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Results for “FIBROSITIS”

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PubMed1996

[Postmenopausal frontal fibrosing alopecia].

We report on two patients with postmenopausal frontal fibrosing alopecia. This recently described disease affects postmenopausal women and is characterized by progressive recession of the frontotemporal and parietal hair margins. It leads to a symmetrical band-like scarring area of alopecia 3-6 cm wide. Histological examination shows perifollicular fibrosis and lymphocytic inflammation concentrated around the isthmus and infundibular areas of the follicles. One of our two patients also had vulvar lichen sclerosus et atrophicus. An effective treatment for this type of postmenopausal alopecia is yet not known, but the course of the disease seems to be self-limited.

Aged↗
PubMed2002

Traction bronchiectasis in cryptogenic fibrosing alveolitis: associated computed tomographic features and physiological significance.

Our objective was to evaluate the associated CT features and physiological consequences of traction bronchiectasis in patients with cryptogenic fibrosing alveolitis (CFA). The CT scans of 212 patients with CFA (158 men, 54 women; mean age 62.2+/-10.6 years) were evaluated independently by two observers. The extent of fibrosis, the proportions of a reticular pattern and ground-glass opacification and the extent of emphysema were scored at five levels. The predominant CT pattern, coarseness of a reticular pattern and severity of traction bronchiectasis were graded semiquantitatively. Physiological indices were correlated with CT features. There was traction bronchiectasis on CT in 202 of 212 (95%) patients. Increasingly severe traction bronchiectasis was independently associated with increasingly extensive CFA ( p<0.0005), a coarser reticular pattern ( p<0.001), a lower proportion of ground-glass opacification ( p<0.005) and less extensive emphysema ( p<0.0005). Increasingly severe traction bronchiectasis was independently related to depression of DL(CO) ( p<0.005), FVC ( p=0.02) and pO(2) ( p<0.0005), but not indices of air-flow obstruction. In CFA, traction bronchiectasis increases with more extensive disease, a lower proportion of ground-glass opacification and a coarser reticular pattern, but it decreases with concurrent emphysema. Increasingly severe traction bronchiectasis is associated with additional physiological impairment for a given extent of pulmonary fibrosis and emphysema.

Bronchiectasis↗
PubMed2004

Nephrogenic fibrosing dermopathy in pediatric patients.

Nephrogenic fibrosing dermopathy (NFD) is a rare and recently recognized sclerosing skin disorder of unknown etiology. Reported cases have occurred in patients with chronic renal failure, with or without renal replacement therapy. All previous cases have been reported in older adult patients. We describe two pediatric patients who recently developed this condition and review the existing literature for NFD. Our patients included an 8-year-old boy on peritoneal dialysis with no prior renal transplant and a 19-year-old boy on hemodialysis with a history of previous failed renal transplants. We speculate that the recent emergence of this condition and occurrence in patients with chronic renal failure suggest an association with some newer pharmacological agent that has recently come into wide use. Since both our patients also had previously experienced large vessel thrombosis, hypercoagulable states may also be implicated.

Adult↗
PubMed2006

Nephrogenic fibrosing dermopathy in children.

Nephrogenic fibrosing dermopathy (NFD) is an entity of unknown etiology that has only been reported in patients with impaired kidney function. It has characteristic diagnostic findings by skin biopsy and due to its systemic involvement in some reported cases, the term "nephrogenic systemic fibrosis" has been recently suggested as more appropriate for the nomenclature of this entity. There is no curative treatment currently available and very few cases have been reported in children. We hereby report two cases so as to alert pediatric health care providers about the existence of this disorder.

Adolescent↗
PubMed2003

Idiopathic pulmonary fibrosis/cryptogenic fibrosing alveolitis.

Idiopathic pulmonary fibrosis (IPF), synonymous with cryptogenic fibrosing alveolitis (CFA), is a progressive and usually fatal disease of unknown cause characterized by sequential acute lung injury with subsequent scarring and end-stage lung disease. Historically, IPF/CFA encompassed a heterogeneous group of different histological and clinical entities arising in an idiopathic setting. Recently, the American Thoracic Society (ATS) and European Respiratory Society (ERS) core committee has redefined diagnostic criteria for both IPF/CFAand idiopathic interstitial pneumonias confining the term IPF/CFA to patients with a histological pattern of usual interstitial pneumonia on lung biopsy. This review attempts to refine the clinico-radiological-pathological features that together define IPF/CFA as it is understood today, and to summarize the rationale of new therapeutic approaches based on the current understanding of the pathogenetic mechanisms.

Humans↗
PubMed2004

Fibrosing cholestatic hepatitis in renal transplant recipient with CMV infection: a case report.

Fibrosing cholestatic hepatitis (FCH) is an uncommon complication of renal transplantation. It is usually associated with hepatitis B and C viral infection. It is further rare in renal transplantation in absence of HBV and HCV infection. To the best of our knowledge, only three cases of FCH in renal transplantation, which were both HBV and HCV negative, have been reported to date. Out of these, two cases were diagnosed to have CMV infection and the third was attributed to azathioprin. We are presenting another case of FCH in a renal transplant recipient with CMV infection.

Adult↗
PubMed1979

Fibrosing alveolitis and hepatitis B surface antigen-associated chronic active hepatitis in a patient with immunoglobulin A deficiency.

Fibrosing alveolitis is described in a 22 year old woman with immunoglobulin A (IgA) deficiency and hepatitis B surface antigen (HBsAg)-associated chronic active hepatitis. At lung biopsy HBsAg was detected by indirect immunofluorescence in the alveolar space but not in the septal fibrosis. We discuss the possible relationships between IgA deficiency on the one hand, and HBsAg-associated lung and liver diseases on the other hand.

Adult↗
PubMed1986

A critical reappraisal of the fibrositis concept.

Physicians could use such terms as "fibrositis" and "the myofascial pain syndrome" simply to describe an easily recognized quality of illness. But the terms are chosen as part of a privileged vocabulary designed to assert a pathophysiologic insight(s). On careful consideration, these insights are tenuous at best. Furthermore, the terms, with their pathophysiologic insights, become labels that may perturb the patient's experience of disease in a fashion that is counterproductive.

Fibromyalgia↗
PubMed1986

Fibromyalgia (fibrositis) in children.

The features of fibromyalgia (fibrositis) in children are similar to those in adults with the syndrome. Both juveniles and adults report diffuse musculoskeletal aches and/or stiffness with typical modulating factors. Moreover, they have no systemic manifestations, and routine laboratory studies yield normal results. On physical examination in both children and adults, multiple soft-tissue tender points are evident at characteristic, symmetric sites but with no evidence of arthritis.

Adolescent↗
PubMed1988

Cryptogenic bilateral fibrosing pleuritis.

We describe four patients with bilateral pleural effusions progressing to diffuse pleural thickening for which we have been unable to find any evidence of an infective, embolic or occupational aetiology. In order to avoid confusion with diffuse pleural thickening attributable to asbestos-related disease, the term cryptogenic bilateral fibrosing pleuritis is suggested. The patients differed from those with pleural shadowing due to asbestos in that none of them gave a history of asbestos exposure, all were ill, presented with chest pain which was not always pleuritic in character, and had dyspnoea, cough or malaise. They had pleural effusions of variable size, pleural shadowing radiographically and raised sedimentation rates. Computed tomography revealed bilateral extensive pleural thickening in all cases. All four were HLA B44 positive. Histology showed that in all cases the pleura was thickened by fibrous tissue. Both layers were affected and the pleural space was often obliterated. Otherwise the pleural surface was covered by organizing fibrin. Focal collections of lymphocytes were often present when the fibrous tissue abutted on the subpleural fat. No asbestos bodies were seen in any of the cases and in one patient electron microscopic fibre counts showed no excess of asbestos. Pleural decortication was successful in three patients. In one of these, contralateral disease was successfully controlled with corticosteroids, but the fourth patient has not improved on corticosteroids.

Adult↗
PubMed1980

The relationship of alpha and delta EEG frequencies to pain and mood in 'fibrositis' patients treated with chlorpromazine and L-tryptophan.

Aspects of sleep stage evaluation and analysis of alpha and delta EEG frequencies in sleep were shown to be related to musculo-skeletal pain and mood disturbance in patients with 'fibrositis syndrome'. Patients were treated at bedtime for 3 weeks with either chlorpromazine, 100 mg (8 patients), or L-tryptophan, 5 g (7 patients). Chlorpromazine, but not L-tryptophan, was associated with increased slow wave sleep and amelioration of pain and mood symptoms. Mean percent time/min or mean percent power/min of alpha frequency during NREM and REM sleep corrlated with overnight increase in pain measures, hostility, and decrease in energy. On the other hand, mean percent time/min of delta in NREM sleep was related to overnight decrease in pain and mean percent delta power/min was associated with decreased anxiety and hostility, and increased energy.

Adult↗
PubMed1991

Alpha-like EEG activity in non-REM sleep and the fibromyalgia (fibrositis) syndrome.

The occurrence and characteristics of alpha-like activity during non-REM (NREM) sleep were examined in 11 subjects suffering from non-inflammatory (non-rheumatoid) musculoskeletal pain--fibromyalgia ('fibrositis'), and in 15 symptom-free controls. Both groups claimed to be good sleepers. Mean percentage alpha-like activity in sleep stages 2, 3, 4 and for NREM as a whole were greatest for the fibromyalgia group, but not significantly different from those of the controls. Overlap in the distribution of NREM alpha-like activity in sleep between the two groups indicated that it is not directly related to musculoskeletal symptoms. Spectral analyses showed a frontal area prevalence of this (kappa?) activity in the fibromyalgia group.

Adult↗
PubMed1992

High-level expression of hepatitis B viral antigens in fibrosing cholestatic hepatitis.

The expression of hepatitis B viral antigens was quantified in liver tissue from four transplant recipients with fibrosing cholestatic hepatitis (FCH) and compared with five other transplant recipients who did not develop this syndrome and 30 patients with chronic hepatitis B virus (HBV) infection. As measured by radioimmunoassays, the liver tissue from patients with FCH had significantly greater amounts of both hepatitis B surface antigen (HBsAg) and nucleocapsid antigens than to transplant patients without this syndrome (P less than 0.01) or patients with chronic HBV infection (P less than 0.001). Intrahepatic expression of pre-S1/pre-S2 in FCH was also extensive with a distribution parallel to that of HBsAg. High-level expression of intrahepatic HBsAg and hepatitis B core antigen in the explanted liver was associated with subsequent development of FCH in the liver graft, suggesting that viral/host factors may also be important. This pattern of intrahepatic hepatitis viral antigen expression, by analogy with Chisari's transgenic mice model and Roingeard's HBV-transfected HepG2 cell model, may be the cause of direct hepatocytopathic injury in this condition.

Adult↗
PubMed1993

Fibrosing cholestatic hepatitis in a transplant recipient with hepatitis B virus precore mutant.

A patient with hepatitis B virus (HBV) precore mutant (seropositive for hepatitis B surface antigen [HBsAg], anti-hepatitis B e antigen [HBeAg], and HBV DNA) who underwent orthotopic liver transplantation for end-stage liver disease is described. Sequencing of the HBV precore region of the pretransplant serum sample confirmed the presence of the precore stop-codon mutant (G-->A mutation in codon 1896) only. The patient received HBV immunoglobulin prophylaxis for 6 months but HBV recurred thereafter with a mild hepatitic flare, and he remained seropositive for HBsAg, anti-HBe, and HBV DNA. The initial hepatitic illness resolved in 3 months. The patient remained well for another 16 months before presenting with fibrosing cholestatic hepatitis (FCH). During his entire initial hepatitic flare, quiescent period, and final FCH phase, he remained seropositive for HBsAg, anti-HBe, and HBV DNA. Moreover, sequencing of the serum HBV DNA in final FCH phase showed the presence of the identical HBV precore mutant. Immunohistochemical staining showed extensive expression of HBsAg/pre-S1, pre-S2, and hepatitis B core antigen, but HBeAg was scarcely detectable. This case illustrates that (1) recurrence of HBV precore mutant infection can occur in liver; (2) it can give rise to FCH; and (3) hepatic accumulation of HBeAg is not essential for the development of FCH.

Adult↗
PubMed1995

Acute neutrophilic myositis in Sweet's syndrome: late phase transformation into fibrosing myositis and panniculitis.

Early in the course of myeloblastic leukemia a patient concurrently developed febrile neutrophilic dermatosis and sterile acute myositis. The dermatitis and myositis were unresponsive to antibiotic therapy but remitted within a few days of institution of steroid treatment. The patient died of myocardial infarction. At autopsy the dermis was normal. Previously effected muscles were scarred. The overlying fascia and subcutaneous septa were fibrotically thickened. In addition, segmental acute aortitis was detected. Acute myositis and aortitis may reflect further organ manifestations of the Sweet's reactivity pattern. It is proposed that Sweet's myositis and dermatitis may evolve into a fibrosing myositis and panniculitis.

Acute Disease↗
PubMed1994

Uncommon evolution of fibrosing alveolitis in a hard metal grinder exposed to cobalt dusts.

This study reports a case of fibrosing alveolitis with uncommon evolution in a 27-year-old male non-smoker, working as a hard metal grinder for 10 years. Personal and occupational history, routine clinical and laboratory check-up, chest X-rays, lung function tests, fiberoptic bronchoscopy with bronchoalveolar lavage and open lung biopsy returned a diagnosis of hard metal lung fibrosis. The patient felt greatly improved after removal from the workplace, without any therapy. At present, he is complaining of only mild exertional dyspnea and shows an attenuation of his ventilatory defect. The radiological picture has clearly improved and the cellular pattern of bronchoalveolar lavage was normal.

Adult↗
PubMed1993

Expression of endothelin-1 in lungs of patients with cryptogenic fibrosing alveolitis.

The vasoconstrictor and mitogenic peptide endothelin-1 (ET-1) is believed to play a part in fibrosis and collagen production. We examined expression of ET-1 in lung tissue from 52 patients with interstitial lung fibrosis, of whom 45 had cryptogenic fibrosing alveolitis (CFA), 10 had CFA and concomitant pulmonary hypertension, and 7 had non-specific focal fibrosis. 17 normal unused donor lungs were studied as controls. Immunohistochemistry and in-situ hybridisation were done with polyclonal antisera to ET-1 and its precursor big ET-1, and complementary RNA probes for preproET-1. Normal lung tissue and that from patients with focal fibrosis expressed very little ET-1. By contrast, there was striking expression of ET-1 in lung tissue from patients with CFA. Immunostains for ET-1 and big ET-1 and expression of ET-1 mRNA were most prominent in airway epithelium and type II pneumocytes, particularly those lining areas of young granulation tissue. ET-1-like immunoreactivity and mRNA were also present in pulmonary vascular endothelial cells, particularly in specimens from patients with pulmonary hypertension. In all patients, there was a significant correlation between ET-1-like immunoreactivity and histological parameters of disease activity (r = 0.78, 95% CI 0.65-0.87, p < 0.001). These findings suggest a possible role for cell-specific expression of ET-1 in the pathogenesis of CFA and associated pulmonary hypertension.

Biomarkers↗