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Query: UMLS:C0042384 (vasculitis)
 20,525 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

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

In the past year, the major contributions to our understanding of Churg-Strauss syndrome and polyarteritis nodosa were the development of classification criteria by which to separate these from other vasculitides. The absence of granulomas in most biopsies from patients with Churg-Strauss syndrome was noted. Other reports suggested that allergic disease may be only one of the hypereosinophilic conditions that predispose to this form of vasculitis. Eosinophilia and vasculitis occurring in a patient after dietary supplementation with L-tryptophan were reported, and the prominent cardiac involvement in Churg-Strauss syndrome was reemphasized. Impressive responses of that disease to cyclophosphamide were documented in one patient. In the American College of Rheumatology Vasculitis Classification Study, polyarteritis nodosa was one of the hardest vasculitides to distinguish. Several reports reiterated the serious risk of major pulmonary hemorrhage from microscopic polyarteritis of the lung, which probably represents a forme fruste of Wegener's granulomatosis. Although no new studies of therapy in polyarteritis were reported, a review of infections in such patients indicated that exposure to more than 15 mg/d of prednisone correlated with infectious complications, and that intra-abdominal infections were particularly problematic.
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PMID:Churg-Strauss syndrome and polyarteritis nodosa. 167 14

The iatrogenic L-tryptophan-induced eosinophilia-myalgia syndrome, often considered to be a "new" disease, has proven to be a remarkable mimic of the classic sclerosing rheumatologic disorders. Although subacute cutaneous lupus erythematosus remains a clinically defined entity, supportive histologic and immunopathologic findings have recently been proposed. Rheumatoid neutrophilic dermatitis needs to be added to our usual differential diagnosis of a neutrophilic dermatosis without leukocytoclastic vasculitis. The antiphospholipid syndrome is associated with noninflammatory vascular thrombosis and often has recognizable cutaneous findings. Finally, ANCA are a valuable adjunct in the systemic evaluation of patients with vasculitis syndromes and suggest a common pathogenesis for several of the systemic vasculitides.
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PMID:Collagen vascular disease. 173 Jan 64

Tryptophan, an essential amino acid commercially available as a dietary supplement, has been implicated in the development of a new and potentially fatal clinical entity: eosinophilia-myalgia syndrome (EMS). EMS reached epidemic proportions in the US in late 1989 and early 1990, with 1536 cases and 27 deaths reported as of August 1990. Features of the syndrome include intense, debilitating myalgias and marked peripheral eosinophilia. Vasculitis, neuropathy, and pulmonary involvement also may be observed but are not pathognomonic. Death typically ensues from ascending polyneuropathy with resulting paralysis and respiratory arrest. Treatment involves discontinuation of tryptophan ingestion. Administration of prednisone may not always alleviate or reverse the symptoms. Recovery is generally slow. The etiology of EMS has been traced to a contaminant in the bulk manufacturing process of tryptophan by a single Japanese company. Efforts are currently underway to confirm the structure of the contaminant by laboratory synthesis and to define its biologic and toxic effects using an animal model for EMS.
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PMID:Tryptophan toxicity: a pharmacoepidemiologic review of eosinophilia-myalgia syndrome. 176 43

A case is presented of interstitial pneumonitis and pulmonary vasculitis ascribed to the ingestion of an L-tryptophan preparation. An unintended rechallenge supported the causal relationship. There was neither myalgia nor peripheral eosinophilia. Bronchoalveolar lavage fluid contained 12% eosinophils but few were present in the surgical lung biopsy specimen. Lung infiltrates receded after withdrawal of the drug and treatment with steroids. Dyspnoea and pulmonary hypertension persisted. Cyclophosphamide had no effect. Sclerodermiform skin lesions appeared as a late sequel. Chromatographic analysis of the L-tryptophan revealed no suspect impurities.
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PMID:Interstitial pneumonitis and pulmonary vasculitis in a patient taking an L-tryptophan preparation. 178 80

A series of four patients with pulmonary infiltrates, pleural effusions, hypoxemia, peripheral eosinophilia, and symptoms of dyspnea, fatigue, and weakness is reported. Lung tissue obtained in three patients revealed interstitial pneumonitis, small-to-medium-vessel mixed-cell vasculitis, and alveolar exudate of histiocytes and eosinophils. All patients reported ingestion of L-tryptophan-containing products at a time when an association between L-tryptophan and the eosinophilia-myalgia syndrome was established. This clinical pattern of pulmonary involvement may be part of the continuum of the eosinophilia-myalgia syndrome. The pathophysiology of this syndrome and the relationship with the ingestion of L-tryptophan-containing products have not yet been identified.
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PMID:Acute eosinophilic pulmonary disease associated with the ingestion of L-tryptophan-containing products. 198 92

Skeletal muscle biopsies from five patients with severe myalgias, peripheral eosinophilia, and a recent history of L-tryptophan ingestion were analyzed. Perimysial inflammation, predominantly mononuclear with variable numbers of eosinophils was seen (in five of five patients), which was perineurial (in three of five) and perivascular (in five of five) in location. Grouping of the myofiber types was identified by enzyme histochemistry in two of four patients; fresh muscle for histochemical studies was unavailable from one patient. An occasional degenerating myofiber was seen in only one patient, who was still ingesting L-tryptophan at the time of biopsy. No vasculitis was seen. The focus of muscle injury in this syndrome appeared to be the perimysium and, in particular, the perineurial and perivascular connective tissue.
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PMID:Perimyositis with perineuritis and myofiber type grouping in the eosinophilia myalgia syndrome associated with tryptophan ingestion. 199 28

This report describes two women who presented with severe respiratory failure and diffuse pulmonary infiltrates in the fall of 1989. Both required prolonged assisted ventilation because of severe shunt physiology. Open lung biopsies on admission revealed a small vessel vasculitis as the sole morphologic abnormality in both patients. Both responded to high dose corticosteroids. Neither patient exhibited evidence of systemic vasculitis, and neither had serologic evidence of an immune disorder. Common to both patients was ingestion of L-tryptophan. One patient exhibited several features of the eosinophilia-myalgia syndrome. The other patient did not appear to have the syndrome, but the temporal relationship between the onset of symptoms and initiation of L-tryptophan treatment was striking. The presentation of these patients alters our notions concerning the spectrum of clinical manifestations caused by this agent, and the response to methylprednisolone supports its efficacy in the treatment of this disorder.
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PMID:Acute respiratory failure caused by pulmonary vasculitis after L-tryptophan ingestion. 200 Oct 80

Eight patients who became ill while taking tryptophan had myalgia, fatigue, rash, fever, edema, alopecia, arthralgias, diminished joint motion, skin tightening, muscle cramping, and distal paresthesias. Three had shortness of breath, and one had pulmonary hypertension. Laboratory abnormalities included peripheral eosinophilia, leukocytosis, thrombocytosis, raised erythrocyte sedimentation rate, and elevated serum levels of aldolase, lactate dehydrogenase, and liver enzymes. Of 4 chest radiographs, 3 were abnormal. Of 5 skin and muscle biopsies, 4 showed sclerosis or mixed inflammatory cell infiltration of the dermis, subcutis, and fascia. Eosinophils were often present, but vasculitis was absent. Muscle inflammation was minimal. We conclude that the "eosinophilia-myalgia syndrome" is related to the ingestion of tryptophan and that abnormalities in the secretion of lymphokines may be important in its pathogenesis.
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PMID:Tryptophan-induced eosinophilia-myalgia syndrome. 221 1

Four patients fulfilling the case definition for eosinophilia-myalgia syndrome are described, including one whose disease began in 1986. Each displayed a variety of symptoms: one suffered principally from myalgia and recovered spontaneously on discontinuation of L-tryptophan therapy; one exhibited progressive sclerodermiform skin changes, neuropathy, and myopathy; a third had prominent neuromuscular disease and sclerodermiform skin changes; and the fourth experienced profound weight loss, an axonal polyneuropathy, and perivascular lymphoid infiltrates simulating a lymphoma. Evidence of T-cell activation was present in peripheral blood and affected tissues during the clinically active progressive phase of disease. Among other manifestations pleural effusion, cutaneous vasculitis, joint contractures, and bloody diarrhea were observed. A history of L-tryptophan ingestion should be sought in patients with myalgia, fatigue, or the above outlined symptoms.
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PMID:Eosinophilia-myalgia syndrome associated with L-tryptophan ingestion. Analysis of four patients and implications for differential diagnosis and pathogenesis. 217 45


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