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PubMed1999

A case of fibrosing mediastinitis with obstruction of superior vena cava and downhill esophageal varices: a rare cause of upper gastrointestinal hemorrhage.

Fibrosing mediastinitis (FM) is an excessive fibrotic reaction that occurs in the mediastinum and may lead to compression of mediastinal structures (especially vascular or bronchial). In the present study we describe the first case report of FM, in a patient who developed downhill esophageal varices and bleeding, which was secondary to superior vena cava obstruction.

Administration, Oral↗
PubMed2002

Fibrosing colonopathy revealing cystic fibrosis in a neonate before any pancreatic enzyme supplementation.

Although its pathogenesis remains still unknown, fibrosing colonopathy (FCP) is considered to be the result of prolonged treatment by high doses of pancreatic enzyme preparations, in a small proportion of patients who present with cystic fibrosis (CF). We present the case of a newborn with meconium ileus (treated by conservative measures), in which, at the age of 3 weeks, the features of intestinal obstruction made necessary the removal of 15 cm of the proximal large intestine. Macroscopical and especially microscopical appearances typical for FCP were found, despite the absence of any enzymatic treatment. These findings raised the suspicion of CF, which was confirmed 4 weeks later at necropsy by the presence of characteristic pancreatic lesions. This case and another similar report in the literature suggest that the mechanism of FCP must be linked with the disease itself, at least in some patients. Thus, for us, FCP is not a "closed subject" and we sustain the importance of continuing studies, which will shed light on its etiopathogenesis.

Colon↗
PubMed1991

Superior vena cava obstruction in fibrosing mediastinitis: demonstration of right-to-left shunt and venous collaterals.

Multiple venous collateral pathways have been described in patients with superior vena cava obstruction. Systemic venous-to-pulmonary venous communication is the most unusual, having been described in a few cases of thoracic malignancy. In a patient with fibrosing mediastinitis radionuclide venography with 99Tcm-macroaggregated albumin demonstrated a systemic venous-pulmonary venous right-to-left shunt in addition to systemic and portal venous collaterals. It is apparent that systemic venous-to-pulmonary venous anastomoses may occur in the absence of malignant disease.

Adult↗
PubMed2002

Fibrosing mediastinitis secondary to zygomycosis in a twenty-two-month-old child.

We report the case of a 22-month-old immunocompetent male child with fibrosing mediastinitis secondary to zygomycosis, an unusual presentation of a rare fungal infection. This patient was successfully treated with amphotericin B and itraconazole for 20 weeks. Stenting of the superior vena cava was helpful in relieving the patient's superior vena cava syndrome.

Amphotericin B↗
PubMedDate not supplied

Sleep-related myoclonus in rheumatic pain modulation disorder (fibrositis syndrome) and in excessive daytime somnolence.

We describe a new syndrome, Rheumatic Pain Modulation Disorder (RPMD) ("fibrositis syndrome") with sleep-related myoclonus (involuntary periodic leg movements). Measures of sleepiness, fatigue and pain, before and after sleep, and aspects of sleep of nine subjects (Ss) with RPMD and sleep-related myoclonus were compared to nine subjects with excessive daytime somnolence and sleep-related myoclonus. In eight of the RPMD with sleep-related myoclonus and three of those with daytime sleepiness, an alpha (7.5-11 Hz) EEG Non-Rapid Eye Movement sleep disorder was demonstrated. The RPMD with sleep-related myoclonus group contained a greater number of women, more pain, morning fatigue, and disturbances in sleep (more stage changes and alpha EEG sleep prior to leg myoclonus); but in comparison to the sleep-related myoclonus, daytime somnolent group, there were no differences in evening and morning sleepiness, number of limb movements, movement arousals, awakenings after sleep onset, sleep duration, and percent sleep stages.

Adult↗
PubMedDate not supplied

Sleep and posttraumatic rheumatic pain modulation disorder (fibrositis syndrome).

The clinical features and sleep physiology of 11 female patients with "fibrositis syndrome" or rheumatic pain modulation disorder (RPMD) were compared with 11 female postaccident pain (PAP) patients who complained of widespread musculoskeletal pain, fatigue, and nonrestorative sleep following a nonphysically injurious motor vehicle or work-related accident. Both groups had similar musculoskeletal pain, fatigue, sleepiness and an alpha (7.5-11 Hz) EEG non-rapid eye movement (NREM) sleep anomaly. A psychophysiologic arousal mechanism during NREM sleep induced by the emotional stress of the accident may mediate the subsequent nonrestorative sleep, musculoskeletal pain, and fatigue symptoms in the posttraumatic pain disorder.

Adult↗
PubMed1997

Alteration of the dismal natural history of fibrosing cholestatic hepatitis secondary to hepatitis B virus with the use of lamivudine.

BACKGROUND: Fibrosing cholestatic hepatitis (FCH) is a severe form of hepatitis B virus (HBV) infection occurring as either primary allograft reinfection after liver transplantation for HBV or as severe HBV reactivation induced by immunosuppression in patients with previously latent infection. Without treatment, FCH is universally fatal within a few months of diagnosis. Some improvement has been reported with long-term ganciclovir, with and without foscarnet, but an effective and easily available treatment has not yet been reported. METHODS: We report the prolonged survival of a renal transplant recipient who developed histologically confirmed FCH 6 months after transplantation and was treated with lamivudine. RESULTS: At the time of diagnosis, the patient had jaundice, ascites, and a serum HBV-DNA level of 3868 pg/ml. Lamivudine was instituted 2 months later, and after 6 months of treatment, the HBV-DNA level was undetectable with the serum bilirubin within the normal range. Twelve months after the diagnosis of FCH, the patient remains stable without progression of liver dysfunction. CONCLUSION: Our experience demonstrates that lamivudine therapy can improve the dismal natural history of FCH.

Antiviral Agents↗
PubMed1998

Hepatitis C virus-associated fibrosing cholestatic hepatitis after renal transplantation: response to interferon-alpha therapy.

Fibrosing cholestatic hepatitis (FCH) has recently been described after solid organ transplantation in patients with hepatitis C virus (HCV) infection. Typically, FCH is characterized by an ominous clinical course leading to progressive hepatic failure and death if liver transplantation is not performed. Two HCV-infected patients underwent cadaveric renal transplantation for end-stage renal disease resulting from membranous nephropathy and diabetic nephropathy. The time intervals between transplantation and the biopsy diagnosis of FCH for the two patients were 7 months and 10 years. Both patients presented with jaundice, hyperbilirubinemia, and mild-to-moderate elevations in serum aspartate aminotransferase. One patient was also found to have type II mixed cryoglobulinemia. Interferon-alpha therapy was begun after a diagnosis of FCH was established by liver biopsy. Liver test abnormalities normalized rapidly. When cholestatic hepatic deterioration develops in an HCV-infected organ allograft recipient, the diagnosis of FCH should be considered and a liver biopsy performed. Our observations indicate that FCH can respond to antiviral therapy.

Aged↗
PubMed2006

Nephrogenic fibrosing dermopathy/nephrogenic systemic fibrosis in 2 patients with end-stage renal disease on hemodialysis.

Nephrogenic fibrosing dermopathy/nephrogenic systemic fibrosis (NFD/NSF) is a newly recognized disorder occurring in patients with renal failure. It is manifested by progressive cutaneous and systemic fibrosis involving the extremities and the trunk with usual sparing of the face and internal organs. NFD/NSF can lead to joint contractures and painful disability. The etiology is unknown and there are no proven effective treatments. NFD/NSF must be distinguished from other clinically similar disorders such as scleroderma, scleromyxedema, and eosinophilic fasciitis among a myriad of others. NFD/NSF can pose many diagnostic challenges to physicians; however, a team of experienced rheumatologists, nephrologists, dermatologists, and dermatopathologists is essential in identifying this disorder. We report 2 new cases of NFD/NSF in patients on hemodialysis.

Adult↗
PubMed2004

Calciphylaxis and metastatic calcification associated with nephrogenic fibrosing dermopathy.

BACKGROUND: Calciphylaxis and metastatic calcification are known complications of chronic renal failure. Recently, a sclerosing condition of the skin termed nephrogenic fibrosing dermopathy (NFD) has been described in patients with renal disease, many of whom have undergone hemodialysis and/or renal transplantation. To our knowledge, the simultaneous occurrence of both conditions in the same patient, in the same lesion, has not been previously reported. CASE REPORT: We report the clinical, microscopic, and immunohistochemical features of two patients with chronic renal failure whose lesional skin biopsies showed both subcutaneous calcification and NFD. We consider the possible mechanisms that might explain the coexistence of these two disorders. RESULTS: Both patients presented with erythematous, indurated skin over the lower extremities. Purpuric, reticulated patches, necrosis, or ulceration were not observed. Microscopic examination showed the characteristic changes of NFD involving dermis and subcutaneous septa. In addition, biopsies of both individuals showed subcutaneous calcification, one in a diffuse distribution and the other involving the walls of subcutaneous vessels, as seen in calciphylaxis. Calcification was not suspected clinically in either case. CONCLUSIONS: Metastatic calcification or calciphylaxis and NFD can occur simultaneously in patients with chronic renal failure and may be found together in the same lesion. Because subcutaneous calcification may not be suspected clinically in these cases, and in view of the adverse outcomes frequently associated with calciphylaxis, we recommend deep incisional biopsy of patients presenting with the clinical features of NFD. Both the fibrosis and the calcification of chronic renal failure may be related to the activity of transforming growth factor-beta/Smad signaling cascades.

Adult↗
PubMed2004

A variant of nephrogenic fibrosing dermopathy with osteoclast-like giant cells: a syndrome of dysregulated matrix remodeling?

Nephrogenic fibrosing dermopathy (NFD) is a disorder characterized by dramatic thickening and hardening of skin in the extremities and trunk, which occurs in individuals on dialysis for renal disease. The pathophysiology is unknown. Increased transforming growth factor-beta (TGF-beta) and collagen deposition have been reported in a small group of patients studied by Jimenez et al.1 We report two patients with NFD and osteoclast-like giant cells in the fibrotic dermis; one patient also had dystrophic cutaneous calcification. These findings have been seen in a small percentage of NFD patients (estimated 2-5%) and may represent a variant of the disease. The hypothesis of altered matrix dysregulation due to altered TGF-beta, metalloproteinases, and activation of osteoclasts as an explanation for this variant is proposed.

Adult↗
PubMed2004

Nephrogenic fibrosing dermopathy.

Nephrogenic fibrosing dermopathy (NFD) is an uncommon, recently described fibrotic skin disorder that has been observed in patients with end-stage renal disease. We describe a 81-year-old man with end-stage renal disease who developed NFD in the seventh year of haemodialysis. Laboratory investigations revealed considerably increased circulating immune complexes and anti double stranded DNA antibodies. Because the pathogenesis of NFD may not entirely be attributable to haemodialysis, the role of circulating factors in the development of cutaneous manifestations requires further investigation.

Aged↗
PubMed1993

Solitary fibrosing paraspinal plaque: solitary morphoea profunda?

Solitary morphoea profunda is a recently described morphological variant of localized scleroderma. Two of the five reported patients had a solitary fibrotic plaque in the paraspinal region. We report a 16-year-old boy with a solitary fibrotic paraspinal plaque which, on histological examination, showed dermal and subcutaneous sclerosis, with a polymorphous infiltrate including plasma cells and eosinophils. Laboratory tests were either negative or normal, except for mild peripheral blood eosinophilia. Antinuclear antibodies were not detected, and the patient had no evidence of Borrelia infection. Magnetic resonance imaging (MRI) showed that the process involved the subcutaneous tissue, but did not extend to the underlying bone. As half of the patients with this entity (three of six) now described have had a solitary fibrotic plaque in a paraspinal location, we suggest that it may be premature to classify all these cases as being a variant of morphoea. We propose the use of the descriptive term solitary fibrosing paraspinal plaque, until the aetiology or aetiologies of this condition are better understood.

Adolescent↗
PubMed1993

Cutaneous fibroses induced by Borrelia burgdorferi.

Three cases of chronic infection with Borrelia burgdorferi are described. The patients presented with nodular or discoid fibrosis, partly in conjunction with acrodermatitis chronica atrophicans (ACA). Juxta-articular fibrotic nodules may develop within a few months of the onset of ACA. Nodular, discoid morphoea-like, and widespread cutaneous fibroses in chronic Borrelia infection may be provoked by trauma, surgery or electromagnetic radiation. They respond well to antibiotic therapy. These lesions offer an in vivo model for studying the evolution of immunologically induced fibrosis.

Adult↗
PubMed1980

Skin tests with tuberculin (PPD) Candida albicans and Trichophyton spp. in cryptogenic fibrosing alveolitis and asbestos related lung disease.

Delayed skin hypersensitivity responses were elicited in patients with cryptogenic fibrosing alveolitis (CFA) (twenty-eight), asbestosis (eight), mesothelioma (eight) and a "control" group, having miscellaneous lung diseases not normally believed to be associated with T cell deficiency (twenty). Three antigens, Candida albicans, trichophyton and purified protein derivative (PPD) in a range of doses were used. There was no evidence of impaired cellular immunity in CFA or in mesothelioma, indeed there was a significantly increased frequency of reactions to PPD in both of these conditions (P less than 0.05 and P less than 0.01 respectively). There was, however, a trend of decreased responsiveness in the group with asbestosis. The dosage regimen used rarely gave completely negative results (only one of thirty-two completed tests), and may provide a basis for a simple and standard regimen for screening patients suspected of having defective T cell responses.

Adolescent↗
PubMed1984

Clinical significance of circulating immune complexes in 'lone' cryptogenic fibrosing alveolitis and those with associated connective tissue disorders.

The clinical, radiographic and physiological features, and progression rates of forty unselected patients with cryptogenic fibrosing alveolitis (CFA) have been studied in relation to serum immune complexes measured by a C1q binding technique. Twenty (50%) had levels greater than normal. Those with associated connective tissue disorders (twenty-four) had a higher frequency of raised C1q binding than those with 'lone' CFA (sixteen) (63% compared to 31%). As observed previously in this group, those with associated disorders also had higher titres of immune complexes. Twelve of thirteen with polyarthritis had immune complexes and a considerably shorter duration of disease compared with those without joint symptoms (P less than 0.01). Several other observations suggest that immune complexes are especially associated with earlier disease. These include a trend towards a younger age and a lesser radiographic profusion score; a shorter duration of symptoms in relation to titres of immune complexes amongst those with raised values (P less than 0.05) and a higher transfer factor coefficient (Kco) (P less than 0.02). The relationship between Kco and the presence of immune complexes was still observed when those with 'lone' CFA were analysed separately. Other clinical features including sex, severity of dyspnoea and lung volumes did not distinguish those with and without complexes, either for the whole group of patients or when those with and without associated connective tissue disorders were analysed separately. Analysis of eighteen patients followed to death showed no correlation between length of survival from first symptoms and immune complexes, neither was there any clear association with corticosteroid responsiveness and immune complexes.(ABSTRACT TRUNCATED AT 250 WORDS)

Antigen-Antibody Complex↗