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Persistent microvasculopathy in chronic eosinophilia-myalgia syndrome
1University of Minnesota, Hennepin Faculty Associates Neuromuscular Center, Minneapolis 55404, USA.
Abstract:
Acute eosinophilia-myalgia syndrome (EMS) due to contaminated L-tryptophan (LT) exposure is an inflammatory microangiopathy of the dermis, fascia, and muscle. Select individuals evolve from acute EMS to have persistence of myalgia, fatigue, cramps, and skin changes for years. Many develop memory dysfunction and confusion. The objective of this study is to delineate the pathology in individuals with chronic EMS. Seventeen patients with ongoing symptoms representing chronic EMS are studied by skin, fascia, and muscle biopsies four to five years after exposure to contaminated LT and initial onset of EMS. All have microvascular disease. Most have lymphocytic inflammatory infiltrates. Several have dermal sclerosis. The findings indicate that persistent microvascular disease is present in chronic EMS. The pathologic changes are similar to those of acute EMS but with notable differences. Tissue eosinophil infiltration is rare in the chronic state as compared to acute EMS. The persistence of endothelial pathology indicates continuing microvascular dysfunction.
Insights
Chronic eosinophilia-myalgia syndrome (EMS) involves persistent microvascular disease, unlike the acute phase. Biopsies reveal ongoing endothelial pathology and dysfunction years after L-tryptophan exposure.
Area of Science:
- Pathology
- Immunology
- Neurology
Background:
- Eosinophilia-myalgia syndrome (EMS) is a serious condition linked to contaminated L-tryptophan (LT) exposure.
- Some individuals experience long-term symptoms including myalgia, fatigue, and cognitive issues, indicating a chronic form of the disease.
Purpose of the Study:
- To investigate the pathological characteristics of chronic EMS.
- To understand the long-term effects of LT exposure on affected tissues.
Main Methods:
- Skin, fascia, and muscle biopsies were performed on 17 patients with chronic EMS symptoms.
- Biopsies were analyzed four to five years after initial LT exposure and EMS onset.
Main Results:
- All patients exhibited microvascular disease.
- Lymphocytic inflammatory infiltrates were common, with some cases showing dermal sclerosis.
- Eosinophil infiltration was rare in the chronic state, contrasting with acute EMS.
Conclusions:
- Persistent microvascular disease is a hallmark of chronic EMS.
- Endothelial pathology indicates ongoing microvascular dysfunction in long-term EMS.
- Pathological findings in chronic EMS differ from acute EMS, particularly regarding eosinophil presence.
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