Eosinophilic fasciitis (Shulman syndrome)—recognition of the histological spectrum allows for new insights into possible pathomechanisms
Authors' conclusion
Our findings highlight a specific immune phenotype of leucocyte infiltrates in EF along features of perifascicular pathology similar to DM, while there is no evidence of hypoxia-mediated or type I IFN-associated processes with perifascicular fibre atrophy, indicating different pathomechanisms of muscle involvement.
Study context
- Population
- Biopsy specimens from patients with eosinophilic fasciitis (Shulman syndrome)16 EF patients
- Comparison
- EF vs anti-Mi-2+ DM and non-disease controls
- EFn = 16
Methodological detailshistology, immunohistochemistry, quantitative reverse transcription PCR
- Histology
- Immunohistochemistry
- Quantitative reverse transcription PCR
Main finding
As reported in the abstract
- Mild to severe inflammation at the muscle-fascia interface, with frequent involvement of the underlying muscle tissue, varying in degree
- CD206+ macrophages predominate; eosinophils detected within the fascia in most cases but in small numbers, infrequently within muscle
- Th2-M2 pathway activators (STAT6, IL-4) upregulated, leading to high CD206 expression
- Th1-M1 pathway activators (STAT1, IFN-γ) upregulated, without significant upregulation of effector COX2
- Activators or chemoattractants of eosinophils not significantly upregulated in EF vs DM
- Perifascicular pathology comparable to DM with MHC class I and II upregulation, but no perifascicular atrophy, type I IFN response or hypoxia-mediated processes
Original publication
Eosinophilic fasciitis (Shulman syndrome)—recognition of the histological spectrum allows for new insights into possible pathomechanisms. Rheumatology (Oxford). Published 20 September 2022. doi:10.
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