An atypical presentation of Ormond’s disease

Retroperitoneal fibrosis is a rare condition defined by an overproduction of fibro-inflammatory tissue in the retroperitoneum, usually involving the abdominal aorta, but also managing to invade adjacent structures. We report a case of a 47-year-old female patient with an atypical radiological presentation of retroperitoneal fibrosis, involving predominantly the right kidney. Ultimately, the diagnosis was performed by an immunohistochemical study that identified IgG4+ plasmocytes after kidney nephrectomy, also known as Ormond’s disease. There are no universal guidelines for the treatment of retroperitoneal fibrosis, due to its rarity and lack of randomized controlled trials comparing different therapeutic strategies.

Ormond’s disease accompanied by ankylosing spondylitis – a case report

Idiopathic retroperitoneal fibrosis, or Ormond’s disease, is a rare syndrome which is in essence the deposition of pathological fibrous tissue in the retroperitoneal space. In most cases, it is initially asymptomatic, but as the disease progresses, clinical symptoms develop. The main complaint are abdominal pain and symptoms resulting from the involvement of ureters, arterial and venous vessels. Diagnosis is based on computed tomography or magnetic resonance imaging, demonstrating a fibrotic tissue mass accumulated in the retroperitoneal space. Due to the typically late presentation of clinical symptoms, a diagnosis is difficult and is often made after complications occur. In our article, we present the case of a 71-year-old patient in whom coexisting ankylosing spondylitis and retroperitoneal fibrosis were diagnosed 15 years after non-specific abdominal pain occurred.

Retroperitoneal Fibrosis and its Differential Diagnoses: The Role of Radiological Imaging

Background Retroperitoneal fibrosis is a rare disease with an incidence of 0–1/100 000 inhabitants per year and is associated with chronic inflammatory fibrosis of the retroperitoneum and the abdominal aorta. This article sheds light on the role of radiological imaging in retroperitoneal fibrosis, names various differential diagnoses and provides an overview of drug and surgical treatment options. Methods A literature search for the keywords “retroperitoneal fibrosis” and “Ormond’s disease” was carried out in the PubMed database between January 1, 1995 and December 31, 2019 (n = 1806). Mainly original papers were selected, but also reviews, in English and German language, with a focus on publications in the last 10 years, without excluding older publications that the authors believe are relevant to the topic discussed in the review (n = 40). Results and Conclusion Ormond’s disease is a rare but important differential diagnosis for nonspecific back and flank pain. Imaging diagnostics using CT or MRI show a retroperitoneal mass, which must be differentiated from lymphoma, sarcoma, multiple myeloma and Erdheim-Chester disease. Patients have an excellent prognosis under adequate therapy. FDG-PET/CT or FDG-PET/MRT should be considered as potential modalities, as hybrid imaging can evaluate both the morphological changes and the inflammation. Key Points: Citation Format

IgG4-related Diseases – A Rare Polycystic Form of Ormond’s Disease

Currently, Ormond’s disease is classified among IgG4-associated diseases. Its clinical manifestation varies and is characterized by the presence of fibrous retroperitoneal tissue that often affects the ureters or abdominal aorta and iliac arteries. We present a unique case of the polycystic form of Ormond’s disease, imitating tumour in the retroperitoneal space. At the time of diagnosis, the disease was not metabolically active and did not require immunosuppressive therapy. The polycystic mass was removed surgically. There has been no exacerbation of the disease during the last 12 months.

Combination of Steroids and Azathioprine in the Treatment of Ormond’s Disease – A Single Centre Retrospective Analysis

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.