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

The widespread use of corticosteroids in clinical practice emphasises the need for a thorough understanding of their metabolic effects. In general, the actions of corticosteroids on carbohydrate, protein, and lipid metabolism result in increased hepatic capacity for gluconeogenesis and enhanced catabolic actions upon muscle, skin, lymphoid, adipose and connective tissues. Because of the morbidity associated with steroid therapy, the clinician must carefully consider in each case the gains that can reasonably be expected from corticosteroid therapy versus the inevitable undesirable side effects of prolonged therapy. Thus, it is important to remember that the enhanced anti-inflammatory activity of the various synthetic analogues of cortisol is not dissociated from the expected catabolic actions of glucocorticoid hormones. Replacement therapy with physiological doses of cortisol in primary or secondary adrenal insufficiency is intended to simulate the normal daily secretion of cortisol. Short term, high dose suppressive glucocorticoid therapy is indicated in the treatment of medical emergencies such as necrotising vasculitis, status asthmaticus and anaphylactic shock. With improvement of the underlying disorder, the steroid dosage can be rapidly tapered and then discontinued over a 2 to 3 day period. Long term, high dose suppressive therapy is often commonly used to treat certain diseases (see sections 4.7.2 and 4.7.3). In this setting, suppression of the hypothalamic-pituitary-adrenal axis may persist for as long as 9 to 12 months following steroid withdrawal if steroid doses are administered in the supraphysiological range for longer than 2 weeks. In general, higher doses, longer duration of usage, and frequent daily administration are all correlated with the severity of pituitary ACTH suppression. When steroid therapy is to be withdrawn, gradual tapering of the dosage is necessary; the steroid dosage should also be given as a single morning dose if possible. Rapid or total withdrawal of the steroid therapy may be associated with exacerbation of the underlying disease or with a steroid withdrawal syndrome. An additional important point to remember in any withdrawal programme is that the steroid dosage should be appropriately increased for an exacerbation of the underlying disease or for intercurrent major stress. Alternate day therapy is recommended as a steroid maintenance programme for patients requiring high dose glucocorticoid therapy over a prolonged period of time. Thus, it is usually employed to maintain a therapeutic benefit which had previously been extablished by daily steroid treatment. Complications resulting from corticosteroid therapy include: (1) proximal muscle weakness; (2) osteopenia; (3) unmasking of latent diabetes mellitus; (4) sodium retention and/or elevation of mean arterial blood pressure; (5) adverse psychiatric reactions; (6) development of glaucoma; and (7) reactivation of latent infections (such as tuberculosis).
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PMID:Corticosteroids: clinical pharmacology and therapeutic use. 20 58

A 68-year-old man with haemotological features consistent with haemopoietic dysplasia (pre-leukaemia) and an abnormal cell clone in the marrow, 46, XY, -18, +t(13;18) (q11;123), developed acute connective tissue disease characterised by vasculitis, dermal changes, marked muscular weakness and serological features suggesting an auto-immune disturbance. Although four other cases of haemopoietic dysplasia ("pre-leukaemia") with unusual connective tissue disease had been reported since the recognition of haemopoietic dysplasia as a distinct entity, a definite association between these two disease states still awaits confirmation by further reports and investigations.
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PMID:The association of haemopoietic dysplasia (pre-leukaemia) and abnormal cell clone with connective tissue disease. 28 61

Wegener's granulomatosis is a multisystem disease distinguished by a triad of necrotizing granulomatous vasculitis involving the upper and lower respiratory tracts, glomerulonephritis and systemic, small vessel vasculitis. The latter can cause neurologic manifestations when the vasa nervorum are affected. A 53-yr-old male presented with a 3-mo history of chronic nasal congestion, arthralgias, pruritic maculopapular eruption, epistaxis and lower extremity weakness. Subsequent lung and chest wall biopsies confirmed diagnosis of Wegener's granulomatosis. Summary of electrodiagnostic data obtained on initial presentation and comparison with later study indicated a sensorimotor polyneuropathy with wide-spread axonal involvement noted particularly in the distal lower extremity musculature. Electrodiagnostic documentation of rapid progression proved useful in directing alteration of immunosuppressive therapy, with favorable clinical and functional outcome. We believe this is the second case presented of a patient with documented Wegener's granulomatosis and overt clinical evidence of poly-neuropathy in whom both electroneurographic and electromyographic studies are described. Electrodiagnostic results are presented with discussion of pertinent literature.
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PMID:Electrodiagnostic characteristics of Wegener's granulomatosis-associated peripheral neuropathy. 131 Aug 60

Twenty-four patients presenting an acute stroke with watershed cerebral infarct on CT scan or MRI were included in this retrospective study. Age was 63 +/- 14 years (mean +/- SD), and sex ratio was 2 men for 1 woman. Main clinical features were: in anterior location, lower limb weakness and frontal syndrome with transcortical motor aphasia in left lesions or spatial dyscalculia in right ones; in posterior location, brachiofacial weakness with constant quadranopsia and hypoesthesia, and Gerstmann syndrome in left lesion. There was no distinctive feature for subcortical and multiple infarcts. In bilateral infarcts, there were one pseudobulbar syndrome, and 2 pseudo brainstem syndromes with neuropsychological signs. Aetiologies were severe carotid artery disease in 14 cases, severe cardiopathy in 6, isolated cerebral angiitis in 1, essential thrombocythemia in 1, protein C deficiency with sickle cell disease in 1, and cholesterol emboli in 1 anatomical case. CBF performed in carotid artery occlusions or tight stenoses showed evidence of haemodynamic changes. Microembolic process can be proposed in the case with cholesterol emboli. Preventive treatment is discussed.
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PMID:Watershed cerebral infarcts: retrospective study of 24 cases. 135

Comparative studies in the evaluation of criteria for clinicopathological diagnosis of AGA which was proposed by Japanese Health and Welfare Ministry (JHWM) in 1988 and American College of Rheumatology (ACR) in 1990 were conducted. Twenty seven Japanese patients with AGA and 231 Japanese controlled patients with polyarteritis nodosa (PAN) and Wegener's granulomatosis (WG) were used as material patients who had been provided a second questionnaire in 1984 sponsored by the JHWM. As a result, the JHWM criteria was 85.2% in sensitivity and 96.5% in specificity, which was superior to the ACR criteria being 74.1% in sensitivity and 93.9% in specificity. These results were due to the low sensitivity and low accuracy in paranasal sinus abnormality and pulmonary infiltrates which were included in the ACR criteria. Furthermore, the JHWM criteria could be diagnosed as AGA using not only the histopathological findings but by the clinical symptoms alone. The controlled patients who were overdiagnosed as AGA using ACR criteria were thought to be included as patients with WG. On the other hand, the controlled patients who were overdiagnosed as AGA using JHWM criteria were thought to be included as patients with overlap syndrome of AGA and PAN. Accuracy of polyneuropathy, muscle weakness, melena and pretibial edema were over 60%, indicating that these clinical symptoms were available for vasculitis symptoms of the JHWM criteria.
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PMID:[Studies on criteria for clinicopathological diagnosis of allergic granulomatous angiitis (AGA)]. 141 91

An 85-year-old man with a 2-year history of progressive lower limb weakness and paresthesia was found to have an IgG kappa monoclonal gammopathy of undetermined significance (mgus). Clinical and electrophysiological studies revealed a severe distal bilateral symmetrical polyneuropathy. A sural nerve biopsy showed extensive nerve fibre loss with the deposition of large amounts of amorphous material throughout the endoneurium. Electron microscopy showed the deposits to be composed of microtubular structures which were located diffusely throughout the endoneurium. The deposits were also located within the lumina of the vasa nervorum, some of which were undergoing disintegration and rupture with release of the proteinaceous material into the endoneurium. The regions of the nerve in which they appeared most numerous showed more severe nerve fibre damage than other areas. These microtubular structures were also observed in disintegrating vessels and adjacent endoneurium. On immunohistochemistry they stained with antibody to IgG. Identical deposits were found in the dermis in which there was a leucocytoclastic vasculitis. Located in linear arrays within the axons of myelinated and unmyelinated fibres were highly organised tubular structures resembling immunotactoids. Identification of immunotactoid-like structures within the nerve is unique and may be another mechanism by which monoclonal proteins can induce nerve fibre injury.
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PMID:Immunotactoid-like endoneurial deposits in a patient with monoclonal gammopathy of undetermined significance and neuropathy. 821 78

A case of polymyositis associated with Bancroftian filariasis in an adult male who presented with generalised painful swelling and weakness of muscles is presented. He had elevated muscle enzymes, a myopathic EMG pattern, focal vasculitis on gastrocnemius muscle biopsy and W. bancrofti in the peripheral blood. There was clinical, biochemical and histopathological evidence of resolution of the disorder and total clearance of microfilaremia with diethyl carbamazine (DEC) therapy.
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PMID:W. bancrofti as a causal agent of polymyositis. 148 32

A 71-year-old male complaining of chest pain was admitted to our hospital. A single cavitary mass shadow was observed on chest X-ray films. Urinalysis revealed microscopic hematuria. CT examination demonstrated a tumorous shadow in the maxillary sinus. The diagnosis of Wegener's granulomatosis was histologically established by biopsy specimens from the nasal mucosa which showed necrotizing vasculitis and granuloma with fibrinoid degeneration. He was treated with combination therapy of prednisolone and cyclophosphamide. The abnormal shadows on chest X-ray and in the maxillary sinus on CT improved rapidly, but the patient developed progressive weight loss and complained of cold intolerance, weakness and dysphagia. Serum T3, T4 and TSH were found to be reduced. Anterior pituitary function tests showed reduction of TSH, GH and ACTH responses, which was probably due to irreversible vasculitis.
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PMID:[A case of Wegener's granulomatosis complicated by hypopituitarism]. 148 37

Juvenile dermatomyositis is a relatively rare, multisystem disease characterized by a nonsuppurative myositis which causes symmetrical weakness, rash and vasculitis; this last can affect the gastrointestinal tract and the myocardium. Late development of calcinosis is seen in approximately two thirds of patients. Its etiology is unknown, although there are clues that it may be an unusual response to a viral infection. Some 50% of children will have a very acute, rapidly progressive disease, while the remainder may present subacutely with rash and a gradually progressive weakness of muscles, joint contractures and very occasionally calcinosis. When there is acute muscle damage, the creatine phosphokinase will be raised, but it is not uncommon to have a normal erythrocyte sedimentation rate, and antinuclear antibodies are usually present. Early in acute cases immune complexes will often be detected. In the presence of vasculitis, monitoring the disease by levels of von Willebrand's factor 8 antigen may be helpful. Although the prognosis for survival has steadily improved, it remains a serious illness and death can occur in the acute phase due to myocarditis, progressive unresponsive myositis, perforation of the bowel as a sequel to vasculitis ulceration or occasionally lung involvement. Intercurrent infections during the course of the disease also give rise to problems. In its management, there is still a question as to whether intravenous pulses of methylprednisone might be more valuable than oral corticosteroids; in either case it must be given in adequate amounts early in the course of the disease to control muscle inflammation. Once this is controlled rehabilitation commences.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Juvenile dermatomyositis. 153 78

We describe the histopathologic changes of skin, muscle, vessels, and fascia in 11 patients with eosinophilia myalgia syndrome, a newly described entity that has been linked to the ingestion of L-tryptophan. This syndrome is defined clinically by severe incapacitating myalgias and a peripheral eosinophilia. Arthralgias, edema of the extremities, morbilliform rashes, skin induration, weakness, fatigue, and respiratory weakness may be present as well. The earliest apparent histologic changes were observed at the septa between subcutaneous fat lobules and in the deep dermis or fascia. The septa and fascia were infiltrated with a sparse mixture of lymphocytes and histiocytes. In the deep fascia, in addition to inflammatory cells, there were distinctive, reactive mesenchymal cells that showed features of both histiocytes and fibrocytes. Minimal tissue eosinophilia was seen despite the extent of blood eosinophilia. Dermal thickening and homogenization of collagen bundles occurred with replacement of fat and adnexa (changes indistinguishable from scleroderma or morphea). Vessel walls in the dermis and fascia showed thickening and endothelial swelling, but no overt vasculitis was noted. Skeletal muscle biopsies showed a perimysial, epimysial, and/or fascial inflammatory infiltrate of lymphocytes and distinctive reactive mesenchymal cells with some eosinophils. Minimal myofiber atrophy, regeneration, or necrosis was seen despite the clinical history of severe myalgias in almost all patients. This syndrome should help gain insight into the mechanisms of fibrosis in environmental-induced, scleroderma-like syndromes and in idiopathic, scleroderma-like disorders as well.
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PMID:Pathologic manifestations of the eosinophilia myalgia syndrome: analysis of 11 cases. 156 45


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