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At least 379 records · Page 21Linked to original sources
PubMed1977

alpha 1-antitrypsin phenotypes in fibrosing alveolitis and rheumatoid arthritis.

alpha 1-antitrypsin (alpha 1-A.T.) phenotypes were determined in 55 patients with rheumatoid arthritis (R.A.), 33 patients with R.A. and either obstructive airways disease or recurrent chest infections, 49 patients with fibrosing alveolitis (F.A.), 22 patients with R.A. and F.A., and 200 healthy controls. A highly significant increase in the frequency of MZ phenotype was found among patients with F.A., both with and without R.A. Patients with R.A. alone had a normal distribution of phenotypes. Inherited modification of immune function may predispose to F.A. Alternatively, lowered levels of alpha 1-A.T. associated with non-M phenotypes may predispose to tissue damage with subsequent fibrosis.

Alleles↗
PubMed1984

Cryptogenic fibrosing alveolitis and Epstein-Barr virus: an association?

13 patients with cryptogenic fibrosing alveolitis (CFA) and 12 with interstitial lung disease (ILD) of known cause were studied for their humoral response to herpes simplex virus (HSV), cytomegalovirus (CMV), and Epstein-Barr virus (EBV). Serum antibodies to HSV and CMV were within the normal range in all patients. 10 patients with CFA had raised serum antibodies to EBV, and IgA against viral-capsid antigen (VCA) was detectable in all 13. In the other 12 patients EBV serological profiles were normal and IgA against VCA was detectable in only 1 patient. The EBV antibody levels did not correlate with the level of circulating immune complexes, the presence of rheumatoid factors, or the cytological findings of the alveolitis. The presence of IgG against VCA in 5 CFA patients suggests local production of EBV-specific immunoglobulins. Elevated IgG and IgA against EBV in CFA may indicate non-specific depression of cell-mediated immunity or that EBV plays a part in the aetiology of CFA.

Adult↗
PubMed1996

Occupational exposure to metal or wood dust and aetiology of cryptogenic fibrosing alveolitis.

BACKGROUND: We have previously suggested that cryptogenic fibrosing alveolitis (CFA) may be caused by occupational exposures, particularly to metal or wood dust. We have specifically investigated this hypothesis in a case-control study of patients with CFA. METHODS: We obtained lifetime occupational histories by postal questionnaire from 218 patients with CFA and 569 controls matched for age, sex, and community, living in the Trent region of the UK. Information was subsequently verified by telephone interview in 165 cases and 408 controls. Serum IgE, rheumatoid factor, and antinuclear antibodies and skin sensitivity to common allergens were measured in cases and in one matched control for each. FINDINGS: The relative risk of CFA, after adjustment for smoking, was significantly increased in relation to questionnaire-reported exposure to metal dust (odds ratio 1.68 [95% CI 1.07-2.65], p = 0.024) or to wood dust (1.71 [1.01-2.92], p = 0.048). Similar results were obtained with the telephone interview data. Significant exposure-response effects were found for both metal-dust and wood-dust exposure. CFA was also associated with the presence of rheumatoid factor or antinuclear antibodies, but not with positive allergen skin tests or raised IgE concentrations. There was no evidence of interaction between the effects of rheumatoid factor, antinuclear antibodies, positive skin allergen tests, or IgE concentrations and exposure to metal or wood dust. The combined aetiological fraction attributable to exposure to metal or wood dust was of the order of 20%. INTERPRETATION: Occupational exposures to metal or wood dust are independent risk factors for CFA. Avoidance or limitation of these exposures may provide an opportunity to prevent the disease.

Autoantibodies↗
PubMed2003

Pentasomy 8q in therapy-related myelodysplastic syndrome due to cyclophosphamide therapy for fibrosing alveolitis.

Trisomy 8/8q is a common cytogenetic event in myelocytic malignancies, ranging from myelodysplastic syndrome (MDS) to acute myelocytic leukemia (AML) to blastic transformation of chronic myelocytic leukemia. Isochromosome 8q results in the same gene dosage effect. Duplication of i(8q), resulting in pentasomy 8q, has been reported only in two cases of AML. A patient with fibrosing alveolitis on prolonged cyclophosphamide treatment developed therapy-related MDS. Karyotyping, FISH, and CGH analysis showed a duplicated i(8q) among other complex abnormalities. The clinical features of 11 cases of myelocytic leukemia with pentasomy and hexasomy 8/8q were summarized. Compared with trisomy and tetrasomy 8, significant features included reduced median survival (90 days), treatment refractoriness (even with transplantation), monocytic differentiation, trilineage dysplasia, and radiation or toxin exposure. Increasing copy numbers of chromosome 8/8q may therefore be a marker of advanced leukemic evolution, exposure to toxins, underlying myelodysplasia, and an overall poor prognosis.

Aged↗
PubMed2000

Rapidly progressive fibrosing interstitial nephritis associated with Chinese herbal drugs.

Rapidly progressive fibrosing interstitial nephritis after a slimming regimen containing aristolochic acid has been identified as Chinese herbs nephropathy (CHNP). From 1995 to 1998, we observed 12 Chinese people from different areas of Taiwan who underwent renal biopsy for unexplained renal failure. Medical history gave no clue to the causes of impaired renal function except for the ingestion of traditional Chinese herbs. Although these patients ingested herbal drugs from various sources for different purposes, their renal biopsy samples showed amazingly similar histological findings, with extensive hypocellular interstitial fibrosis and atrophy and loss of tubules in all cases. Glomeruli were apparently intact. They also had similar clinical features, such as normal or mildly elevated blood pressure, early and severe anemia, low-grade proteinuria, glycosuria, and insignificant urinary sediments. Renal function deteriorated rapidly in most patients despite discontinuation of the herbal medicines. Seven patients underwent dialysis, and the remainder experienced slowly progressive renal failure. Bladder carcinoma was found in one patient. Morphologically and clinically, the nephropathy in our patients was similar to CHNP, reported in Belgium. Because of the complexity and unknown types of herbs used in different clinical situations, unidentified phytotoxins other than aristolochic acid might be responsible for this unique disease entity. We conclude that the relation of this nephropathy to the consumption of Chinese herbs is striking. Using uncontrolled herbal remedies carries a high risk for developing interstitial renal fibrosis and urothelial malignancy.

Adult↗
PubMed2003

Fibrosing mediastinitis and thrombosis of superior vena cava associated with Behçet's disease.

We present CT, MRI and venography findings in 13-year boy with mediastinal fibrosis and superior vena cava (SVC) thrombosis associated with Behçet's disease. Fibrosing mediastinitis is an excessive fibrotic reaction that occurs in the mediastinum and may lead to compression of mediastinal structures (especially vascular). This condition is usually idiopathic, though many (and perhaps most) cases in the USA are thought to be caused by an abnormal immunologic response to Histoplasma capsulatum infection. SVC syndrome secondary to extrinsic compression by mediastinal fibrosis combined with Behçet's disease has rarely been described. Radiological investigations of this syndrome are necessary to avoid a useless anticoagulant therapy.

Adolescent↗
PubMed2000

Thoracic epidural anesthesia for modified radical mastectomy in a patient with cryptogenic fibrosing alveolitis: a case report.

A case of advanced cryptogenic fibrosing alveolitis (CFA) with multiple bullae and extensive pulmonary fibrosis, scheduled for modified radical mastectomy for carcinoma of breast, is presented. This patient had ischemic heart disease, corticosteroid-induced hypertension, diabetes mellitus, and a difficult airway. Thoracic epidural segmental anesthesia was successfully given to this patient. Preoperative problems, perioperative management, and alternative anesthetic techniques are discussed.

Anesthesia, Epidural↗
PubMed1989

Advanced cryptogenic fibrosing alveolitis: preliminary report on treatment with cyclosporin A.

Seven patients with fibrosing alveolitis who failed to respond to prednisolone and cyclophosphamide and were deteriorating, were treated with cyclosporin A. An initial favourable response was seen but was not maintained. When compared to a control population, matched for the extremely severe disease present in the treated group, survival was doubled from 2.5 to 5 months. With growing awareness of the possibility of heart-lung transplantation in this disease, even this trivial increase in survival may be of crucial importance in individual patients.

Cyclosporins↗
PubMed1999

Hepatitis C virus-related fibrosing cholestatic hepatitis after cardiac transplantation: is azathioprine a contributory factor?

We report a patient who acquired hepatitis C virus (HCV) infection at cardiac transplantation, developing fibrosing cholestatic hepatitis (FCH) with early liver failure and a fatal outcome. FCH is a recently described clinicopathological entity characterized by a cholestatic pattern of serum liver enzyme abnormalities, a progressive course leading to liver failure, and a pathological picture defined by periportal fibrosis, neutrophilic infiltrates and signs of histological cholestasis. Although it was initially described secondary to hepatitis B virus infection, it has also been recently related to HCV infection. Some histopathological features consistent with azathioprine hepatotoxicity like cholestasis, perisinusoidal fibrosis, veno-subocclusive lesions and nodular regenerative hyperplasia were also observed in this case. Therefore, a direct cytopathic effect of HCV and the concurrent pathogenic role of azathioprine hepatotoxicity may be involved in the development of this complication of transplantation.

Azathioprine↗
PubMed2005

Peritoneal fibrosing syndrome: pathogenetic mechanism and current therapeutic strategies.

Peritoneal dialysis (PD) has been established as a main renal replacement therapy for approximately 20 years. However, long-term peritoneal exposure to high glucose and other unphysiologic contents in the PD solution may potentiate the development of peritoneal fibrosing syndrome (PFS) in PD patients. PFS is composed of a wide spectrum of peritoneal alterations, which has been observed in PD patients. Molecular studies have shown that the fibrogenic effect of peritoneal mesothelial cells and the accompanying accumulation of extracellular matrix in the peritoneum are key events leading to PFS. In this review, we highlight the impact of PFS and its pathogenetic factors, including bioincompatible PD solution, multidisciplinary inflammatory mediators, and stimulatory cytokines in the peritoneal cavity. Current therapeutic strategies based on both clinical and basic evidence for the prevention or treatment of PFS are also reviewed.

Dipyridamole↗
PubMed1989

Combined treatment of large head and neck capillaro-venous malformation by a fibrosing agent.

We report our experience of seven cases suffering from capillaro-venous malformation localized in the head and neck area, treated preoperatively by a fibrosing agent--ETHIBLOC. Four of the cases are presented and analysed. We suggest the pre-operative injection of Ethibloc as the treatment of choice for capillaro-venous malformations in the head and neck region.

Adolescent↗
PubMed2002

Fatal reactivation of hepatitis B virus following cytotoxic chemotherapy for acute myelogenous leukemia: fibrosing cholestatic hepatitis.

Hepatitis B virus (HBV) is a well known pathogen that sometimes causes fulminant hepatitis in patients undergoing cytotoxic chemotherapy. Fibrosing cholestatic hepatitis (FCH) is a recently recognized unique variant of viral hepatitis, which has been occasionally reported in HBV-infected recipients of liver, renal, or bone marrow transplantation. We present here a 48-yr-old male in whom HBV was reactivated during post-remission chemotherapy for acute myelogenous leukemia, which resulted in rapidly fatal outcome. He manifested with deterioration of liver function in association with enormous replication of HBV. Liver biopsy showed marked ballooning of hepatocytes, cholestasis, and periportal fibrosis with minimum infiltrates. Immunostaining revealed that hepatocytes were strongly positive for hepatitis B surface antigen. Under the diagnosis of FCH, he was treated with lamivudine and interferon beta, which was not effective. Autopsy showed severe atrophy of the liver and marked degeneration of hepatocytes. Hematologists should be aware that FCH is a fatal complication that can develop under post-chemotherapy immunosuppressed conditions. Although there is no convincing evidence, prophylactic administration of lamivudine seems to be a reasonable strategy.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed2003

Postmenopausal frontal fibrosing alopecia.

Recently a new entity, postmenopausal frontal fibrosing alopecia, was added to the established subtypes of scarring alopecias affecting postmenopausal women. This condition is characterized by a progressive frontal hairline recession associated with scarring. We studied the clinical and histopathologic features in four women with this disorder. Of note, a history of bilateral oophorectomy in two of them appears to be a new association. All four cases had frontoparietal recession of the hairline and two of them also had loss of their eyebrows. None of our four patients had any mucous membrane or other skin lesions. Histological examination showed perifollicular fibrosis and lymphocytic inflammation around the isthmus and infundibular areas of the follicles. No effective treatments have emerged for this type of postmenopausal alopecia, but progression of the hair loss and scarring appears to be self-limiting. We believe that this condition is a distinct clinicopathological variant of lichen planopilaris.

Adult↗
PubMed2003

Post-menopausal frontal fibrosing alopecia.

Post-menopausal frontal fibrosing alopecia (PFFA) has become an increasingly recognized distinct clinical entity in recent years. Most cases have been reported from Australia; however, it seems likely that the condition is under-recognized. PFFA has similarities to lichen planopilaris but is differentiated by a distinctive symmetrical fronto-temporal distribution and progressive course. We report two cases from the UK and review the literature.

Aged↗
PubMed2004

[Two cases of frontal fibrosing alopecia in postmenopausal women].

Frontal fibrosing alopecia (FFA) is an uncommon, slowly progressive, cicatricial alopecia which mainly affects postmenopausal women. It is considered to be a variant of lichen planopilaris. We describe two postmenopausal women who developed over 11 and 24 months an asymptomatic atrophic alopecia, restricted to the frontal hairline. The diagnosis of FFA was confirmed by biopsy showing a perifollicular lymphocytic infiltrate with fibrosis. Topical corticosteroids, in one case combined with minoxidil, administered for 3 months arrested the hair loss. The treatment of FFA is often difficult. In most cases, the disease resolves spontaneously after several years. Immunomodulators such as corticosteroids and calcineurin antagonists should be tried in the early stage of FFA (frontal effluvium with perifollicular erythema) in order to arrest the disease in its inflammatory phase.

Administration, Topical↗
PubMed2002

Four cases of hepatitis B virus-related fibrosing cholestatic hepatitis treated with lamivudine.

Fibrosing cholestatic hepatitis (FCH) is a rare and extremely severe form of hepatitis B virus (HBV) infection. This condition was originally described in HBV-infected recipients after a liver transplantation. Recently, FCH has been reported not only in liver transplant recipients, but also in other immunosuppressed patients. It is characterized clinically by cholestatic hepatic dysfunction, and pathologically by severe periportal fibrosis, cholestasis, widespread balloon degeneration of hepatocytes, and only a mild infiltration of inflammatory cells. Without treatment, FCH is universally fatal within a few months of diagnosis. There have been only two isolated case reports of FCH with long-term patient survival, and one case report with treatment failure after lamivudine therapy. Because of the rarity of this clinical entity, the therapeutic efficacy of lamivudine in patients with FCH cannot be evaluated systematically. Here, we present four patients with HBV-related FCH treated with lamivudine. One received antineoplastic therapy for acute lymphoblastic leukemia, and the other three were renal graft recipients. Two patients who developed FCH after a renal transplantation survived with an improvement in liver function and were followed up for 20 and 30 months, respectively, and were found to be in good health. However, the other two patients died of sepsis, possibly as a consequence of the immunosuppression with hepatic failure despite lamivudine treatment. Our experience suggests that lamivudine can alter the grave natural history of FCH.

Adult↗