Idiopathic retroperitoneal fibrosis and its overlap with IgG4-related disease.

Retroperitoneal fibrosis (RPF) is a rare disease characterised by fibrous tissue proliferation in the retroperitoneum, with encasement of the ureters and large vessels of the abdomen as the most destructive of potentially severe complications. It can either be idiopathic, or secondary to infections, malignancies, or the use of certain drugs. The idiopathic form accounts for approximately 75% of the cases, and is usually responsive to immunosuppressive therapy. In recent years, the emergence of a new clinical entity, IgG4-related disease (IgG4-RD), shed light on many fibro-inflammatory disorders once thought to be separate clinical entities, although frequently associated in the so-called multifocal fibrosclerosis. Among these, together with sclerosing pancreatitis and cholangitis, pseudotumour of the orbit, idiopathic mediastinal fibrosis and other conditions, is idiopathic retroperitoneal fibrosis (IRF). Both IRF and IgG4-RD can be associated with a wide variety of disorders, usually governed by immune-mediated (and particularly auto-immune) mechanisms. In our review, we discuss the clinical and therapeutic challenges IRF presents to the internist, as well as the meaning of its recent inclusion in the IgG4-RD spectrum from a clinical practice standpoint.

Vasovagal reflex emergency caused by Riedel's thyroiditis: A case report and review of the literature.

Riedel's thyroiditis is a rare type of chronic thyroiditis, associated with fibroinflammatory process and invasion into surrounding tissues, leading to compressive symptoms. A 45-year-old man had a left thyroid mass, presenting with hypotension and bradycardia many times. He was diagnosed with vasovagal reflex caused by cervical vessel compression due to a thyroid lesion. We performed the emergency operation, and most of the left thyroid was removed to relieve the compression on cervical vessels. The result of pathology proved to be Riedel's thyroiditis. The vasovagal reflex did not occur any more during the 28-month follow up, except on the 3(rd) day after the surgery. Six months after the thyroidectomy, the patient was found to have retroperitoneal fibrosis, diagnosed by biopsy during a laparotomy for biliary disease. Riedel's thyroiditis can lead to a vasovagal episode and might not be a primary thyroid disease but rather a manifestation of the systemic disorder, multifocal fibrosclerosis.

[Immunoglobulin G4 (IgG4)-related disease. A review of head and neck manifestations]. / Immunglobulin-G4(IgG4)-assoziierte Erkrankung. Gesichertes und Kontroverses der häufigsten Kopf-Hals-Manifestationen.

Immunoglobulin G4 (IgG4)-related disease (also known as hyper-IgG4 disease) is a recently defined emerging condition with highly heterogeneous clinicopathological features and variable disease manifestations. This disorder is characterized by unifocal or multifocal (multiorgan) involvement by tumefactive plasma cell-rich inflammatory infiltrates associated with prominent fibrosclerosis. This not uncommonly interferes with organ function resulting in diverse clinical symptoms. The autoimmune pancreatitis represents the prototype of this disease; however, to date almost all organs have been reported to be involved in this disorder. In the head and neck area several presentations of this disease may be encountered in salivary glands, lacrimal glands, thyroid gland, lymph nodes, soft tissue of the neck, ear and sinonasal tract. However, IgG4 positive plasma cells are occasionally prominent in non-specific chronic inflammatory conditions of the head and neck and the oral cavity unrelated to autoimmune diseases or systemic disorders, thus representing diagnostic pitfalls. The diagnosis of IgG4-related disease should be based on a combination of typical histological, clinical and serological findings.

Immunoglobulin g4 non-related sclerosing disease with intracardiac mass mimicking mitral stenosis: case report.

The cardiovascular system may be one of the target organs of both immunoglobulin G4 related and non-related systemic multifocal fibrosclerosis. We present a case of IgG4 non-related systemic multifocal fibrosclerosis mimicking mitral stenosis on echocardiography. For a more detailed differential diagnosis, we used multimodal imaging techniques. After surgical biopsy around the abdominal aortic area in the retroperitoneum, histological examination revealed IgG4 non-related systemic multifocal fibrosclerosis. We describe the multimodal imaging used to diagnose IgG4 non-related systemic multifocal fibrosclerosis and a positive response to steroid treatment. There have been no previous case reports of IgG4 non-related systemic multifocal fibrosclerosis with intracardiac involvement. Here, we report a case of IgG4 non-related systemic multifocal fibrosclerosis mimicking mitral stenosis.

IgG4-related multiorgan disease: report of the first autopsy case.

IgG4-related disease (IgG4RD) is a chronic recurring fibro-inflammatory pathology that is considered to be of autoimmune origin. Histopathology is considered to be the gold standard method for diagnosis. IgG4RD affects multiple organs. IgG4RD was first identified in the pancreas and was called autoimmune pancreatitis (AIP). During the following years, the disease spectrum was expanded and it was realised that the extrapancreatic lesions can precede, coexist or appear after the diagnosis of AIP. At present, several illnesses such as Mikulicz disease, Küttner tumour, multifocal fibrosclerosis, etc, are considered to be part of the IgG4RD spectrum. The symptoms of the disease tend to appear over months and years and diagnosis is achieved on average 13.5 months (4-60 months) after the onset. The purpose of this report was to provide information about a case that was sadly fatal but that permitted a complete histopathological study of the damaged tissues.

Cochleovestibular nerve involvement in multifocal fibrosclerosis.

OBJECTIVES: To report a case of multifocal fibrosclerosis with a nine-year follow up, and to discuss this disease's radiological appearance and management. The disease is a rare systemic disorder of unknown cause characterised by fibrous proliferation involving multiple anatomical sites. CASE REPORT: A 50-year-old woman presented with histological findings characterised by similar inflammatory processes involving the meninges, pituitary gland, peritoneum, retroperitoneum and orbits, prompting a search for a common pathophysiology. A diagnosis of multifocal fibrosclerosis was postulated. Symptom improvement was noted after treatment with prednisone and azathioprine. CONCLUSION: This is the first documented case of involvement of the cochleovestibular nerve in a patient with multifocal fibrosclerosis. The rare association between fibrotic diseases and masses showing various clinical patterns should be kept in mind by otolaryngologists, and imaging performed to investigate for multifocal fibrosclerosis. However, diagnosis can only be confirmed with tissue biopsy and histopathological examination.

Sclerosing orbital inflammation and systemic disease.

PURPOSE: To determine the clinical differences between sclerosing orbital inflammation (SOI) isolated to the orbit and SOI manifesting as part of systemic inflammatory disease. METHODS: For the case series, the authors identified patients with SOI from their patient database and tabulated their clinical data. For the literature review, a search for case reports of SOI in the English literature was performed. The clinical data of each were tabulated and analyzed. RESULTS: For the case series, 13 cases of SOI were identified, 2 with multifocal fibrosclerosis (MFF) and 4 with other systemic inflammatory diseases. Of the 5 bilateral cases, 4 (80%) had some form of systemic inflammatory disease including 2 with MFF. Only 2 (25%) of the 8 unilateral cases had systemic inflammatory disease. The average ESR was 7 mm/hour for those without systemic disease and 41 mm/hour with evidence of systemic disease (p = 0.038). For the literature review, the authors identified 68 case reports of SOI not previously reported in large case series, 34 associated with MFF, 5 associated with other systemic disease, and 29 not associated with any systemic disease. Sixty-nine percent of cases with systemic disease had bilateral orbital involvement, whereas only 3.6% of those without systemic disease were bilateral (p = 1.15 × 10(-8)). Twenty of the cases with MFF noted ESR (83 mm/hour, mean), 95% of which were above normal. ESR was noted in only one non-MFF, nonsystemic disease case and was normal. CONCLUSIONS: SOI in the setting of MFF or other systemic inflammatory disease is often bilateral and associated with an elevated ESR.

High serum immunoglobulin G4-related retrosternal fibrosclerosis.

A 65-year-old man with a history of exposure to asbestos complained of left leg edema. Computed tomography showed a flat, symmetrical, and longitudinal retrosternal thickening in addition to a presacral tumor. Retroperitoneal fibrosis was suspected. Serum immunoglobulin G4 (IgG4) levels were elevated (213 mg/dL). Thoracoscopic biopsy was performed. Histopathologic findings showed fibrotic tissue accompanied by proliferation of IgG4-positive plasma cells (proportion of IgG4/IgG-positive plasma cells ≥70%) indicative of multifocal fibrosclerosis.

[A case of multifocal fibrosclerosis presenting with chronic subdural hematoma].

Multifocal fibrosclerosis(MFS) is a rare disorder of unknown etiology, characterized by chronic inflammation with dense fibrosis and lymphoplasmacytic infiltration into the connective tissue of various organs. Recently, MFS was classified as IgG4-related systemic disease. In this paper, we report a 60-year-old man with no history of head injury presenting with chronic subdural hematoma(CSDH). After surgery, he complained of severe, continuous headache and persistent high-grade fever. Extensive evaluation, including 67Ga scintigraphy suggesting inflammations in various organs, liver needle biopsy showing sclerosing cholangitis, and blood examination showing elevated serum IgG4 levels, led to the diagnosis of MFS. To our knowledge this is the first report of MFS causing CSDH. The mechanism of the formation of CSDH is presumed to involve reactive granular membrane together with exudative subdural collection caused by MFS, which gives rise to minor and repeated bleeding. In this case, oral corticosteroid therapy was dramatically effective in the treatment of the condition.

Unexpected sclerosing mediastinitis involving the ascending aorta in the setting of a multifocal fibrosclerotic disorder.

Sclerosing mediastinitis is an uncommon condition associated with infections, inflammatory diseases, autoimmune disorders, and malignancies. It predominantly affects the trachea, superior vena cava, pulmonary veins, and pulmonary arteries. We herein present the first case of sclerosing mediastinitis involving the ascending aorta as part of a multifocal fibrosclerotic disorder.