Periaortitis Dotaz Zobrazit nápovědu
Chronic periaortitis involving idiopathic retroperitoneal fibrosis, inflammatory aneurysm of the abdominal aorta and perianeurysmal retroperitoneal fibrosis is a rare disease characterised by the development ofan inflammatory fibrotising process surrounding large vessels and organs of the retroperitoneum. According to some authors, chronic periaortitis results from a systemic autoimmune inflammation with vasculitis affecting aortal vasa vasorum and medium and small calibre retroperitoneal vessels. The disease is assumed to have immunopathogenetic background. Chronic periaortitis is diagnosed with the use ofultrasonography, computed tomography or magnetic resonance, and needle biopsy is mostly used for biopsy examinations. Positron emission tomography (PET) is a non-invasive diagnostic method which shows the bio-distribution of positron emitter labelled radiopharmaceuticals in the body. Due to the fact that not only tumorous, but in certain conditions also some inflammatory cells may exhibit increased accumulation of 18F-FDG (2-[18F]-fluoro-2-deoxy-D-glukose, 18F-FDG PET can be used to diagnose and to ascertain the activity of some types of chronic inflammations. We report a case ofa 60-year old man with idiopathic retroperitoneal fibrosis. 18F-FDG PET examination shoed an increased accumulation of the radiopharmaceutical around the abdominal aorta and of common ileac veins of the retroperitoneum. 18F-FDG PET fusion and computed tomography (CT) showed a correlation between the metabolically active tissue (PET) and the soft tissue infiltrate visible in CT images. A control exam done 4 months after the start of systemic therapy showed that the pathological accumulation of the radiopharmaceutical in the retroperitoneum had disappeared. 18F-FDG PET may be a useful tool to improve diagnosis of chronic periaortitis. A significant contribution of this method is the possibility to assess the activity of the pathologic process while monitoring the effect of the treatment.
- MeSH
- fluorodeoxyglukosa F18 * MeSH
- lidé středního věku MeSH
- lidé MeSH
- pozitronová emisní tomografie * MeSH
- radiofarmaka * MeSH
- retroperitoneální fibróza diagnostické zobrazování MeSH
- Check Tag
- lidé středního věku MeSH
- lidé MeSH
- mužské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
- kazuistiky MeSH
- Názvy látek
- fluorodeoxyglukosa F18 * MeSH
- radiofarmaka * MeSH
Ormond's disease is a relatively rare disease with unclear etiology, characterized by chronic periaortitis and retroperitoneal fibrosis. The inflammatory process affects the infrarenal part of the abdominal aorta and the iliac arteries, and the presence of infiltrates encasing the ureters and inferior vena cava. This disease is currently classed as an IgG4-related disease. In our review we analyse the clinical history, diagnostic approaches, surgical and immunosuppressive therapies.
- Klíčová slova
- Hydronefrosis, Immunosuppression, Ormond’s disease, Periaortitis, Surgical treatment,
- MeSH
- autoimunitní nemoci diagnóza imunologie terapie MeSH
- imunoglobulin G * MeSH
- imunosupresiva terapeutické užití MeSH
- retroperitoneální fibróza diagnóza imunologie terapie MeSH
- urologické chirurgické výkony metody MeSH
- Publikační typ
- časopisecké články MeSH
- práce podpořená grantem MeSH
- přehledy MeSH
- Názvy látek
- imunoglobulin G * MeSH
- imunosupresiva MeSH
Ormond's disease is a systemic autoimmune disease with serious complications. We present our retrospective analysis of 83 patients diagnosed with and treated for idiopathic retroperitoneal fibrosis (Ormond's disease) in our department from 1997 to 2023. In this retrospective study, we analysed the diagnostic approaches, the clinical history and surgical and immunosuppressive therapies, and their subsequent effects on our patients. Patients with established disease activity were given immunosuppressive treatment, using corticosteroids alone or in combination with azathioprine, in patients with exacerbation of the disease mycophenolate mofetil. Three patients with Ormond's disease and systemic complications (IgG4-related disease) were treated with rituximab. In the entire cohort, 83 patients received immunosuppressive therapy; the next 5 patients did not receive this treatment because they did not present inflammatory activity from the disease. In these 83 patients, computed tomography showed that immunosuppressive treatment resulted in partial or complete regression of the inflammatory infiltrate. Out of the 83 patients, 10 patients experienced disease exacerbation 7 and 24 months after the immunosuppressive treatment was discontinued. The follow-up ranged from 24 months to 26 years.
- Klíčová slova
- Hydronephrosis, Immunosuppression, Ormond’s disease, Periaortitis,
- MeSH
- dospělí MeSH
- imunosupresiva * terapeutické užití MeSH
- lidé středního věku MeSH
- lidé MeSH
- retroperitoneální fibróza * diagnóza farmakoterapie MeSH
- retrospektivní studie MeSH
- senioři MeSH
- Check Tag
- dospělí MeSH
- lidé středního věku MeSH
- lidé MeSH
- mužské pohlaví MeSH
- senioři MeSH
- ženské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
- Názvy látek
- imunosupresiva * MeSH
Ormond disease - idiopathic retroperitoneal fibrosis - is a rare condition characterized in situ by the development of fibrous plaques in the retroperitoneal space and anatomicaly dependent structures. The associated encasement of both ureters and progress to hydronefrosis of the kidney are typical clinical manifestations. Less typical manifestations are possible (for example chronic periaortitis), where clinical diagnosis is more difficult. The laboratory findings are not specific for this disease and a biopsy is not always possible for anatomical reasons. In these cases, the use of positron emission tomography/computed tomography - has been found to be the solution, specifically for patients with periaortitis. Ormond disease is generally idiopathic, and secondary - to the use of certain drugs, malignant diseases, infections. Idiopathic retroperitoneal disease is thought to result from the clinical manifestation of a systemic autoimmune disease. The purpose of this article is to present two casuistics, one of a less than usual clinical manifestation. Both positron emission tomography/computed tomography were used in the diagnostics. The treatment ofOrmond disease involves the combination of surgical and immunosuppressive treatment.
- MeSH
- dospělí MeSH
- lidé MeSH
- počítačová rentgenová tomografie MeSH
- pozitronová emisní tomografie MeSH
- retroperitoneální fibróza * komplikace diagnóza MeSH
- Check Tag
- dospělí MeSH
- lidé MeSH
- mužské pohlaví MeSH
- ženské pohlaví MeSH
- Publikační typ
- anglický abstrakt MeSH
- časopisecké články MeSH
- kazuistiky MeSH
We present a retrospective analysis of patients treated in our Department of Clinical Biochemistry, Haematology and Immunology, Na Homolce Hospital, during 1997-2013 for Ormond's disease. We analyse the clinical history, diagnostic approaches, surgical, and immunosuppressive therapies and their subsequent effect on our patients. 28 patients treated for Ormond's disease were included. Patients with established disease activity (26 patients) were given immunosuppressive treatment, using corticosteroids in combination with azathioprine. Treatment response was evaluated using clinical symptomatology, inflammatory parameters and imaging methods. In the cohort as a whole, immunosuppressive therapy was applied in 26 patients; in two patients it was not used as no inflammatory activity was found with the disease. In all 26 patients, computed tomography showed that immunosuppressive treatment resulted in partial or complete regression of inflammatory infiltrate. Out of the total number of 26 patients, two patients experienced disease exacerbation 7 and 16 months after the immunosuppressive treatment was discontinued. The longest follow-up period was 16 years; the shortest one was 21 months. Idiopathic retroperitoneal fibrosis--Ormond's disease--is a disease with serious complications. Standard treatment involves a combination of surgery and immunosuppressive treatment. The combination of corticosteroids and azathioprine represents a potentially safe and useful method of treatment.
- Klíčová slova
- Hydronephrosis, Immunosuppression, Ormond’s disease, Periaortitis, Surgical treatment,
- MeSH
- azathioprin terapeutické užití MeSH
- glukokortikoidy terapeutické užití MeSH
- imunosupresiva terapeutické užití MeSH
- lidé středního věku MeSH
- lidé MeSH
- následné studie MeSH
- počítačová rentgenová tomografie metody MeSH
- posouzení stavu pacienta MeSH
- retroperitoneální fibróza * diagnóza farmakoterapie patofyziologie MeSH
- retroperitoneální prostor patologie MeSH
- retrospektivní studie MeSH
- výsledek terapie MeSH
- zánět * farmakoterapie patofyziologie MeSH
- Check Tag
- lidé středního věku MeSH
- lidé MeSH
- mužské pohlaví MeSH
- ženské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
- práce podpořená grantem MeSH
- Geografické názvy
- Česká republika MeSH
- Názvy látek
- azathioprin MeSH
- glukokortikoidy MeSH
- imunosupresiva MeSH
An aggressive periaortic lymphoma could very rarely invade the aortic wall. We present a unique case of a patient with symptomatic thoracic aneurysm and imminent rupture due to the periaortic lymphoma, in which endovascular treatment using stent graft was applied. After stabilization of the aorta and histological confirmation of aggressive B-cell lymphoma by computed tomography-guided biopsy, the antilymphoma therapy was initiated. Despite the full treatment, the patient died 12 months later.
- Klíčová slova
- aortic aneurysm, lymphoma, stent graft,
- MeSH
- aneurysma hrudní aorty diagnostické zobrazování etiologie patologie chirurgie MeSH
- aorta thoracica diagnostické zobrazování patologie chirurgie MeSH
- aortografie metody MeSH
- B-buněčný lymfom komplikace diagnostické zobrazování farmakoterapie patologie MeSH
- cévní protézy MeSH
- cévy - implantace protéz * přístrojové vybavení MeSH
- CT angiografie MeSH
- endovaskulární výkony * přístrojové vybavení MeSH
- fatální výsledek MeSH
- invazivní růst nádoru MeSH
- lidé MeSH
- PET/CT MeSH
- progrese nemoci MeSH
- senioři MeSH
- stenty MeSH
- ultrazvukem navigovaná biopsie MeSH
- výsledek terapie MeSH
- Check Tag
- lidé MeSH
- mužské pohlaví MeSH
- senioři MeSH
- Publikační typ
- časopisecké články MeSH
- kazuistiky MeSH
- MeSH
- imunoglobulin G metabolismus MeSH
- lidé MeSH
- retroperitoneální fibróza diagnóza etiologie imunologie MeSH
- Check Tag
- lidé MeSH
- Publikační typ
- komentáře MeSH
- úvodníky MeSH
- Názvy látek
- imunoglobulin G MeSH
In 1972-1991 at the urological clinic in Hradec Králové 13 patients with retroperitoneal fibrosis were treated. In six idiopathic fibrosis, primary fibrosis (morbus Ormond) was involved. By surgery only one patient with the primary disease was treated, five patients with secondary fibrosis only conservatively (by nephrostomy, by introduction of an ureteral endoprosthesis and prednisone administration 1200-1500 mg in total). Seven patients, incl. five with primary fibrosis, had combined treatment, an open operation with subsequent administration of prednisone. All remained under dispensary care of the clinic. During check-up examinations renal function and congestion in the urinary pathways was examined. In morbus Ormond treatment was successful in 83% of the patients (one patient died from myocardial infarction on the fifth day after operation); in patients with the secondary disease treatment was successful in 60%.
- MeSH
- dospělí MeSH
- lidé středního věku MeSH
- lidé MeSH
- retroperitoneální fibróza terapie MeSH
- senioři MeSH
- Check Tag
- dospělí MeSH
- lidé středního věku MeSH
- lidé MeSH
- mužské pohlaví MeSH
- senioři MeSH
- ženské pohlaví MeSH
- Publikační typ
- anglický abstrakt MeSH
- časopisecké články MeSH
Retroperitoneal fibrosis (RF) is a rare disease characterized by inflammation and fibrosis of retroperitoneal soft tissues. It is classified into two types: idiopathic (iRF) and secondary (sRF). The aim of the study was to investigate the relationship between iRF and IgG4-related disease (IgG4-RD) and to eventually extend the clinicopathological features of this condition by analysis of the sample comprising six iRF and six sRF patients. The iRF patients included four males and two females, aged 12-62 years (median 55 years). Two lesions were periaortic, one was periureteral, and three cases showed both periaortic and periureteral localization. Two patients had increased serum levels of IgG4. None of the patients developed any manifestation of IgG4-RD during the follow-up period ranging for 15-133 months (median 43 months). Microscopically, in two iRF cases, fibrosis was highly cellular encircling the vessels, nerves, and paraganglia. Phlebitis was found in all cases and being obliterative in four. Lymphocytic inflammation with formation of follicles and plasma cell infiltration were scored as severe in five iRF cases. The numbers of IgG-positive plasma cells ranged 0-373 per 1 HPF (high power field; median 132) and of IgG4-positive plasma cells 0-238 per 1 HPF (median 91). The IgG4/IgG ratio values ranged 0.38-0.74 (median 0.68). Two of the iRF cases were diagnosed as definite and three cases as probable IgG4-RD. To the contrary, none of the sRF cases met the diagnostic criteria for either definite, probable, or possible IgG4-related disease. Our results indicate that a substantial portion of iRF cases, including some of very rare pediatric cases, is a manifestation of IgG4-RD.
- MeSH
- autoimunitní nemoci diagnóza etiologie imunologie MeSH
- dítě MeSH
- dospělí MeSH
- flebitida diagnóza imunologie patologie MeSH
- imunoglobulin G imunologie metabolismus MeSH
- lidé středního věku MeSH
- lidé MeSH
- plazmatické buňky imunologie patologie MeSH
- retroperitoneální fibróza imunologie MeSH
- retroperitoneální prostor patologie MeSH
- senioři MeSH
- Check Tag
- dítě MeSH
- dospělí MeSH
- lidé středního věku MeSH
- lidé MeSH
- mužské pohlaví MeSH
- senioři MeSH
- ženské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
- práce podpořená grantem MeSH
- Názvy látek
- imunoglobulin G MeSH
The authors describe an uncommon case of a localized form of idiopathic retroperitoneal fibrosis in a 70-year-old patient. They considered the tumour in the right hypogastrium (20 x 18 x 10 cm) malignant. The result of histological examination was surprising--Ormond's disease. The process could not be removed without damaging the common iliac artery. Simple suture of the artery proved unsuitable. During the postoperative period occlusion of the artery developed which was successfully resolved by an aorto-femoral bypass. Half a year after surgery the patient did not suffer a relapse.
