possibilities of sendibilization. In the presence of data about drug allergy, the patients should be tested for allergy to the respective mild-fibrosing agents prior to the fibrosing therapy.

Drug Hypersensitivity↗
PubMed1997

Fibrosing alveolitis in systemic sclerosis: indices of lung function in relation to extent of disease on computed tomography.

OBJECTIVE: Thin-section computed tomography (CT) provides a sensitive and reproducible method of quantifying the morphologic extent of disease in the clinical management of fibrosing alveolitis associated with systemic sclerosis (FASSc). The aim of this study was to determine which indices of lung function best reflect the extent of disease on CT in FASSc, and to determine the independent influences of smoking history, extent of fibrosing alveolitis, demographic features, and concurrent treatment upon functional impairment in FASSc. METHODS: Sixty-four patients with FASSc were studied using CT and static and exercise lung function testing. Statistical relationships were determined by multiple regression analyses. RESULTS: Five patients with overt pulmonary hypertension were characterized by severe impairment in 3 indices of lung function: diffusing capacity for carbon monoxide (DLCO), DLCO adjusted for alveolar volume (KCO), and arterial partial pressure of oxygen. On multiple regression analysis, the major determinant of functional impairment was the extent of fibrosing alveolitis on CT. A history of smoking was independently associated with preservation of total lung capacity and depression of KCO, but did not otherwise influence functional-morphologic correlations. The percent predicted DLCO correlated better with extent of disease on CT (r = -0.70) than did oxygen desaturation on exercise (r = 0.55), the physiologic component of the clinical-radiographic-physiologic score (CRP index) (r = 0.52), or other indices of lung function. Lung volume measures correlated poorly with disease extent on CT. CONCLUSION: The percent predicted DLCO best reflects the extent of fibrosing alveolitis in FASSc, and therefore should be measured in routine evaluations. Exercise testing may also have a useful role in staging the severity of pulmonary fibrosis, but the CRP index offers no additional advantage over the DLCO and exercise testing.

Carbon Monoxide↗
PubMed1982

Fibrositis and psychologic disturbance.

Thirty patients with fibrositis and 2 control groups, one of rheumatoid arthritis patients and the other of arthritis patients with other than rheumatoid arthritis, were compared on the basis of the Minnesota Multiphasic Personality Inventory (MMPI) to assess the role of psychologic factors in fibrositis. Patients with fibrositis differed in both elevation and variability in their MMPI profiles, indicating that they were more psychologically disturbed than patients with rheumatoid or other types of arthritis. The fact that almost all of the fibrositis patients' MMPI scales were higher suggests that we might be dealing with a number of different psychologic disturbances that have stiffness and musculoskeletal pain as principal and common symptoms.

Adult↗
PubMed1986

Immunofluorescent studies of skin in primary fibrositis syndrome.

Primary fibrositis syndrome is a painful musculoskeletal disorder of unknown cause. Although numerous hypotheses have been proposed to explain its clinical features, few studies have demonstrated a reproducible tissue abnormality in this condition. Recently, five reports of cutaneous immunoreactant deposition in primary fibrositis syndrome have become available and form the basis of this review. Four of these reports have described immunoglobulin G deposition at the dermal-epidermal junction of sun-exposed skin with a prevalence ranging from 12 to 76 percent. One study reported immunoglobulin M deposition in the dermal vasculature of patients with primary fibrositis syndrome. The reasons for the lack of uniformity in reported prevalence rates and types of skin immunofluorescence in primary fibrositis syndrome are unknown but are assumed to be due to differences in patient populations and immunofluorescence techniques between reporting centers. These factors are discussed in detail and speculations as to the significance of these skin findings are expressed, particularly with respect to the concept of enhanced vascular permeability in these patients.

Epidermis↗
PubMed1986

Is there a metabolic basis for the fibrositis syndrome?

Patients with the fibrositis syndrome experience moderately severe musculoskeletal discomfort, mood changes associated with nonrestorative sleep, and tenderness to palpation at specific body sites. There is no characteristic abnormal laboratory finding in these patients to help identify the population. A report by Moldofsky and Warsh (Pain 1978; 5: 65-71) of low serum levels of free tryptophan in patients with severe fibrositis syndrome is intriguing but remains unexplained. Those data plus the observation by Hudson et al (Am J Psychiatry 1985; 142: 441-446; Biol Psychiatry 1984; 19: 1489-1493) that patients with fibrositis syndrome exhibit an increased prevalence of anxiety and depression suggest a number of possible avenues for further study. They include potential alterations in the homeostasis of catecholamines, corticosteroids, serotonin, aromatic amino acids, platelet membrane receptor levels, and the activity of platelet membrane monoamine oxidase. Among these possibilities, evidence is now available that suggests an increased production of catecholamines in fibrositis syndrome.

Animals↗
PubMed1986

Secondary fibrositis.

Fibrositis is considered primary when there is no associated underlying disorder, and secondary when it occurs in patients with underlying rheumatic or other organic disease. Since fibrositis has become better defined, the list of underlying disorders has grown, and its identification requires careful diagnostic study. The differentiation of primary and secondary fibrositis has therapeutic implications. Secondary fibrositis should respond to treatment of the underlying disorder, whereas the primary syndrome may require additional management directed to the musculoskeletal pain and sleep and emotional disturbances commonly recognized as major manifestations of this syndrome.

Arthritis, Rheumatoid↗
PubMed2002

Interstitial fibroblast-like cells express renin-angiotensin system components in a fibrosing murine kidney.

Recently, the renin-angiotensin system (RAS) was implicated in organ fibrosis. However, few studies have examined the localization of RAS components, such as angiotensin II receptors, renin (REN), angiotensinogen (AGTN), and angiotensin-converting enzyme (ACE), in the fibrosing kidney. To localize these components in the fibrosing kidney, we used a murine model of renal fibrosis that shows an enhanced expression of angiotensin II type 1A receptor (AT(1A)R) and AGTN. Our results indicate that the overall expression of angiotensin II type 2 receptor (AT(2)R) and ACE was attenuated in this model, whereas REN expression was unchanged. In addition to tubular epithelial cells that were positive for AT(1A)R, AT(2)R, REN, and AGTN, interstitial fibroblast-like cells expressed AT(1A)R, REN, AGTN, and ACE in the fibrosing kidney. The interstitial fibroblast-like cells that were positive for AT(1A)R mRNA were further characterized as positive for the expression of vimentin and transforming growth factor-beta1. These data provide strong evidence for a tubulointerstitial RAS within the fibrosing kidney, and a linkage between the RAS and renal fibrogenesis.

Animals↗
PubMed2006

[Hair darkening close to a patch of frontal fibrosing alopecia].

BACKGROUND: Fibrosing frontal alopecia is scarring form alopecia and is most often seen in menopausal women. It is currently considered as an anatomoclinical form of lichen planopilaris of selective topography. We report a case of hair repigmentation during the course of post-menopausal frontal fibrosing alopecia. CASE REPORT: A 78-year-old woman developed alopecia of the scalp and eyebrows in 1997. Her eyebrows had been white for some ten years. In 1999, she noted repigmentation of a strip of hair at the edge of her scalp, but the rest of her hair remained white. The patient had not been taking any long-term drugs or any hair treatment. Histological examination of the regions of alopecia revealed lesions of lichenoid appearance at the junction with erosion by lymphocytes and keratinocytic necroses of the lower layers of the epidermis. Direct cutaneous immunofluorescence testing was negative. A diagnosis of post-menopausal fibrosing frontal alopecia was made on the basis of the clinical and laboratory evidence. DISCUSSION: 96 cases of post-menopausal fibrosing frontal alopecia have so far been reported in the literature, but to our knowledge, this is the first case combined with or inducing hair repigmentation. The other cases of repigmentation were eliminated by history-taking, and clinical or laboratory examinations. The mechanism of hair repigmentation in our patient could have been the result of a post-inflammatory process.

Aged↗
PubMed1984

Quantitation of types I and III collagen in biopsy lung samples from patients with cryptogenic fibrosing alveolitis.

The role of changes in the relative amounts of types I and III collagen in cryptogenic fibrosing alveolitis was investigated using a biochemical method adapted for small amounts of human lung tissue. Measurements were performed in 18 biopsy and seven post-mortem lung samples from patients with cryptogenic fibrosing alveolitis and the results were compared with clinical, physiological, radiological and histological data. The mean percentage type III collagen, compared with type I collagen, was significantly lower in post-mortem lung samples from patients with cryptogenic fibrosing alveolitis who died from the disease compared with the biopsy lung samples (p less than 0.01) and with the control lung samples (p less than 0.05). There was a positive correlation between the percentage of type III collagen and response to treatment at six months as shown by the improvement in the percentage predicted Kco (p less than 0.005) and FVC (p less than 0.01) and with the radiological changes (p less than 0.05). These changes remained significant at one year (p less than 0.05 in all cases). These results suggest that patients with a higher proportion of type III collagen have earlier disease and show a better response to treatment than those with a lower proportion of type III collagen. The potential advantages of measuring collagen types for staging cryptogenic fibrosing alveolitis are discussed.

Aged↗
PubMed1997

Postmenopausal frontal fibrosing alopecia: a frontal variant of lichen planopilaris.

BACKGROUND: Lichen planopilaris usually produces multifocal areas of scarring alopecia. Recently, a condition in postmenopausal women characterized by progressive frontal hairline recession associated with scarring has been described. OBJECTIVE: Our purpose was to study the clinical and histopathologic features and results of treatment in a group of women with the frontal variant of lichen planopilaris and to compare the immunohistochemical profile of scalp biopsy specimens from this subset with that found in the multifocal variant of lichen planopilaris. METHOD: The clinical data as well as the histopathologic findings in 16 women with frontal fibrosing alopecia were collated. The immunohistochemical profile of six scalp biopsy specimens from the frontal hairline were compared with six specimens from women with multifocal lichen planopilaris. RESULTS: In addition to the progressive frontal fibrosing alopecia in all 16 women, total loss or a marked decrease of the eyebrows was observed in 13. No evidence of lichen planus was observed at other sites. In one patient multifocal areas of lichen planopilaris developed in the scalp. The frontal fibrosing alopecia was slowly progressive but has stabilized in five patients. Biopsy specimens from the frontal hairline showed histologic changes identical to lichen planopilaris. Immunophenotyping failed to reveal any significant differences between the frontal and multifocal variants. No effective treatments emerged although oral steroids and antimalarials may temporarily slow the course. Hormone replacement therapy did not appear to influence the course of the alopecia. CONCLUSION: Progressive frontal fibrosing alopecia is a clinically distinct variant of lichen planopilaris that affects in particular elderly women and frequently involves the eyebrows. The basis for this lichenoid tissue reaction targeting frontal scalp follicles and eyebrows is unknown.

Administration, Oral↗
PubMed1991

Sleep physiology and psychological aspects of the fibrositis (fibromyalgia) syndrome.

Fibrositis (fibromyalgia) patients were compared with normal controls in terms of electrophysiology (EEG), self-report indicants of awakening, quality of sleep, behaviourally signalled awakenings, and Symptom Check List 90R (SCL-90R) scores. The results differentiated fibrositis patients from normal controls in terms of SCL-90R scores, with fibrositis patients showing significantly more psychopathology. Fibrositis patients had more alpha EEG sleep and less REM and Stage 1 sleep. They were better able to recall their behaviourally signalled awakenings the following morning and reported qualitatively less satisfying sleep than the normal controls. The alpha EEG sleep anomaly may reflect a vigilant arousal state during nocturnal sleep and result in the daytime experience of unrefreshing sleep, psychologic distress, that re-enforces the perpetuation of the sleep-related symptoms.

Adult↗
PubMed2005

Nephrogenic fibrosing dermopathy, a new mimicker of systemic sclerosis.

Nephrogenic fibrosing dermopathy is a unique fibrosing disorder recently identified to occur exclusively among patients with renal disease. The cutaneous findings are similar to those of systemic sclerosis, but it is important to differentiate between these two disorders because of significant prognostic and therapeutic implications. Nephrogenic fibrosing dermopathy is usually a diagnosis of exclusion, but the condition does have distinct clinical and histopathologic findings. It appears to be multifactorial in pathogenesis, and no specific cause has been identified. No specific treatment modality has been consistently effective, but there have been reports of improvement that occurred either spontaneously with renal recovery or after renal transplantation. We present an interesting case of a 33-year-old woman diagnosed with nephrogenic fibrosing dermopathy, along with a review of the literature.

Adult↗
PubMed2006

Nephrogenic fibrosing dermopathy in a patient with acute renal failure never requiring dialysis.

Nephrogenic fibrosing dermopathy (NFD) is a newly recognized cutaneous fibrosing disorder. To date the etiology, pathogenesis, and clinical course remains unknown. The majority of cases have been in renal dialysis or renal transplant patients. Only four cases have been reported in which patients had acute renal failure and never required dialysis. Currently, there is no effective treatment. A 65-year-old man was hospitalized for pneumonia. During hospitalization he developed acute renal failure secondary to acute tubular necrosis. The patient had woody indurated plaques on his upper extremities and trunk, and brown indurated plaques on his trunk. Histopathological examination revealed a spindle cell proliferation infiltrating through the dermis and subcutis with mild mucin deposition. Nephrogenic fibrosing dermopathy is a novel fibrosing disorder diagnosed clinically and histopatholigically. Currently no risk factors other than renal failure have been identified. Our patient is a unique example of NFD observed in acute renal failure secondary to acute tubular necrosis, never requiring dialysis. We also review the four other cases of acute NFD never requiring dialysis that have been reported in the literature.

Acute Kidney Injury↗
PubMed1984

Asbestosis and cryptogenic fibrosing alveolitis: a radiological and functional comparison.

This study compares the relationship between lung function and radiological abnormality in 46 subjects with asbestosis and 38 subjects with cryptogenic fibrosing alveolitis. Radiographs were graded separately by two observers according to the 1980 International Labour Organisation criteria for classification of the pneumoconioses. The correlation between the transfer factor of the lungs and the radiographic profusion of small parenchymal opacities was found to be greater in subjects with asbestosis than in subjects with cryptogenic fibrosing alveolitis. At any level of radiological profusion transfer factor was higher in cases with asbestosis than in cases with fibrosing alveolitis. As pleural thickening is seen commonly in asbestosis and may influence lung volumes and the ratio of transfer factor to effective alveolar volume, the results of these measurements were compared only in the cases showing absent or minimal pleural thickening. Both of these variables were higher in the subjects with asbestosis. The results indicate that despite pathological, functional and radiographic similarities, lung function for a given degree of radiographic parenchymal abnormality is better in subjects with asbestosis than in subjects with cryptogenic fibrosing alveolitis, and that changes in the plain chest x-ray appearances in asbestosis correlate more closely with the results of lung function tests.

Asbestosis↗
PubMed2005

Frontal fibrosing alopecia: a survey in 16 patients.

BACKGROUND: Postmenopausal frontal fibrosing alopecia (PFFA) was described by Kossard et al. as a progressive recession of the frontal hairline affecting particularly postmenopausal women. Further cases of PFFA have been reported to date, all of them considering it as a variant of lichen planopilaris on the basis of its clinical, histological and immunohistochemical features. OBJECTIVE: To describe clinical features, and response to treatment of 16 cases of frontal fibrosing alopecia diagnosed at our department in the last 6 years. METHODS: In addition to clinical data, biopsies and laboratory tests (antinuclear antibodies, sex hormones, thyroid hormones) were performed in order to rule out other causes of scarring alopecia. Patients were treated with intralesional corticosteroids, finasteride, and minoxidil, depending on the stage of the disease and association to androgenetic alopecia. RESULTS: All patients presented progressive alopecia localized to the frontal and temporal hairlines. Eight patients (50%) had loss of eyebrows, and six patients (37.5%) had axillar alopecia. Ages ranged from 45 to 79. Three of these women were premenopausal. Androgenetic alopecia was evident in seven patients (43.8%). All patients biopsied showed perifollicular lymphocitic infiltrate with lamelar fibrosis limited to the upper portions of the follicle. The progression of the condition stopped in most patients after a variable period on treatment. When treatment was abandoned the alopecia progressed to 'clown alopecia' appearance. DISCUSSION: Cases of Kossard's type scarring alopecia affecting premenopausal women made us consider that this condition is not exclusive of postmenopausal women. Differential diagnosis should take into account conditions like female androgenetic alopecia, fibrosing alopecia in a pattern distribution, alopecia areata, and chronic lupus erythematosus. Except for the pattern of alopecia, lichen planopilaris and frontal fibrosing alopecia are indistinguishable, thus the latter is included as a variant of lichen planopilaris. Although the disease tends to spontaneous stabilization, intralesional and topical corticosteroids, and anti-androgens may stop the progression of the disease and improve the female androgenetic alopecia that usually is associated to FFA.

Aged↗
PubMed1997

Is fibrosing colonopathy an immune mediated disease?

Fibrosing colonopathy, a recently described complication of patients with cystic fibrosis, manifests clinically approximately 7-12 months after starting high dose pancreatic enzyme treatment. Although the pathogenesis of fibrosing colonopathy is unknown, it is highly correlated with pancreatic enzyme dose. In this study, immune mediated factors which may be associated with fibrosing colonopathy were explored. Sera from 14 patients with cystic fibrosis and meconium ileus were collected at diagnosis and then longitudinally for four to five years after enzyme treatment. Sera were analysed for total IgG and antiporcine trypsin IgG using an ELISA assay. Before enzyme treatment, serum antiporcine trypsin IgG concentrations were negligible, at 2.9 (SD 0.3) micrograms/ml. Thirteen patients (93%) developed a significant antibody response to porcine trypsin after starting enzyme treatment, reaching a peak concentration of 69.4 (20.1) micrograms/ml 7-12 months after the introduction of enzymes. Since peak IgG concentrations coincided with published reports of time of onset of symptoms of fibrosing colonopathy, local injury by protease or by immune mediated mechanisms may be responsible for the pathological changes in this iatrogenic disease.

Animals↗
PubMed1990

What causes cryptogenic fibrosing alveolitis? A case-control study of environmental exposure to dust.

OBJECTIVE: To investigate the role of occupational and domestic exposure to dust in the aetiology of cryptogenic fibrosing alveolitis. DESIGN: Matched case-control study. SUBJECTS: 40 Patients with cryptogenic fibrosing alveolitis and 106 community controls matched for age and sex who responded to a questionnaire. MAIN OUTCOME MEASURE: Responses to self administered questionnaire asking about lifetime exposure to dust, animals, and smoke at home and at work. RESULTS: The patients with cryptogenic fibrosing alveolitis were more likely to report occupational exposure to metal dust (matched odds ratio 10.97 (95% confidence interval 2.30 to 52.4), p less than 0.001) or wood dust (2.94 (0.87 to 9.90), p = 0.08), to have worked with cattle (10.89 (1.24 to 96.0), p = 0.01), and to have lived in a house heated by a wood fire (12.55 (1.04 to 114), p = 0.009). A history of smoking and social class based on occupation were not significantly related to disease state. CONCLUSION: Environmental exposure to dust may be an important factor in the aetiology of cryptogenic fibrosing alveolitis.

Aged↗