Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0042384 (vasculitis)
 20,525 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Nonthrombotic occlusion or stenosis of the mesenteric veins is a rare cause of intestinal ischemia that usually occurs in association with systemic vasculitis. The current report includes four male patients with segmental ischemic colitis caused by idiopathic myointimal hyperplasia in the small mesenteric veins and their intramural branches; neither vasculitis nor arterial involvement were present. Three of the four patients were less than or equal to 38 years of age; the fourth was 67. All four patients were previously healthy and had no history of drug use of any kind. Clinical findings included abdominal pain, diarrhea, bloody stools, and colonic strictures discovered by barium enema. The intima of the mesenteric and intestinal mural veins was focally thickened by a marked increase in cells and matrix between the endothelium and internal elastic lamina, whereas the vessel walls external to the thickened intima appeared normal. Histochemistry and immunoreactivity with antibodies to muscle-specific actins (HHF-35) disclosed that the intimal thickening was caused by proliferation of smooth muscle cells in a proteoglycan matrix. All patients recovered completely after segmental resection of the ischemic portion of the colon and had no recurrence of intestinal symptoms on follow-up of up to 7 years. These unusual venous lesions do not appear to have been previously described; their etiology and pathogenesis remain unknown.
 ...
PMID:Idiopathic myointimal hyperplasia of mesenteric veins. 206 29

In a sequential study the pathogenesis of chronic polyarthritis occurring spontaneously in MRL mice was analysed by light microscopy. A total of 128 MRL mice of both substrains (MRL/Mp-lpr/lpr and +/+) and different age groups was studied. In 50 lpr/lpr mice, tinctorial and histochemical methods were applied for the identification of fibrin/fibrinoid, iron compounds, amyloid, and proteoglycan. The earliest lesions seen in mice of substrain lpr/lpr at the age of 2 months were proliferation of synovial lining cells and loss of tinctorially demonstrable proteoglycan in the articular cartilage. Beginning at 3 months, severe joint destruction associated with pannus formation was encountered usually in knee, carpal and tarsal joints. Besides inflammatory processes in tendons, nerves and musculature fibrinoid-necrotising panarteritis occurred in the intra- and extraarticular tissue. Furthermore, fibrin-containing exudations and deposits of fibrinoid material, occurred in the synovium of large joints and in the periarticular connective tissue of phalangeal joints. The occurrence of these morphological changes, destructive arthritis, vasculitis and periarticular inflammatory changes, was, at the age of 3 months, associated with a highly significant increase of circulating immune complexes. In mice of substrain +/+ aged 1 year and older, arthritic changes with synovial lining cell proliferation, cartilage destruction and inflammatory periarticular lesions developed.
 ...
PMID:[Pathogenesis of spontaneous chronic polyarthritis in MRL mice]. 318 80

Antineutrophil cytoplasmic antibodies (ANCA) are present in systemic vasculitis with or without renal involvement and in inflammatory bowel diseases, conditions which share damage in proteoglycan content of basal membrane. In diabetes, there is a reduction in proteoglycans in the kidney basal membrane, responsible for the decrease in fixed anionic charges and, consequently, for the prevalent anionic proteinuria (albumin, IgG4) even in the early preclinical stage of nephropathy. The aims of this study were to search for the presence of ANCA in long-standing type 1 diabetic patients and to evaluate possible correlations with size- and/or charge-selective proteinuria. Twenty-two type 1 diabetic patients (duration of diabetes 24 years, range 9-30) selected and grouped according to albumin excretion rate values, were studied together with 21 age and sex comparable normal subjects. ANCA, albumin excretion rate, and the clearances of albumin, of prevalently cationic total IgG (IgG) and of anionic IgG4 were evaluated. ANCA were measured using ELISA and indirect immunofluorescence methods; albumin, IgG and IgG4 were tested with RIA or ELISA methods developed in our laboratory. ANCA were found in five patients, three of whom showed proteinuria. 33.3% and 18.2% of patients with normal IgG and albumin clearances respectively had elevated IgG4 clearance. This study shows for the first time the presence of ANCA in long-standing type 1 diabetic patients and confirms a prevalent anionic protein excretion in these patients, but does not show a correlation between the presence of ANCA and proteinuria, even if the presence of ANCA in diseases sharing alterations in proteoglycan content of vascular basal membrane is noteworthy.
 ...
PMID:Antineutrophil cytoplasmic antibodies are present in long standing type 1 diabetics but do not correlate with selective proteinuria. 884 49

Although disease of the sclera is unusual, when it occurs it can rapidly destroy both the eye and vision. However, normally the sclera provides an opaque protective coat for the intraocular tissues and a stable support during variations in internal pressure and eye movements, which would otherwise perturb the visual process through distortion of the retina and the lens/iris diaphragm. This stability, which is vital for clear vision is made possible by the organisation and viscoelastic properties of scleral connective tissue. Microscopically, the sclera displays distinct concentric layers including, from outside, Tenon's capsule, episclera, the scleral stroma proper and lamina fusca, melding into underlying choroid. Two sites exhibit specialised structure and function: the perilimbal trabecular meshwork, through which aqueous filters into Schlemm's canal, and the lamina cribrosa, which permits axons of the optic nerve to exit the posterior sclera. Throughout, sclera is densely collagenous, the stroma consisting of fibrils with various diameters combining into either interlacing fibre bundles or defined lamellae in outer zones. Scleral fibrils are heterotypic structures made of collagen types I and III, with small amounts of types V and VI also present. Scleral elastic fibres are especially abundant in lamina fusca and trabecular meshwork. The interfibrillar matrix is occupied by small leucine-rich proteoglycans, decorin and biglycan, containing dermatan and dermatan/chondroitin sulphate glycosaminoglycans, together with the large proteoglycan, aggrecan, which also carries keratan sulphate sidechains. Decorin is closely associated with the collagen fibrils at specific binding sites situated close to the C-terminus of the collagen molecules. Proteoglycans influence hydration, solute diffusion and fluid movement through the sclera, both from the uvea and via the trabecular meshwork. As the sclera is avascular, nutrients come from the choroid and vascular plexi in Tenon's capsule and episclera, where there is an artery to artery anastomosis in which blood oscillates, rather than flows rapidly. This predisposes to the development of vasculitis causing a spectrum of inflammatory conditions of varying intensity which, in the most severe form, necrotising scleritis, may destroy all of the structural and cellular components of the sclera. Scleral cells become fibroblastic and the stroma is infiltrated with inflammatory cells dominated by macrophages and T-lymphocytes. This process resembles, and may be concurrent with, systemic disease affecting other connective tissues, particularly the synovial joints in rheumatoid arthritis. Current views support an autoimmune aetiology for scleritis. Whilst the role of immune complexes and the nature of initial pro-inflammatory antigen(s) remain unknown, the latter may reside in scleral tissue components which are released or modified by viral infection, injury or surgical trauma.
 ...
PMID:Scleral structure, organisation and disease. A review. 1510 41

Optic neuropathy is an uncommon manifestation of relapsing polychondritis (RPC), a rare systemic disease affecting cartilaginous and proteoglycan-rich structures. The optic neuropathy has been attributed to ischemia, intrinsic inflammation of the optic nerve, or spread of inflammation to the nerve from adjacent intraconal orbital tissues. We report a case of recurrent corticosteroid-responsive optic neuropathy in which MRI did not show ocular, optic nerve, or intraconal orbital abnormalities but did show periosteal thickening and enhancement in the apical orbit and adjacent intracranial space consistent with periostitis. The periostitis, which is a manifestation of a systemic vasculitis or an autoimmune reaction to progenitors of cartilage, probably caused the optic neuropathy by compression or inflammation. It is important to recognize this mechanism of optic neuropathy as its imaging features may be a subtle yet critical clue to an underlying systemic condition that can be life-threatening if not properly managed.
 ...
PMID:Optic neuropathy associated with periostitis in relapsing polychondritis. 1741 67

Patient A, 58 years, is referred by the Rheumatologist to the Geriatrician concerning apathy. History reviews skipping arthritis, reddened and inflamed eyes, apathy and loss of interest. Physical examination revealed red tearing eyes, bradyphrenia and bradykinesia. Laboratory examination showed inflammation markers. The patient develops fever and bilateral reddened and inflamed ears. Diagnosis of relapsing polychondritis is made, the patient is treated with prednisone and the symptoms disappear. Relapsing polychondritis is a chronical disease associated with inflammation and destruction of cartilaginous structures and proteoglycan rich structures. We diagnosed Relapsing Polychondritis with a reversible dementia, probably due to cerebral vasculitis.
 ...
PMID:[Reversible cognitive decline in a patient with relapsing polychondritis]. 2114 Sep 56

Relapsing Polychondritis (RP) is an uncommon, chronic, and potentially life-threatening multisystem disorder characterized by recurrent inflammatory episodes affecting the cartilaginous tissues of the external ears, nose, peripheral joints, larynx and tracheobronchial tree, sometimes leading to their destruction. RP can also inflame other proteoglycan-rich structures, such as the eye, heart, blood vessels and inner ears. Systemic symptoms are common, and vasculitis affecting skin or internal organs may occur. The etiology of RP is still unknown, but the pathogenetic role of the autoimmunity is suggested by frequent overlaps with various autoimmune diseases, and by the presence of autoantibody against cartilage in the serum of patients with RP. Although several reports have demonstrated the clinicopathologic manifestations and radiologic findings of RP, there are no specific features of RP. Therefore, it is difficult to show the diagnosis of RP. Airway involvements are major causes of morbidity and mortality, and they have accounted for most of the deaths due to RP. To suppress the inflammation of airway mucosa and cartilage is extremely important in the successful treatment for RP. Above all, earlier diagnosis would lead to better outcomes.
 ...
PMID:[Airway involvement in relapsing polychondritis]. 2279 May 75

Recent advances in the understanding of the initiation and perpetuation of the immune response strongly suggest that all forms of noninfective immunologically induced scleral inflammation have a common origin. Analysis of the progress of patients with scleritis corroborates the current clinical classification that, together with studies of the immunohistology fluoresceine/ICG angiography, 3D proteoglycan, and keratan sulphate electron microscopy of scleritis, strongly suggests that from the initiation of the inflammatory process, necrotizing scleritis and diffuse and nodular scleritis not only pursue a different course but also have a different pathogenesis; nonnecrotizing scleritis being the consequence of an auto immune response, whereas necrotizing scleritis being the complication of an already present (if not always manifest), systemic immune-mediated systemic disease and its associated vasculitis. The increasing imaging capacity of anterior segment ocular coherence tomography (OCT) and en face OCT enables the changes occurring in the sclera during the course of the disease to be observed for the first time. These observations suggest that the inflammatory changes involve the potential suprachoroidal space between choroid and sclera, an observation supported by the presence of subscleral granulomas on histopathology. New imaging techniques have also been able to explain the changes seen in the cornea as a complication of scleritis. These findings have implications for investigation and the treatment of these conditions.
...
PMID:The impact of new methods of investigation and treatment on the understanding of the pathology of scleral inflammation. 2487 28

Relapsing polychondritis is an uncommon, immune-mediated condition characterized by episodes of inflammation of cartilaginous structures, especially the ears, nose, joints and respiratory tract. RP also affects proteoglycan-rich structures such as the eyes, heart, blood vessels and inner ear. Around one third of cases are associated with other diseases such as vasculitides, connective tissue diseases or myelodysplastic syndrome. Disorders of the inner ear occur in 40-50% of patients. Profound hearing loss is rare. The aim of this study was to describe the case of a patient with relapsing polychondritis associated with severe bilateral hearing loss and clinical manifestations of systemic vasculitis. This study reinforces the importance of an early diagnosis and immediate treatment in case of severe manifestations of the disease.
...
PMID:Relapsing polychondritis with severe hearing loss. 2544 Jul 12

FDG is a tracer for visualizing glucose metabolism. PET/CT using FDG is widely used for the diagnosis of cancer, because glycolysis is elevated in cancer cells. Similarly, active inflammatory tissue also exhibits elevated glucose metabolism because of glycolysis in activated macrophages and proliferating fibroblasts. Elevated FDG uptake by active inflammatory tissues, such as those affected by arthritis, vasculitis, lymphadenitis, and chondritis, has enabled the diagnosis of inflammatory diseases using FDG-PET/CT. Rheumatoid arthritis (RA) is a systemic, chronic inflammation of the joints resulting in synovitis. Several clinical studies of RA have demonstrated that FDG uptake in affected joints reflects the disease activity of RA, with strong correlations between FDG uptake and various clinical parameters having been noted. Furthermore, the use of FDG-PET for the sensitive detection and early monitoring of the response to RA therapy has been reported. RA is sometimes associated with subclinical vasculitis, which is related to systemic inflammation. FDG-PET/CT can be used to evaluate subclinical vasculitis in the aorta or carotid artery. Polymyalgia rheumatica (PMR) is an autoimmune musculoskeletal disease of unknown etiology characterized by pain and stiffness in the shoulder, neck, and pelvic girdle, but not in the small finger joints in the hands, together with fever, fatigue, and weight loss. There is no specific test for PMR, and its diagnosis is based on clinical diagnostic criteria and the exclusion of other diseases with similar symptoms. However, FDG-PET/CT reveals a characteristic FDG uptake by the bursitis in ischial tuberosity, greater trochanter, lumbar or cervical spinous process, and scapulohumeral joint. A combination of FDG-PET/CT findings showed a high diagnostic value for PMR in a differential diagnosis from RA. FDG-PET/CT is also very useful for evaluating large vessel vasculitis, which is often associated with PMR. Relapsing polychondritis is a rare multisystem disease of unknown etiology involving cartilaginous and proteoglycan-rich structures. Its rarity and diversity of symptoms often result in a delayed diagnosis. FDG-PET/CT reveals unique FDG uptake findings for chondritis in the auricular, nasal, trachea, bronchial tree, and costal cartilage and in the cartilage of joints. Thus, the spread of knowledge regarding these very specific FDG-PET/CT findings could promote the early diagnosis and improved disease control of relapsing polychondritis.
 ...
PMID:Clinical Value of FDG-PET/CT for the Evaluation of Rheumatic Diseases: Rheumatoid Arthritis, Polymyalgia Rheumatica, and Relapsing Polychondritis. 2858 80


1