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At least 433 records · Page 24Linked to original sources
PubMed2006

Frontal fibrosing alopecia versus lichen planopilaris: a clinicopathological study.

BACKGROUND: Frontal fibrosing alopecia (FFA) is an acquired scarring alopecia currently considered a clinical variant of lichen planopilaris (LPP). Our purpose was to examine the clinicopathological features of FFA. In addition, we investigated the similarities and differences between FFA and LPP. METHODS: Biopsies from the scalp lesions of eight patients with FFA and eight patients with LPP were microscopically analyzed. Two cases of FFA and four cases of LPP were studied using direct immunofluorescence. RESULTS: In spite of the completely different clinical characteristics of FFA and LPP patients, the histopathological findings for the two entities were similar. Common microscopic findings for both FFA and LPP included an inflammatory lymphocytic infiltrate involving the isthmus and infundibulum of the hair follicles, the presence of apoptotic cells in the external root sheath, and a concentric fibrosis surrounding the hair follicles that resulted in their destruction with subsequent scarring alopecia. Biopsies taken from FFA patients showed less follicular inflammation and more apoptotic cells than those from LPP patients. In some cases of LPP, the inflammatory infiltrate involved the interfollicular epidermis, a finding never present in our FFA cases. Direct immunofluorescence was negative in the two cases of FFA studied and showed deposits of immunoglobulins and/or complement in two of the four LPP cases examined. CONCLUSIONS: The characteristic findings for FFA were more prominent apoptosis and less inflammation than found in LPP, along with spared interfollicular epidermis. FFA cases showed a rather characteristic histopathological pattern, although we could not find any clear-cut histological differences between FFA and LPP.

Aged↗
PubMed1998

Fibrosing cholestatic hepatitis after living related-donor renal transplantation.

A 43-year-old man underwent living related-donor renal transplantation because of chronic renal failure in 1991. During the transplant period, both donor and recipient were seronegative for hepatitis B surface antigen (HBsAg). The donor was seropositive for antibody to hepatitis B surface antigen (anti-HBs) due to hepatitis B virus (HBV) vaccination. After transplantation, FK506 and methylprednisolone had been administered to the patient as immunosuppressants. In 1993, HBsAg appeared in his serum. His alanine aminotransferase level elevated gradually during 1995 and then in 1996, general fatigue, ascites and jaundice developed. At this time his serum was positive for hepatitis B e antibody, contained more than 100000 Meq/mL HBV-DNA and 100% precore mutant. Despite subsequent intensive therapy, liver dysfunction progressed and this patient died of hepatic failure 2 months following admission. At autopsy, the liver exhibited cholestasis, fibrosis extending from the portal tracts, mild inflammation and hepatocytes with a ground-glass appearance. In addition, HBsAg and hepatitis B core antigens had accumulated in the hepatocytes. Consequently, the final diagnosis was fibrosing cholestatic hepatitis (FCH) due to precore mutant HBV infection contracted after renal transplantation. It is unclear when and where the recipient liver became HBV infected. Nevertheless, after renal transplantation, while receiving immunosuppressive drugs, HBV appeared to have the potential to cause hepatic failure and FCH may have been a fatal complication for the recipient.

Adult↗
PubMed1984

Synchronous bilateral double primary lung cancer associated with diffuse interstitial fibrosing pneumonitis (DIFP).

Double primary lung cancer with diffuse interstitial fibrosing pneumonitis (DIFP) was detected in a 61 year-old man at autopsy. Histologically, alveolar septa in whole lobes of right and left lungs were thickened diffusely with fibrosis, edema, and inflammatory cell infiltration. Alveolar cavities contained fibrinous exudate. A tumor mass measuring 8 X 5 X 5 cm occupying the right hilar portion was epidermoid carcinoma involving the right superior lobar bronchus and hilar lymph nodes. There were no metastatic foci in the right middle and lower lobes. In the lower lobe of the left lung, scattered foci of bronchiolo-alveolar carcinoma and those of epidermoid carcinoma coexisted. There were no metastatic foci in the left upper, lower lobes, and hilar lymph nodes. Both atypical bronchiolar epithelial proliferation associated with bronchiolo-alveolar carcinoma and squamous metaplasia associated with epidermoid carcinoma were detected at the same time. The clinical and pathological characteristics among 81 reported cases of lung cancer associated with DIFP are reviewed.

Adenocarcinoma, Bronchiolo-Alveolar↗
PubMed1996

Cryptogenic fibrosing alveolitis: have we made any progress?

The interstitial lung diseases (ILD) are a heterogeneous group of disorders the most common of which is cryptogenic fibrosing alveolitis (CFA). This article has summarized recent work in particular on the diagnosis, pathogenesis and treatment of CFA, by reviewing published data accessed through Medline searching. Recent reports suggest a higher prevalence of CFA than previously documented (13.2-20.2/100,000 population) and a rising mortality rate. The prognosis is universally poor with 50% of patients dying within 5 years. Although approximately 30% of patients may live for long periods (> 10 years), morbidity is significant and quality of life in the long-term survivors is poor. Diagnosis is traditionally based on an open lung biopsy, however, more recently the high resolution computer tomography (HRCT) is often used; however, its use without a tissue diagnosis remains controversial. In conclusion, we know substantially more about the pathogenesis of the disease and from this work have a number of possibilities for new therapeutic strategies that will hopefully reach the bedside in the near future. Additionally we have some new non-invasive tests that offer hope for stratifying patients but require further evaluation. For assessing both therapy and investigations we will need substantial groups of patients in multicentre studies to provide sufficient power to allow a conclusion to be reached. To ensure any further progress we must collaborate and enter our patients into such trials.

Adrenal Cortex Hormones↗
PubMed1990

Management of fibrosing alveolitis with polymyositis dermatomyositis.

A 38-year-old man with polymyositis-dermatomyositis developed acute life-threatening fibrosing alveolitis. We report a two year follow-up of this patient, during which control of alveolitis was obtained using prednisolone, and was maintained with the addition of cyclophosphamide. Relapse of myositis responded to increased prednisolone. Serial measurements of vital capacity and transfer factor assisted management of alveolitis, but changes in vital capacity preceded changes in transfer factor, and were more convenient to perform during frequent outpatient review.

Adult↗
PubMed2004

Fibrosing alveolitis in systemic sclerosis: the need for early screening and treatment.

Abnormalities in lung function occur in 70% of patients with systemic sclerosis (SSc). Fibrosing alveolitis in SSc (FASSc) is more commonly seen in the diffuse cutaneous form of SSc, particularly in the presence of antitopoisomerase antibodies (Scl70), and with the decreasing incidence of scleroderma renal crisis it is now the major cause of mortality in this patient population. Screening of patients recently diagnosed with SSc by pulmonary function tests and the performance of high resolution computed tomography when physiological abnormalities are identified has resulted in the identification of significant numbers of patients with early, asymptomatic FASSc. Whether these patients should be further investigated with a surgical lung biopsy or receive immunosuppression is unclear, because it cannot yet be reliably predicted who will develop progressive disease and the evidence to support the efficacy of treatment is not strong. The objective of the present article was to review the evidence to support the use of immunosuppressive therapy in FASSc and, based on these data, to propose an algorithm for the investigation and management of this difficult clinical problem.

Bronchoalveolar Lavage Fluid↗
PubMedDate not supplied

Case study: fibrosing alopecia in a pattern distribution localized on alopecia androgenetica areas and unaffected scalp.

A 54-year-old man with a 24-year history of androgenetic alopecia was referred to the Department of Dermatological Sciences with follicular inflammatory lesions leading to scleroatrophy in the vertex region (Figure 1) of 1-year duration. These lesions appeared a year ago. There was no previous history of this condition. On examination, the patient showed confluent infiltrative follicular lesions on the frontoparietal and occipital scalp (Figure 2). Some lesions evolved into erosions that developed in ivory white scleroatrophy within weeks. These lesions were localized both in and outside of are as affected by alopecia androgenetica and were associated with mild pruritus. Histopathologic examination, performed on an early lesion of the vertex, documented a mild thinning of follicular epithelium associated with an intense lymphohistiocytic perifollicular infiltrate. The damage of the basal cell layer was limited to the follicle, while epidermis was intact. In particular, follicular keratinocytes under the isthmus showed a very intense degeneration exactly where the infiltrate was the most prominent. The damage of the hair sheath was under the isthmus and involved the lower portions of the follicles (including the hair bulbs). The inflammatory infiltrate was exclusively represented by perifollicular lymphohistiocytes. Finally, a connective fibrotic shell with numerous fibroblasts formed a sheath around the atrophic follicle (Figure 3). Results of laboratory investigations (including complete blood cell counts, basal thyroid-stimulating hormone, C-reactive protein, serum ferritin levels, B and C hepatitis markers, antinuclear antibodies, and cultural examinations) were negative.We diagnosed the patient with fibrosing alopecia in a pattern distribution.

Alopecia↗
PubMed1998

First adult patient with fibrosing colonopathy.

An adult patient with cystic fibrosis and chronic abdominal complaints developed symptomatic fibrosing colonopathy several months after the beginning of high dose pancreas enzyme therapy. Despite the absence of a stricture, surgical resection was performed. Since then the abdominal complaints have not recurred.

Abdominal Pain↗
PubMed2002

Rapidly progressive fibrosing cholestatic hepatitis--hepatitis C virus in HIV coinfection.

Fibrosing cholestatic hepatitis (FCH) is a severe and progressive form of liver dysfunction seen in organ transplant recipients infected with hepatitis B virus or hepatitis C virus (HCV) and has been attributed to cytopathic liver injury. To date, no case of FCH due to HCV has been reported in HIV-positive individuals. We describe two cases of HCV-induced FCH in two patients coinfected with HIV, culminating in rapidly progressive liver failure and death. Histological features and progression in both cases were not consistent with drug effect or obstruction. Late institution of interferon-based therapy was ultimately unsuccessful. The HCV RNA was not markedly elevated in these cases, suggesting that the cytopathic effect of HCV in these patients was not simply a consequence of viral load. FCH may in part explain the accelerated development of cirrhosis previously observed among coinfected patients. Clinicians should remain vigilant for FCH in the HIV/HCV population and consider antiviral treatment in this setting.

Adult↗
PubMed2006

Nephrogenic fibrosing dermopathy and calciphylaxis with pseudoxanthoma elasticum-like changes.

Nephrogenic fibrosing dermopathy (NFD) and calciphylaxis are rare conditions that are associated with chronic kidney disease. Histopathologic changes, including dystrophic dermal calcification, often in association with elastic fibers have been observed in NFD and calciphylaxis. A pattern of dermal elastic fiber calcification that mimics pseudoxanthoma elasticum (PXE) has been previously reported as an incidental finding in the setting of calciphylaxis. Despite a shared association with renal disease and abnormal calcium deposits, however, NFD and calciphylaxis are discrete pathologic processes with distinct clinical and histopathologic features. Criteria for each are reviewed through case presentation of a patient meeting the clinical and histopathologic criteria for both NFD and calciphylaxis with histologic features mimicking PXE.

Adult↗
PubMed1975

Desquamative fibrosing alveolitis unresponsive to steroid or cytotoxic therapy.

A case of desquamative fibrosing alveolitis beginning in early infancy is described. The disease was characterized by tachypnoea, hypoxia relieved by O2, absence of signs in the chest or clubbing, and radiological and physiological evidence of hyperinflation. The diagnosis was made by needle biopsy of the lung. Treatment with steroids and immunosuppression was without effect and the infant died at 9 months. The disease has a high mortality in infancy, only 2 out of 9 reported cases having survived. The difference from the usual course in adults and older children is noted.

Azathioprine↗
PubMed1996

Fibrosing colonopathy in cystic fibrosis.

The introduction of enteric coated pancreatic enzyme supplements in the early 1980s was undoubtedly one of the major advances in the care of children with cystic fibrosis. Further refinements in the presentation of these preparations inevitably followed, to improve patient acceptability and compliance. The emergence of fibrosing colonopathy took clinicians dealing with cystic fibrosis completely by surprise, and in the last two years there has been a gradual appreciation that as far as pancreatic enzyme products are concerned 'More is not necessarily better'. However, it is encouraging that, in the UK, there have been no histologically confirmed cases in children receiving high strength pancreatic enzyme preparations since July 1994. Hopefully this trend will continue and the causal factors will be defined, ensuring that this serious complication can be effectively prevented in the future.

Child↗
PubMed1987

Sjögren's syndrome and fibrosing alveolitis complicated by pulmonary lymphoma.

The case of a middle aged woman who presented with fibrosing alveolitis, in her mid-forties, followed by a sicca syndrome and who subsequently developed a pulmonary lymphoma (B cell) while receiving azathioprine therapy is recorded. Of interest was the absence of polyclonal B cell activation, e.g., production of rheumatoid factor, hypergammaglobulinaemia, high titre antinuclear antibodies or antibodies to extractable nuclear antigens (ENA) during most of her illness. Persistently raised IgM levels and low IgA levels were demonstrated. The relevance of azathioprine to development of the lymphoma is discussed.

Female↗
PubMed1999

Pulmonary involvement in diffuse cutaneous systemic sclerosis: broncheoalveolar fluid granulocytosis predicts progression of fibrosing alveolitis.

OBJECTIVE: The clinical course of fibrosing alveolitis (FA) in patients with systemic sclerosis (SSc) may vary considerably from stable condition for years to continuous fatal progression. This prospective study aimed at identifying the prognostic value of bronchoalveolar lavage fluid (BALF) analysis in FASSc. METHODS: Seventy three consecutive patients with SSc and clinical signs of pulmonary involvement were enrolled. Every patient underwent clinical examination, lung function tests, computed tomography (CT), gallium scan, echocardiography, and bronchoalveolar lavage (BAL). Forty nine patients, 26 with pathological and 23 with normal BALF findings were prospectively followed up for two years and re-evaluated annually. RESULTS: At baseline, 51 subjects (70%) showed radiological signs of lung fibrosis and/or alveolitis by CT and diffusion capacity for carbon monoxide (DLco) was decreased in 47 patients (64%). Thirty five patients (48%) had pathological BALF findings. BALF differential counts included BALF granulocytosis in 18, BALF lymphocytosis in 12, and a mixed increase of both granulocytes and lymphocytes in five patients. On follow up, a progression of FA with a significant decrease of DLco was only observed in patients with BALF granulocytosis. In contrast, patients with BALF lymphocytosis or normal BALF cell count had stable lung function parameters during the study period. In none of our patients echocardiography showed evidence of pulmonary hypertension. CONCLUSION: BALF granulocytosis predicts progression of FA with deterioration of lung function, which is most sensitively monitored by DLco. Immunosuppressive treatment is recommended in patients with granulocytic FASSc.

Bronchoalveolar Lavage Fluid↗
PubMed2002

Predictors of progression of HRCT diagnosed fibrosing alveolitis in patients with rheumatoid arthritis.

BACKGROUND: Fibrosing alveolitis (FA) is the most serious pleuropulmonary extra-articular feature of rheumatoid arthritis (RA). Features that predict progression of FA in patients with RA have not yet been determined. OBJECTIVE: To identify clinical features that predict progressive FA in patients with RA. METHODS: An unselected cohort of 29 patients with RA and FA confirmed by high resolution computed tomography (HRCT) were studied prospectively for 24 months. Three monthly clinical assessment, four monthly pulmonary function tests, and yearly HRCT scanning was undertaken on these patients. Progressive FA was defined as >15% fall in carbon monoxide transfer factor (TLCO) with evidence of increasing FA on HRCT or death as a result of FA. RESULTS: During 24 months of follow up 10/29 (34%) patients had progressive FA. Progression on HRCT was seen as acute ground glass exacerbations or increasing reticular pattern lung involvement. Progressive FA was associated with the presence of bibasal crackles (p=0.041), TLCO (p=0.001), and extent (p=0.026) and distribution (p=0.031) of lung involvement on HRCT at initial presentation. When multiple logistic regression was used, only TLCO remained significant. Receiver operator curve analysis was employed to identify presenting TLCO of progressive FA. A TLCO <54% of the predicted value demonstrated 80% sensitivity and 93% specificity in predicting progressive FA. CONCLUSIONS: A TLCO <54% of the predicted value is a highly specific predictor of disease progression.

Aged↗
PubMed1971

Lung transplantation in a patient with fibrosing alveolitis.

The transplantation of the right lung into a man aged 40 who was suffering from cryptogenic fibrosing alveolitis is described. Before transplantation he had been dependent on oxygen, even at rest, for 24 hours a day for almost two years. The donor was a boy of 16 years who had had a fatal cerebral haemorrhage. The transplanted lung functioned perfectly from the time of operation until the patient's sudden death two months later from an overwhelming haemoptysis apparently from a small peribronchial abscess rupturing into the pulmonary artery. By the third postoperative week the patient had been able to walk unaided and without distress outdoors. The problem of differentiating infection from incipient rejection is discussed. We conclude that clinically successful lung transplantation can be achieved, but only if the problems of lung function, infection, and immunosuppression can all be overcome.

Adult↗
PubMed1974

Fibrosing alveolitis and treatment with sulphasalazine.

A woman with ulcerative colitis was treated with sulphasalazine and prednisolone. After four months' treatment she began to get short of breath and she died a month later with severe subacute fibrosing alveolitis. It is believed that the lung disease was caused by sulphasalazine and that prednisolone was not able to prevent its development. A reversible form of lung disease has previously been attributed to this drug.

Autopsy↗