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PubMed2000

Expression of decorin, biglycan, and collagen type I in human renal fibrosing disease.

BACKGROUND: The extracellular matrix proteoglycans decorin and biglycan may have a pathogenic role in renal fibrosing disease via regulation of the activity of growth factors, such as transforming growth factor-beta, and effects on collagen type I fibrillogenesis. The expression of decorin and biglycan in human glomerular diseases characterized by mesangial sclerosis is unknown. METHODS: Decorin, biglycan, and collagen type I were localized immunohistochemically in human renal biopsy cases of amyloidosis (N = 18), diabetic nephropathy (N = 11), fibrillary glomerulonephritis (N = 5), immunotactoid glomerulopathy (N = 5), light-chain deposition disease (N = 4), idiopathic mesangial sclerosis (N = 4), and nephrosclerosis (N = 6), and in morphologically normal tissues obtained from tumor nephrectomies (N = 8). Decorin and biglycan mRNA synthesis was evaluated by in situ hybridization. RESULTS: Decorin and biglycan protein were not identified in normal glomeruli. Decorin accumulated in amyloid deposits, but not in deposits of fibrillary glomerulonephritis or immunotactoid glomerulopathy. Biglycan weakly accumulated in amyloid deposits, and both decorin and biglycan weakly stained mesangial nodules in cases of morphologically advanced light-chain deposition disease and diabetic nephropathy. In all analyzed cases, irrespective of the underlying disease, decorin and biglycan accumulated in glomeruli in areas of fibrous organization of the urinary space and in areas of tubulointerstitial fibrosis. Biglycan, but not decorin, accumulated in the neointima of arteriosclerotic blood vessels. Decorin and biglycan mRNA synthesis was detected at sites of proteoglycan accumulation in glomeruli, interstitium, and neointima. Collagen type I colocalized with decorin and biglycan deposits. CONCLUSIONS: Differences in extracellular matrix proteoglycan composition may be diagnostically useful in distinguishing morphologically similar diseases. Distinct patterns of proteoglycan expression may be related to modulation of specific growth factor activity in different glomerular diseases.

Amyloidosis↗
PubMed2003

Selective depletion of fibroblasts preserves morphology and the functional integrity of peritoneum in transgenic mice with peritoneal fibrosing syndrome.

BACKGROUND: A peritoneal fibrosing syndrome (PFS) can progressively reduce peritoneal ultrafiltration during chronic peritoneal dialysis in patients with renal failure. The pathogenesis of PFS is unclear and the role of peritoneal fibroblasts has not been evaluated experimentally. METHODS: We followed the fate of fibroblasts producing PFS in a mouse model using fibroblast-specific protein 1 (FSP1) as a marker. PFS was induced by daily peritoneal infusions of chlorhexidine gluconate (CHG) saline into transgenic mice expressing the thymidine kinase (Delta tk) gene under the control of the FSP1 promoter (FSP1.Delta tk mice). To demonstrate the role of fibroblasts in PFS, we treated these FSP1.Delta tk mice with a nucleoside analogue to induce DNA chain termination and fibroblast death. RESULTS: Mice receiving peritoneal infusions of CHG saline every other day for 2 weeks developed increasing numbers of FSP1+ fibroblasts in the subserosal layers of the visceral peritoneum. Mac-3+ monocytes (macrophages) subsequently accumulated over the next 2 weeks in association with increased deposition of type I collagen and increased endothelial vascularity (CD31+) in these subserosal tissues. Since these peritoneal fibroblasts expressed monocyte chemoattractant protein-1 (MCP-1), heat shock protein 47 (HSP47), and vascular endothelial growth factor (VEGF), we suspect they were partially responsible for macrophage recruitment, matrix production, and the neoangiogenesis in the subserosal tissue. Treatment of PFS in FSP1.Delta tk transgenic mice with a nucleoside analogue selectively reduced the numbers of peritoneal fibroblasts and attenuated the attendant changes in peritoneal histology. Rescuing the peritoneal membrane from chronic thickening and neoangiogenesis by reducing the number of fibroblasts also preserved ultrafiltration. CONCLUSION: Peritoneal fibroblasts play a pivotal role in PFS, and their deletion using a fibroblasts-specific transgene was effective in preventing peritoneal fibrogenesis.

Animals↗
PubMed2001

Fibrosing cholestatic hepatitis-like syndrome in hepatitis B virus-negative and hepatitis C virus-negative renal transplant recipients.

Cholestatic hepatitis and diffuse liver fibrosis have been described in immunosuppressed patients with hepatitis B virus or hepatitis C virus infection as fibrosing cholestatic hepatitis (FCH). FCH is characterized by cholestasis, with only a modest increase in aminotransferase levels. The pathologic picture typically shows periportal and perisinusoidal fibrosis, scarce mixed infiltrates, hepatocellular ballooning, and histologic cholestasis. We report two patients with diffuse fibrosis and cholestasis quite similar to the histologic picture of FCH, but in whom neither hepatitis B virus nor hepatitis C virus infection could be shown, highlighting the potential contribution of cytomegalovirus infection and azathioprine toxicity in the development of this severe complication of solid-organ transplantation.

Adult↗
PubMedDate not supplied

Desquamative form of cryptogenic fibrosing alveolitis in a cat.

Bronchopulmonary disease is not uncommon in cats, many cases falling into the categories of chronic bronchitis and the "feline asthma syndrome". We report a case of chronic bronchopulmonary disease in an adult cat, which was initially diagnosed as chronic bronchitis. Failure to respond to appropriate therapy led to euthanasia. At necropsy, the lungs exhibited multifocal areas of consolidation, especially at the periphery of the diaphragmatic lobes. Histopathological examination revealed a striking variability of lesions, with interstitial fibrosis and intra-alveolar accumulations of macrophages in addition to alveolar epithelialization and smooth muscle hyperplasia. These changes were consistent with those described for the desquamative form of cryptogenic fibrosing alveolitis in human beings.

Animals↗
PubMed2003

Incidence and prevalence of cryptogenic fibrosing alveolitis in a Norwegian community.

This study assesses the incidence and prevalence of cryptogenic fibrosing alveolitis (CFA) in a well-defined and stable Norwegian population of 250,000 inhabitants during a period of 15 years. We conducted a file survey of all patients (n = 376) aged 16 years or older with a clinician's diagnosis of pulmonary fibrosis (ICD 8: 517 and ICD 9: 515 and 516). Cases with a history of exposure to fibrogenic agents or with collagen vascular disease were excluded and the remaining 158 cases were defined as CFA. The average annual incidence of hospitalised CFA was 4.3 per 100,000. No change was observed in the annual incidence during the 15-year study period. The prevalence was 19.7 and 23.9 per 100,000 by 31.12. 1991 and by 31.12. 1998, respectively. The incidence increased considerably and significantly with age. No significant gender differences were observed. The lack of gender differences and the increase with age were also found when the diagnosis of CFA was exclusively based on cases with hospital file records of breathlessness, bilateral crackles and bilateral shadows on chest radiograph.

Adolescent↗
PubMed2001

Idiopathic fibrosing pancreatitis presenting with obstructive jaundice in a child.

Idiopathic fibrosing pancreatitis (IFP) is a rare cause of obstructive jaundice in children. The obstruction is caused by circumferential compression of the intrapancreatic portion of the common bile duct (CBD). Various forms of biliary decompression, including surgical sphincteroplasty, choledochoduodenostomy, and Roux-en-Y choledochojejunostomy are employed as a treatment. We report a 14-year-old boy with IFP who was successfully treated by a temporary drainage of compressed CBD with the help of a stent placed by endoscopic retrograde cholangiopancreatography (ERCP). The diagnosis of IFP was confirmed histologically after open surgery. The patient remains well after 3.5 years of follow-up with no evidence of pancreatic insufficiency. This report offers an additional case of IFP causing obstructive jaundice treated by temporary internal drainage of CBD, which is safer and less invasive than biliodigestive surgical decompression.

Adolescent↗
PubMed1983

[Bird breeder's lung with conversion to fatal fibrosing alveolitis].

An 11-year-old girl developed typical symptoms of bird breeder's lung after several years of contact with budgerigars and other birds. The clinical diagnosis was confirmed by immunological and lung-function tests. Despite exclusion of exposure to allergens and treatment with steroids and D-penicillamine no improvement occurred, the exogenous allergic alveolitis progressing to fibrosing alveolitis of which the girl died.

Alveolitis, Extrinsic Allergic↗
PubMed2002

Azygous vein to right atrium bypass graft in a patient with idiopathic fibrosing mediastinitis and symptomatic superior and inferior vena cava obstructions.

We report an unusual case of a patient with diminished cardiac output caused by systemic venous occlusions of unclear etiology with a history of idiopathic fibrosing mediastinitis and retroperitoneal fibrosis. On thoracic, abdominal, and pelvic venograms, the patient was previously known to have superior vena caval and infrarenal inferior vena caval occlusions. Subsequent studies revealed retrograde flow through the azygous and hemiazygous veins and numerous caval-portal anastomoses providing venous return from the upper and lower extremities. After an extensive hypercoagulable state work-up, no abnormalities were found. To relieve the symptoms, the patient underwent an elective right thoracotomy with azygous vein to right atrial bypass graft. The patient has had complete amelioration of the disabling symptoms and participates in many activities that he could not tolerate previously. Because this condition and procedure are unreported, we present a novel surgical treatment option to palliate symptoms caused by concurrent superior and inferior caval obstructions by improving venous return to the heart.

Adult↗
PubMed2003

Nephrogenic fibrosing dermopathy: two pediatric cases.

We report two pediatric cases of nephrogenic fibrosing dermopathy (NFD), first described in 2000. NFD is a condition in which individuals with renal dysfunction have development of extensive skin hardening and histopathologic evidence of a scleromyxedema-like condition.

Adolescent↗
PubMed1982

Fibrosing alveolitis. Causes, characteristics, and consequences.

Fibrosing alveolitis may be cryptogenic (idiopathic) or secondary to a wide variety of disease processes. In almost all cases, acute and subacute inflammatory lesions progress to fibrosis and finally to a terminal state called honeycomb lung. Diagnosis is based on clinical and radiologic findings together with demonstration of typical pathologic changes on lung biopsy. The biopsy specimen should be large and taken from less severely involved lung areas to increase the probability that early, more specific lesions will be harvested. Prednisone is usually recommended for specific therapy. Therapy is a factor in duration of survival, although patient response varies.

Biopsy↗
PubMed1988

Four related fibrosing diseases. When you find one, look for another.

Peyronie's disease, Dupuytren's contracture, aponeurotic plantar fibrosis, and knuckle pads are fibrosing diseases with many common features. Recognition of these disorders and their interrelationships will result in rewarding discoveries and, quite possibly, in earlier treatment when disease is more responsive.

Child↗
PubMed1992

Special concerns in Lyme disease. Seropositivity with vague symptoms and development of fibrositis.

Fear of Lyme disease may be as powerful as the disease itself. Patients may insist on being tested for infection although little evidence of it exists, and a positive result in the face of vague symptoms can add to the problem. Physicians should explain to these patients the differences in "background" seropositivity in various geographic locations and the drawbacks of instituting unnecessary treatment. Fibrositis may evolve over time after Lyme disease infection. Many factors may trigger this disorder, but some investigators propose that it is a result of musculoskeletal pain, sleep disturbance, and anxiety over the disease.

Antibodies, Bacterial↗
PubMed1999

Fibrosing alveolitis predating microscopic polyangiitis.

A 65 year old male was diagnosed with "cryptogenic fibrosing alveolitis (CFA)" and treated successfully with Prednisone. In the year following Prednisone-tapering he presented with livedo reticularis, segmental pauci-immune glomerulonephritis and necrotizing vasculitis of the peripheral nerves, increased pulmonary fibrosis, and the presence of p-ANCA antibodies. Aggressive immunosuppressive treatment of this microscopic polyangiitis (MPA) was successful and also resulted in stabilization of the pulmonary fibrosis. This case illustrates that MPA may present itself monosymptomatic as CFA.

Aged↗
PubMed1991

Fibrositis (fibromyalgia syndrome) and the dental clinician.

The purpose of this article is to inform the general dentist treating the temporomandibular joint complex about fibrositis (fibromyalgia syndrome). Patients may present with spasms in the muscles of mastication, which may mimic joint pain or cause joint dysfunction. Tooth pain, which may mimic endodontic pain, may also be referred from a trigger pain in a muscle.

Adult↗
PubMed1993

Transverse myelitis as the presentation of Jo-1 antibody syndrome (myositis and fibrosing alveolitis) in long-standing ulcerative colitis.

A 32-yr-old woman with long-standing ulcerative colitis developed paraesthesiae, sensory loss and muscle weakness shown by magnetic resonance scan to be due to transverse myelitis in the cervical spinal cord. More than 12 months later, she developed dermatomyositis and fibrosing alveolitis and was found to have the Jo-1 antibody. Her condition worsened despite high dose oral prednisolone but improved following intravenous methylprednisolone, immunosuppressive therapy and plasma exchange. This is the first reported case of a neurological presentation of the Jo-1 antibody syndrome. We speculate that the association with ulcerative colitis may have been causal rather than coincidental and suggest that plasma exchange should be considered together with aggressive immunosuppression in seriously ill patients with this disease.

Adult↗
PubMed2001

Interstitial lung disease in patients with rheumatoid arthritis: a comparison with cryptogenic fibrosing alveolitis.

OBJECTIVES: There is a lack of information on the natural history of patients with rheumatoid arthritis (RA) and associated interstitial lung disease (ILD). However, cryptogenic fibrosing alveolitis (CFA) is known to have a poor long-term prognosis. As part of a longitudinal prospective study, we compared baseline characteristics in 18 patients with RA-ILD and 18 patients with CFA matched for age, sex and symptoms. We wished to establish whether there were significant baseline differences in clinical, physiological or radiological parameters. METHODS: A diagnosis of ILD was confirmed by high-resolution computed tomography (HRCT) and supported by clinical and physiological findings in all patients. A number of clinical, immunological, physiological and radiological parameters were compared between the two groups. The median age in each group was 77 yr and 10 patients in each group were male. RESULTS: Twelve of the RA patients had smoked in excess of 10 pack yr as compared with nine patients with CFA (not significant). Clubbing was found in five patients with RA-ILD and in 14 with CFA (P=0.008). Pulmonary function tests showed no significant differences between the groups in forced expiratory volume in 1s, vital capacity or gas transfer factor. HRCT showed more ground glass shadowing and peripheral disease in RA patients, but more established basal disease in those with CFA. Additionally, HRCT evidence of honeycombing was associated with an absence of rheumatoid factor and a low gas transfer factor. CONCLUSIONS: Clubbing is more common in patients with CFA, while RA-ILD patients have a higher prevalence of rheumatoid factor. Together with the differences in baseline HRCT, these variables in two groups of patients with similar physiological impairment at baseline may be important predictors of outcome in the longer term.

Aged↗
PubMed1997

Localized chronic fibrosing vasculitis of the skin: an inflammatory reaction that occurs in settings other than erythema elevatum diutinum and granuloma faciale.

Erythema elevatum diutinum (EED) and granuloma faciale (GF) are chronic, localized forms of cutaneous leukocytoclastic vasculitis that result in patterned (storiform or concentric) fibrosis. EED often occurs in systemically ill patients as bilaterally symmetrical plaques, papules, or nodules, often over the dorsa of joints. GF occurs as one or a few plaques on the face. Eosinophils and plasma cells are prominent in GF, whereas neutrophils are plentiful in EED. Rarely, extrafacial lesions accompany facial ones in GF, and there are a few reports of upper respiratory tract masses with GF-like histologic features. We report on eight patients with solitary cutaneous lesions with histologic features similar to those of EED or GF, but whose clinical picture was not that of either disease. One, whose histology resembled GF, had a large multinodular dermal and subcutaneous mass that persisted despite attempted resection. Unusual histologic findings in other cases included storiform fibrosis with dense infiltrates of plasma cells, branching nerve fascicles admixed with EED-like changes, and EED-like areas adjacent to zones mimicking a sclerotic fibroma. Chronic fibrosing venulitis can be seen outside the stereotypic settings of GF and EED and is an inflammatory reaction pattern that does not signify a specific diagnosis. Because of transitions between EED or GF-like areas and those of patterned sclerosis with plasma cell-rich infiltrates, we believe that some "inflammatory pseudotumors" of the skin, and perhaps of other sites could be the result of localized vasculitis.

Adult↗
PubMed1995

Frequent occurrence of c-Ki-ras gene mutations in well differentiated endometrial adenocarcinoma showing infiltrative local growth with fibrosing stromal response.

The frequency of c-Ki-ras mutation was examined by polymerase chain reaction (single-strand conformation polymorphism and direct sequencing analysis) and its association with histological parameters was analyzed in 110 endometrial adenocarcinoma hysterectomy specimens. The c-Ki-ras mutation was detected in 24 cases (22%). It occurred irrespective of tumor size, stage or histological grade, and was more frequent in tumors associated with endometrial hyperplasia (11 of 20, 55%) than in those without (10 of 73, 14%). In addition, the frequency of the mutation was significantly higher in tumors showing an infiltrative growth pattern accompanied by a stromal response consisting of edematous fibrous tissue (19 of 58, 33%), than in those which revealed an expansive growth pattern without such a stromal reaction (five of 52, 10%). Even among tumors showing the infiltrative growth pattern, the frequency of c-Ki-ras mutation was especially high (48%) in grade I cases in comparison with grade II or III cases (20% and 19%). Mutational activation of the c-Ki-ras gene may act at two points in the developmental pathway of endometrial adenocarcinoma by enhancing evolution of hyperplasia to adenocarcinoma, and by producing an infiltrative growth with a fibrosing stromal response.

Adenocarcinoma↗