Systemic Fibrosis Research Articles

Nephrogenic Systemic Fibrosis Mimicking Inflammatory Breast Carcinoma

Nephrogenic systemic fibrosis, previously known as nephrogenic fibrosing dermopathy, is a newly recognized systemic fibrosing disorder primarily affecting patients with chronic renal failure. Patients with skin involvement often develop papules and plaques with peau d'orange surface changes. The lower extremities and trunk are most commonly affected. The most important histologic differential diagnosis is with scleromyxedema. To our knowledge, we report the first case of nephrogenic systemic fibrosis involving the breasts of a 61-year-old woman with end-stage renal disease, clinically mimicking inflammatory breast carcinoma. We propose that nephrogenic systemic fibrosis be considered in the differential diagnosis as a rare possibility when cutaneous changes in the breast suggest inflammatory breast carcinoma in a patient with renal failure.

Gadolinium deposition in nephrogenic fibrosing dermopathy

There is growing recognition of the association between the use of gadolinium-containing radiocontrast agents for magnetic resonance imaging and the serious dermal and systemic disease nephrogenic fibrosing dermopathy/nephrogenic systemic fibrosis (NFD/NSF). The pathogenesis of this entity remains unclear; however, our recent observations suggest a likely mechanism for the initial dermal manifestations of this gadolinium toxicity.

Hyper-IgG4 disease: report and characterisation of a new disease

BackgroundWe highlight a chronic inflammatory disease we call 'hyper-IgG4 disease', which has many synonyms depending on the organ involved, the country of origin and the year of the report. It is characterized histologically by a lymphoplasmacytic inflammation with IgG4-positive cells and exuberant fibrosis, which leaves dense fibrosis on resolution. A typical example is idiopathic retroperitoneal fibrosis, but the initial report in 2001 was of sclerosing pancreatitis.MethodsWe report an index case with fever and severe systemic disease. We have also reviewed the histology of 11 further patients with idiopathic retroperitoneal fibrosis for evidence of IgG4-expressing plasma cells, and examined a wide range of other inflammatory conditions and fibrotic diseases as organ-specific controls. We have reviewed the published literature for disease associations with idiopathic, systemic fibrosing conditions and the synonyms: pseudotumour, myofibroblastic tumour, plasma cell granuloma, systemic fibrosis, xanthofibrogranulomatosis, and multifocal fibrosclerosis.ResultsHistology from all 12 patients showed, to varying degrees, fibrosis, intense inflammatory cell infiltration with lymphocytes, plasma cells, scattered neutrophils, and sometimes eosinophilic aggregates, with venulitis and obliterative arteritis. The majority of lymphocytes were T cells that expressed CD8 and CD4, with scattered B-cell-rich small lymphoid follicles. In all cases, there was a significant increase in IgG4-positive plasma cells compared with controls. In two cases, biopsies before and after steroid treatment were available, and only scattered plasma cells were seen after treatment, none of them expressing IgG4. Review of the literature shows that although pathology commonly appears confined to one organ, patients can have systemic symptoms and fever. In the active period, there is an acute phase response with a high serum concentration of IgG, and during this phase, there is a rapid clinical response to glucocorticoid steroid treatment.ConclusionWe believe that hyper-IgG4 disease is an important condition to recognise, as the diagnosis can be readily verified and the outcome with treatment is very good.

Impact of chronic inflammation on bone during childhood

Conditions such as juvenile idiopathic arthritis, inflammatory bowel diseases, systemic lupus erythematosus and cystic fibrosis are now being targeted with therapies that have markedly improved outcome and survival. However, as the classical clinical features of these diseases become better controlled, the problem of bone damage determined by chronic inflammation during childhood comes to the fore. Prolonged disease activity leads to stunted linear growth with reduced final height and reduced peak bone mass, which is a crucial factor in determining premature osteoporosis. The mechanisms leading to these skeletal alterations are many, but clinical observations and experimental models show that inflammation itself plays a major role in bone remodeling in the growing skeleton. A better understanding of these clinical features, together with insight into what is currently known about their pathogenesis, is essential in developing correct strategies to prevent and treat them.

Bosentan for the treatment of systemic sclerosis-associated pulmonary arterial hypertension, pulmonary fibrosis and digital ulcers

Systemic sclerosis (SSc) is a devastating multisytemic autoimmune disease associated with widespread vascular damage. Pulmonary arterial hypertension (PAH) occurs in a significant proportion of patients and contributes to the morbidity and mortality that occurs in this disease. The recent development of specific therapies for the treatment of PAH mandates the early recognition, appropriate evaluation and judicious management of PAH in patients with SSc. Because endothelin (ET)-1 plays an important role in the development of PAH in SSc, and may also contribute to the vascular damage and fibrosis that occur in multiple organs in patients with the disease, inhibiting the production and activity of ET-1 is an appealing strategy for the treatment of SSc. This article reviews the pathophysiology of SSc and its vascular complications, and critically evaluates the current knowledge regarding the potential role of the ET-1 receptor blocker bosentan in the management of patients with SSc.

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.

Nephrogenic systemic fibrosis: An update

Nephrogenic systemic fibrosis (NSF), previously known as nephrogenic fibrosing dermopathy, is an emerging systemic fibrosing disorder that develops in the setting of renal insufficiency. Since its recognition in 1997, several case reports of NSF have been published in the rheumatology literature, reflecting the increasing incidence and recognition of this disorder in the United States and abroad. As rheumatology professionals are commonly the first to encounter newly developing cases, it is imperative that they recognize the symptoms and comorbidities of NSF and initiate work-up and treatment. To date, the precise causes of NSF have not been established; however, several promising lines of inquiry have led to a new model of the disorder. This model speculates that circulating fibrocytes of bone marrow origin are aberrantly recruited to the skin and other systemic sites by a process likely triggered or exacerbated by endothelial damage.

Nephrogenic fibrosing dermopathy/nephrogenic systemic fibrosis with diaphragmatic involvement in a patient with respiratory failure

Nephrogenic fibrosing dermopathy/nephrogenic systemic fibrosis (NFD/NSF) is a disorder occurring exclusively in patients with renal disease. Until recently, it has been considered a fibrosing disorder essentially confined to the skin and underlying superficial soft tissue. Recent reports, however, have described patients with involvement of other organ systems, suggesting that this disorder is actually a systemic disease with preferential cutaneous manifestations. We describe a patient with end-stage renal disease with diagnosed NFD/NSF who subsequently developed respiratory failure leading to his death. Autopsy findings showed NFD/NSF involving the skin of all extremities, as well as diffuse diaphragm involvement.

Comparison of Apoptosis Detection Markers Combined with Macrophage Immunostaining to Study Phagocytosis of Apoptotic Cells in Situ

Efficient phagocytosis of cells undergoing apoptosis by macrophages is important to prevent immunological responses and development of chronic inflammatory disorders such as systemic lupus erythematosus, cystic fibrosis and atherosclerosis. To study phagocytosis of apoptotic cells (AC) by macrophages in tissue, we validated different apoptosis markers (DNA fragmentation, caspase-3 activation and cleavage of its substrate poly(ADP-ribose)polymerase-1) in combination with macrophage immunostaining. Human tonsils were used as a model because they show a high apoptosis frequency under physiological conditions as well as efficient phagocytosis of AC by macrophages. On the other hand, advanced human atherosclerotic plaques were examined since plaques show severely impaired phagocytosis of AC. Our results demonstrate that the presence of non-phagocytized terminal deoxynucleotidyl transferase end labelling (TUNEL)-positive AC represents a suitable marker of poor phagocytosis by macrophages in situ. Other markers for apoptosis, such as cleavage of caspase-3 or PARP-1, should not be used to assess phagocytosis efficiency, because activation of the caspase cascade and cleavage of their substrates can occur in AC when they have not yet been phagocytized by macrophages.

Stem Cells Defects in Systemic Sclerosis.

Systemic Sclerosis (SSc) is a connective tissue disease characterized by early generalized microangiopathy and culminating in systemic fibrosis. Recent studies have provided evidence that SSc is associated with a reactive but ineffective angiogenesis, so that the disease finally leads to the irreversible loss of capillaries. Aim of the study was to investigate whether impaired vasculogenesis in SSc is due to defective characteristics in BM microenvironment. Peripheral blood (PB) samples were collected from 70 patients (pts): circulating endothelial progenitors (CEPs) were characterized as CD45−/CD133+ and evaluated by flow cytometry. BM samples were collected from 14 SSc pts and hematopoiesis evaluated by various assays. CD133+ cells were isolated by immunomagnetic sorting (IMS) and grown in order to induce endothelial differentiation. Long-term bone marrow cultures (LTBMC) were assessed and the number of stromal clonogenic precursors evaluated by a CFU-F (colony-forming unit fibroblast) assay. Mesenchymal stem cells (MSC) were separated by IMS for the expression of the nerve growth factor-receptor (NGF-R+) and grown in order to assess the clonogenic potential and the proliferative capacity, while their multipotential differentiation ability was determined after culture in different conditioned media. Phenotypic analysis of BM mononuclear cells showed a greater expression of the surface markers P1H12 and CD105 TGF-β receptor (1.2%±0.6 vs 0.5%±0.1 in normal controls, p=0.01 and 9.9%±5 vs 4.7%±3, p=0.02 respectively), but lower percentages of NGF-R+ stromal cell precursors (0.73±0.5 vs 1.61±0.6, p=0.02) and CD133+ cells (0.36%±0.4 vs 1.2%±0.8, p=0.05). On the contrary, the absolute number of CEPs in PB was higher in patients with SSc than in healthy controls (mean values 2.1 cells/μL vs 0.26 cells/μL, p=0.04). When BM CD133+ cells were grown in the presence of VEGF, only 3/12 cases gave endothelial differentiation, but always with a reduced proliferative ability. All pts showed a defective stromal compartment and a reduced number of BM stromal precursors, as detected by the LTBMC and by the lower CFU-F frequency (4%±3.2 vs 43%±19.8/1x10(e)6 LDMNCs, p=0.002 and 7±12.8 vs 69±61/1x10(e)5 NGF-R+ cells, p=0.01). Interestingly, NGF-R+ MSC overexpressed KDR and CD117 (26.4%±7.4 vs 4.6%±1.7, p=0.01 and 87.7%±5.1 vs 57.6%±11, p=0.03 respectively): when grown in the presence of VEGF they gave rise to endothelial colonies, only in 2/8 cases they formed a confluent layer with fibroblastic morphology but a reduced proliferative ability, while in the presence of adipogenic or osteogenic inductive media they failed to origin specific differentiation. Moreover, all “in vitro” differentiated endothelial cells even before activation showed high levels of CD62-E, VCAM-1 and CD105 expression, suggestive of the presence of increased levels of proangiogenic factors in BM. The results of this study provide evidence that patients with SSc have a stem cell defect involving both the hematopoietic and the stromal cells compartments. The higher expression of KDR on NGF-R+ cells suggests a role for VEGF in inducing endothelial differentiation of MSC, so resulting in a depletion of stromal precursors. The continuous recruitment of endothelial progenitors to sites of vascular injury, suggested by the high numbers of CEPs in PB, might lead to the irreversible BM damage we observed.

Nephrogenic Fibrosing Dermopathy/Nephrogenic Systemic Fibrosis: Report of a New Case With Literature Review

Nephrogenic fibrosing dermopathy (NFD) is a fibrosing condition of the skin that is being described increasingly in patients with renal diseases, many of whom are on dialysis therapy or have undergone renal transplantation. Its etiopathology is unknown, and no standard therapy currently exists. We describe a patient with NFD for whom histopathologic studies indicated that the fibrotic process affected subcutaneous tissue, striated muscles, diaphragm, pleura, pericardium, great vessels of the heart, left ventricle and septum, and tunica albuginea in addition to the dermis. Fibroblast-like cells positive for CD34 and CD45RO and scattered CD68-positive cells were found in affected tissues. The presentation of our case is unusual in that the disease process started in the lower abdomen and upper extremities and involved the upper extremities to a greater extent than the lower extremities. Our findings indicate that the fibrosis associated with NFD can extend beyond dermis and, as part of a systemic fibrosing disorder, can involve subcutaneous tissues, striated muscles, diaphragm, pleura, pericardium, and myocardium. We therefore suggest that "nephrogenic systemic fibrosis" would be a more appropriate term for this disease entity.

Methysergide-induced scleroderma-like changes of the legs

Conflicts of interest: none declared. Sir, Methysergide is a semisynthetic ergot alkaloid ergometrine derivative, acting as a serotonin receptor antagonist, used in migraine prophylaxis. Common reported side‐effects include cardiac and arterial vasospastic symptoms and systemic fibrosis, mainly retroperitoneal. We describe the first case, to our knowledge, of methysergide‐associated scleroderma‐like changes of the legs. A 63‐year‐old woman presented with a 2‐month history of inflammatory oedema of the legs and feet. Her medical history included: (i) migraine treated with methysergide 1·65 mg daily, which she had taken continuously for more than 10 years; (ii) depression treated with lorazepam 5 mg daily for 5 years; (iii) breast carcinoma treated 3 years previously by tumorectomy, lymphadenectomy, chemotherapy and radiotherapy; since then, she had been taking tamoxifen 20 mg daily and had so far remained in remission; (iv) gastritis treated with esomeprazole 20 mg daily for 2 years; and (v) hypercholesterolaemia treated with pravastatin 20 mg daily for 2 years.

High Prevalence of Hypothyroidism in Patients with Autoimmune Pancreatitis

Autoimmune pancreatitis is a unique form of chronic pancreatitis and has been correlated with various extrapancreatic lesions. To search for a correlation between autoimmune pancreatitis and thyroid lesions, we measured thyroid functions in 41 patients with autoimmune pancreatitis and in 41 patients with chronic calcifying pancreatitis and investigated the correlation between HLA antigens and hypothyroidism. We found a significant difference in the prevalence of antithyroglobulin antibody and hypothyroidism between patients with autoimmune pancreatitis and those with chronic pancreatitis (34.1 vs. 7.3%, P = 0.005, and 26.8 vs. 0%, P = 0.0005, respectively). Patients with hypothyroidism had a significantly higher frequency of antithyroglobulin antibody (63.6%) than those without hypothyroidism but showed no differences in other findings, including serum IgG4 concentration. We could find no significant association between any HLA antigens and the hypothyroid state of autoimmune pancreatitis. One quarter of the patients with autoimmune pancreatitis have hypothyroidism that may be independent of the active state of the pancreatic lesion or systemic fibrosing disorder, and thus patients suspected of having autoimmune pancreatitis should be evaluated for possible hypothyroidism.

Dialysis-associated systemic fibrosis (nephrogenic fibrosing dermopathy): study of inflammatory cells and transforming growth factor beta1 expression in affected skin.

Nephrogenic fibrosing dermopathy (NFD) is a newly recognized cutaneous fibrotic disorder occurring in individuals with end-stage renal disease (ESRD). The aim of the present study was to describe the clinical and histopathologic features of 9 new cases and to characterize the inflammatory cells and expression of transforming growth factor beta1 (TGFbeta1) in affected skin. Clinical and laboratory assessments, including serology and pulmonary function studies, were performed in 9 patients undergoing long-term dialysis (8 hemodialysis; 1 peritoneal dialysis) for ESRD of diverse etiologies. Skin, fascia, striated muscles, lungs, and heart were examined by histopathology. Inflammatory cells were characterized by immunophenotyping using specific monoclonal antibodies. TGFbeta1 expression was determined by in situ hybridization. All patients displayed cutaneous features resembling both systemic sclerosis and diffuse fasciitis, with severe loss of motion and flexion contractures in multiple joints. Six patients displayed woody induration of the muscles of the legs, thighs, and forearms. Five of the 6 patients with lung involvement had a reduced diffusion capacity for carbon monoxide on pulmonary function testing. Marked elevations of the erythrocyte sedimentation rate and/or C-reactive protein level were found in 6 patients. Antinuclear antibodies were present at low titers in 4 patients. Histopathologic studies indicated that in addition to the dermis, the fibrotic process affected the subcutaneous tissue, fascia, striated muscles, lungs, and myocardium. Large numbers of CD68+/factor XIIIa+ dendritic cells and increased expression of TGFbeta1 were found in affected skin and muscle. Our findings indicate that the fibrotic process of NFD affects not only the dermis, but also the subcutaneous tissues, fascia, and other organs, including striated muscles, heart, and lungs. We therefore believe this is a systemic fibrosing process, and we suggest that dialysis-associated systemic fibrosis would be a better term for the condition.

Retroperitoneal fibrosis localised to the pelvis, imitating a gynaecologic tumour

BJOG: An International Journal of Obstetrics & GynaecologyVolume 110, Issue 2 p. 224-226 Retroperitoneal fibrosis localised to the pelvis, imitating a gynaecologic tumour Maria Grazia Cantù, Maria Grazia Cantù Divisione di Ostetricia e Ginecologia, Istituto di Scienze Biomediche S. Gerardo, Università degli Studi di Milano, Monza, ItalySearch for more papers by this authorElisabetta Marinetti, Elisabetta Marinetti Divisione di Ostetricia e Ginecologia, Istituto di Scienze Biomediche S. Gerardo, Università degli Studi di Milano, Monza, ItalySearch for more papers by this authorPatrizia Perego, Patrizia Perego Servizio di Anatomia Patologica, Istituto di Scienze Biomediche S. Gerardo, Università degli Studi di Milano, Monza, ItalySearch for more papers by this authorMaria Grazia Valente, Maria Grazia Valente Servizio di Anatomia Patologica, Istituto di Scienze Biomediche S. Gerardo, Università degli Studi di Milano, Monza, ItalySearch for more papers by this authorMarilena Urso, Marilena Urso Divisione di Ostetricia e Ginecologia, Istituto di Scienze Biomediche S. Gerardo, Università degli Studi di Milano, Monza, ItalySearch for more papers by this authorFabio Landoni, Corresponding Author Fabio Landoni Divisione di Ostetricia e Ginecologia, Istituto di Scienze Biomediche S. Gerardo, Università degli Studi di Milano, Monza, Italy* Dr F. Landoni, Divisione di Ostetricia e Ginecologia, Istituto di Scienze Biomediche San Gerardo, Via Solferino, 16, 20052 Monza, Italy.Search for more papers by this author Maria Grazia Cantù, Maria Grazia Cantù Divisione di Ostetricia e Ginecologia, Istituto di Scienze Biomediche S. Gerardo, Università degli Studi di Milano, Monza, ItalySearch for more papers by this authorElisabetta Marinetti, Elisabetta Marinetti Divisione di Ostetricia e Ginecologia, Istituto di Scienze Biomediche S. Gerardo, Università degli Studi di Milano, Monza, ItalySearch for more papers by this authorPatrizia Perego, Patrizia Perego Servizio di Anatomia Patologica, Istituto di Scienze Biomediche S. Gerardo, Università degli Studi di Milano, Monza, ItalySearch for more papers by this authorMaria Grazia Valente, Maria Grazia Valente Servizio di Anatomia Patologica, Istituto di Scienze Biomediche S. Gerardo, Università degli Studi di Milano, Monza, ItalySearch for more papers by this authorMarilena Urso, Marilena Urso Divisione di Ostetricia e Ginecologia, Istituto di Scienze Biomediche S. Gerardo, Università degli Studi di Milano, Monza, ItalySearch for more papers by this authorFabio Landoni, Corresponding Author Fabio Landoni Divisione di Ostetricia e Ginecologia, Istituto di Scienze Biomediche S. Gerardo, Università degli Studi di Milano, Monza, Italy* Dr F. Landoni, Divisione di Ostetricia e Ginecologia, Istituto di Scienze Biomediche San Gerardo, Via Solferino, 16, 20052 Monza, Italy.Search for more papers by this author First published: 22 December 2003 https://doi.org/10.1046/j.1471-0528.2003.01102.xRead the full textAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinked InRedditWechat Volume110, Issue2February 2003Pages 224-226 RelatedInformation

Retroperitoneal fibrosis localised to the pelvis, imitating a gynaecologic tumour

locally invasive condition that usually arises at or just below the level of the bifurcation of the aorta and then spreads through the retroperitoneum in a perivascular distribution, leading to encasement and subsequent obstruction of retroperitoneal structures. Atypical sites of extension are the small bowel mesentery, duodenum, colon, bladder and rarely the epidural space. Fibrous encasement of the ureters with resultant hydronephrosis is the hallmark of the disease. Histologic features include recent inflammation and the formation of scar tissue as the result of past inflammation. A wide variety of factors may incite fibrosis in the retroperitoneum, including haemorrhage; extravasation of urine; irradiation; surgery; non-specific gastrointestinal inflammation including Crohn’s disease, appendicitis and diverticulitis; infection, such as tuberculosis, histoplasmosis, syphilis and actinomycosis 3 ; malignancy 4 ; systemic fibrosing disease, including fibrous pseudotumour of the orbit, Riedel’s thyroiditis, sclerosing cholangiitis and mediastinal fibrosis; atherosclerosis 5 and drug misuse 6 ; but most cases are idiopathic. The disorder can affect all ages, but is more common between the ages of 50 and 70 years and occurs about two to three times more frequently in men than in women. Because of its histologic features and its association with numerous autoimmune diseases, such as ankylosing spondylitis, systemic lupus erythematosus, Wegener’s granulomatosis, Raynaud’s disease, polyarteritis nodosa and systemic vasculitis, it is possible that an immune-mediated process may be an aetiologic factor. The signs and symptoms of the disease are vague and non-specific, and therefore the diagnosis relies on radiologic findings followed by biopsy of the affected tissue.

Les interférons ont-ils une action antifibrosante ?Le point de vue de l'interniste

Purpose. -The antifibrotic action of interferon alpha on the liver is now established in hepatitis C (cf. article by T. Poynard). Numerous in vitro experimental evidences may lead people into believing in the efficacy of interferon gamma in patients with mucous membrane and pulmonary fibrosis. Current knowledge and key points. -A randomized study has confirmed the efficacy of interferon gamma in the idiopathic pulmonary fibrosis, probably by an anti TGF beta action. Several open studies have shown the efficacy of interferon gamma in patients with post-radiation cutaneous fibrosis (Chernobyl survivors, sequelae of radiotherapy for cancer). In systemic sclerosis, several teams have shown that interferon gamma reduced collagen synthesis by sclerodermic fibroblasts in vitro. Four open studies have also confirmed the efficacy of interferon gamma in systemic sclerosis. In our experience concerning 20 patients with diffuse systemic sclerosis, the five years survival with long term therapy was 85% and we observed an improvement in the cutaneous suppleness in 40% of these cases. There was no serious side effect. Future prospects and projects. -These encouraging results need to be confirmed by large randomized studies in pulmonary fibrosis and systemic sclerosis. Other indications should be assessed, particularly extra-cutaneous radiation sequelae and in systemic fibrosis (retroperitoneal, mediastinal and cervical).

Hydronephrosis associated with retroperitoneal fibrosis and sclerosing pancreatitis

Sclerosing pancreatitis is associated with raised concentrations of IgG4. We treated 22 patients with sclerosing pancreatitis, and identified and followed-up three with concomitant hydronephrosis caused by ureteral mass, later diagnosed as retroperitoneal fibrosis. We histologically examined the ureteral and pancreatic lesions of these patients and noted abundant infiltration of IgG4-bearing plasma cells in both tissues. Treatment with corticosteroids lowered serum concentrations of IgG4. IgG4 might also have a pathological role in a systemic fibrosing process that includes pancreatic and retroperitoneal lesions.

Dermatofibrosarcoma Protuberans With Lung Metastasis in a Patient With Progressive Systemic Sclerosis

A female patient with progressive systemic sclerosis and pulmonary fibrosis had a dermatofibrosarcoma protuberans on the right thigh. After resection of the tumor, new lesions occurred in the scar, and wide excision was repeated. Two years later, a lung metastasis was discovered, and segmental resection was done. After 1 year of follow-up, no local recurrences or metastases were found.

A Case of Spontaneous Resolution of Idiopathic Mediastinal Fibrosis

Mediastinal fibrosis is pathologically characterized by chronic inflammation and fibrosis of mediastinal soft tissue. Mediastinal fibrosis is local expression of a family of systemic fibrosing syndroms. This can result in compression of adjacent mediastinal structures. Idiopathic fibrosing syndromes include retroperitoneal fibrosis, sclerosing cholangitis of the orbit and fibrosis of the thyroid gland(Riedel's struma). The cause of these disorders is obscure, in some instance there is an underlying malignancy, infection, history of drug ingestion, or trauma with retroperitoneal bleeding. Treatment of mediastinal fibrosis depends on structures involved by the fibrotic process. The disease is self limited in most case or improved by steroids uses. We experienced a case of idopathic sclerosing mediastinitis with orbital fibrous dysplasia of unknowm cause, which was confirmed by open lung biopsy, so reported it with a review of literature.