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
BACKGROUND: Idiopathic retroperitoneal fibrosis is characterized by the development of inflammatory infiltrates with marked fibrosis along the large retroperitoneal vessels. Rituximab in combination with glucocorticoids constitute an effective therapy, but the responses are not long-lasting. In other similar situations, addition of cyclophosphamide to the combination achieved longer and deeper responses. This was the reason to use the triple combination in this case. CASE: A 56-year-old man came with four weeks lasting abdominal pain with CT finding of retroperitoneal fibrosis with unilateral ureteral occlusion. Biopsy confirmed retroperitoneal fibrosis with histological findings of IgG4-associated disease. Treatment with prednizone was poorly tolerated. Therefore, the patient was switched to the combination of rituximab 375 mg/m2 on day 1, cyclophosphamide 300 mg/m2 in infusion in days 1 and 15, plus dexamethasone 20 mg in infusion on days 1 and 15, repeated in a 28-day cycle. RESULTS: Fluorodeoxyglucose (FDG) positron emission tomography (PET/CT) examination after 4 months of treatment showed a marked decrease in FDG accumulation and complete disappearance of the fibrotic mass. After 8 months, the induction therapy was followed by maintenance therapy with rituximab 1,000 mg plus dexamethasone 20 mg in 6-month intervals. Control PET/MR examination after 3 years is consistent with complete remission. The number of circulating plasmablasts correlated with the disease activity. CONCLUSION: Treatment of retroperitoneal fibrosis with the tripple combination of rituximab, cyclophosphamide and dexamethasone achieved a very rapid disappearance of pathological FDG accumulation and fibrotic retroperitoneal mass, with complete disappearance achieved after 4 months of treatment. After 3 years of maitenance therapy, the diesease is still in complete remission on PET/MR examination. We suggest to continue the maintenance therapy with rituximab because of some increase in the number of circulating plasmablasts after prolongation of the intervals between rituximab administration.
- Klíčová slova
- Cyclophosphamide, IgG4-related disease, circulating plasmablasts, cyclophosphamide, retroperitoneal fibrosis, rituximab,
- MeSH
- cyklofosfamid * terapeutické užití aplikace a dávkování MeSH
- dexamethason * terapeutické užití aplikace a dávkování MeSH
- fluorodeoxyglukosa F18 * MeSH
- kombinovaná farmakoterapie MeSH
- lidé středního věku MeSH
- lidé MeSH
- PET/CT MeSH
- retroperitoneální fibróza * farmakoterapie diagnostické zobrazování MeSH
- rituximab * terapeutické užití MeSH
- Check Tag
- lidé středního věku MeSH
- lidé MeSH
- mužské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
- kazuistiky MeSH
- Názvy látek
- cyklofosfamid * MeSH
- dexamethason * MeSH
- fluorodeoxyglukosa F18 * MeSH
- rituximab * MeSH
Idiopathic retroperitoneal fibrosis (IRF) is a rare condition characterized by the development of a peri-aortic and peri-iliac tissue showing chronic inflammatory infiltrates and pronounced fibrosis. Ureteral entrapment with consequent obstructive uropathy is one of the most common complications, which can lead to acute renal failure and, in the long term, to varying degrees of chronic kidney disease. Common symptoms at onset include lower back, abdominal or flank pain, and constitutional symptoms such as malaise, fever, and anorexia and weight loss. Pain is frequently referred to the hip, to the groin and to the lateral regions of the leg, with nocturnal exacerbations, and typically does not modify with position. We report a case of 56 year-old male with recurrent lower back pain and lower abdominal pain. Contrast-enhanced computed tomography and was suggestive of retroperitoneal fibrosis and unilateral ureteral occlusion. Histologic examination with immunohistochemical staining for IgG4 demonstrate IgG4-related retroperitoneal fibrosis. Therapy was started with prednison 1 mg/kg, but the tolerance of this dose was poor. Therefore the therapy was switched to combination of rituximab 375 mg/ m2 on day 1, cyclophosphamide 300 mg/m2 mg infusion and dexamethasone 20 mg total dose infusion on day 1 and 15 in 28 days cycle. FDG-PET/CT control in fourth month showed residual accumulation of FDG in retroperitoneal fibrotic mass, and therefore the therapy was prolonged to 8 month. The subjective symptoms of this diseases disappeared in the 8th month. Then the maintenance therapy, administration of rituximab in 6 month interval, was started. The activity of this disease be further evaluated by FDG-PET/CT imagination. Glucocorticoids are considered the cornerstone of therapy. The use of other immunosuppressive agents, including cyclophosphamide, azathioprine, methotrexate, mycophenolate mofetil and biological agents such as rituximab, tocilizumab and infliximab and sirolimus have been reported as a valuable option mostly in case reports, cases series and small studies. This agents allowed to reduce cumulative dose of glucocorticoids and its adverse effects. Therefore in our patients we preferred combination of rituximab cyclophosphamide s dexamethasone with lover dose of prednisonem. This combination is preferable for patients who cannot tolerate glucocorticoids or who are likely to suffer from significant glucocorticoids -related toxicity.
- Klíčová slova
- Cyclophosphamide, IgG4 related disease, cyclophosphamide, retroperitoneal fibrosis, rituximab,
- MeSH
- cyklofosfamid terapeutické užití MeSH
- dexamethason terapeutické užití MeSH
- fluorodeoxyglukosa F18 terapeutické užití MeSH
- glukokortikoidy terapeutické užití MeSH
- IgG4 asociovaná nemoc * komplikace farmakoterapie MeSH
- imunoglobulin G terapeutické užití MeSH
- lidé středního věku MeSH
- lidé MeSH
- PET/CT MeSH
- retroperitoneální fibróza * komplikace farmakoterapie diagnóza MeSH
- rituximab terapeutické užití MeSH
- Check Tag
- lidé středního věku MeSH
- lidé MeSH
- mužské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
- kazuistiky MeSH
- Názvy látek
- cyklofosfamid MeSH
- dexamethason MeSH
- fluorodeoxyglukosa F18 MeSH
- glukokortikoidy MeSH
- imunoglobulin G MeSH
- rituximab 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
We describe a case of an Erdheim-Chester disease patient. First line chemotherapy treatment with 2-chlorodeoxyadenosine did not reduce fluorodeoxyglucose accumulation in pathological lesions. The patient had continuously increased CRP values of 17-20 mg/l. The disease continued to cause subfebrile temperatures and significant fatigue that made the patient to spend most of the daytime in bed. To manage the permanently increased inflammation markers, we decided to start treatment with anakinra, successfully used in some other autoinflammatory diseases (e.g. Schnitzler syndrome). We have now been able to evaluate the first 6 months of treatment. Daily subcutaneous administration of anakinra (KineretTM 100 mg daily) led to normalization of CRP values, cessation of subfebrile temperatures and, importantly, significant reduction of fatigue. Time periods the patient was able to spend out of the bed increased significantly. Consequent to the reduced fatigue, the patient was able to perform basic household tasks he was unable to undertake without treatment. After 3 months of treatment, fatigue of the same intensity returned following a short interruption of therapy. The CRP values went up again to 12 mg/l. CRP value returned back to norm and fatigue ceased after re-initiation of daily Kineret injections. Objective treatment response was assessed by measuring the degree of fluorodeoxyglucose accumulation in pathological bone lesions. PET-CT was performed before and 3 and 6 months after anakinra initiation. Intensity of accumulation did not change significantly after the first 3 months of therapy but decreased after 6 month therapy. Follow up CT of abdominal cavity was performed at the end of the 6th month of treatment. Presented CT images from before and 6 months after the treatment evidence an obvious reduction in fibroid changes in the retroperitoneum. Daily administration of anakinra to a patient with active Erdheim-Chester disease significantly reduced intensity of fatigue and improved quality of life, led to a reduction in inflammatory markers and regression in retroperitoneal fibrotization.
- MeSH
- antagonista receptoru pro interleukin 1 aplikace a dávkování MeSH
- C-reaktivní protein analýza MeSH
- dospělí MeSH
- Erdheimova-Chesterova nemoc krev komplikace farmakoterapie MeSH
- injekce subkutánní MeSH
- lidé MeSH
- retroperitoneální fibróza komplikace farmakoterapie MeSH
- rozvrh dávkování léků MeSH
- Check Tag
- dospělí MeSH
- lidé MeSH
- mužské pohlaví MeSH
- Publikační typ
- anglický abstrakt MeSH
- časopisecké články MeSH
- kazuistiky MeSH
- přehledy MeSH
- Názvy látek
- antagonista receptoru pro interleukin 1 MeSH
- C-reaktivní protein MeSH
Retroperitoneal fibrosis (RPF) is a rare inflammatory disease which is characterized by the development of a fibrous process that surrounds the major vessels and organs located within the retroperitoneum. About two thirds of all cases of RPF are idiopathic and are thought to be immunological in origin. Diagnosis of RPF should be considered in patients with unexplained abdominal and low back pain and retroperitoneal lesions. We present a case report of a 59-year-old white male with idiopathic RPF with history of low back pain and weight loss as only symptoms and treated by tamoxifen, corticosteroids, and insertion of JJ endoureteric catheters due to the obstruction of the upper urinary tract.
- MeSH
- antagonisté estrogenu terapeutické užití MeSH
- lidé středního věku MeSH
- lidé MeSH
- lumbalgie etiologie MeSH
- magnetická rezonanční tomografie MeSH
- retroperitoneální fibróza komplikace farmakoterapie MeSH
- tamoxifen terapeutické užití MeSH
- Check Tag
- lidé středního věku MeSH
- lidé MeSH
- mužské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
- kazuistiky MeSH
- Názvy látek
- antagonisté estrogenu MeSH
- tamoxifen MeSH
