Efficacy of eculizumab against Eosinophilic Fasciitis associated with Paroxysmal Nocturnal Haemoglobinuria

Frumholtz, L.; Sébert, Marie; Masson, Adèle de; Attias, Philippe; Adès, Lionel; Roux, Jonathan; Cabannes‐Hamy, Aurélie; Noguera, Maria‐Eléna; Attencourt, Christophe; Bagot, M.; Socié, Gèrard; Rybojad, M.; Tour, Régis Peffault de la; Bouaziz, Jean‐David

doi:10.1111/jdv.13819

Cited by 6 publications

(3 citation statements)

References 6 publications

(7 reference statements)

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Taken together, our data suggest that blood Eos at diagnosis identifies GPA subsets with different phenotypes, approaching EGPA features for some aspects. GPA patients with HE, although free from asthma, displayed some similarities with EGPA such as a higher proportion of skin involvement [8,9] and peripheral neuropathy [2]. Skin manifestations were observed in about one-third of GPA patients with normal Eos and in half of those with HE, this latter being comparable to that reported in EGPA [8].…”

Section: Discussionsupporting

confidence: 60%

“…GPA patients with HE, although free from asthma, displayed some similarities with EGPA such as a higher proportion of skin involvement [8,9] and peripheral neuropathy [2]. Skin manifestations were observed in about one-third of GPA patients with normal Eos and in half of those with HE, this latter being comparable to that reported in EGPA [8]. Similarly, peripheral neuropathy, reported at diagnosis in up to 50% in EGPA [2], was two times more common in GPA with HE vs those with normal blood Eos.…”

Section: Discussionmentioning

confidence: 95%

See 1 more Smart Citation

Significance of eosinophilia in granulomatosis with polyangiitis: data from the French Vasculitis Study Group Registry

et al. 2021

View full text Add to dashboard Cite

Objective To describe disease presentation and long-term outcome of granulomatosis with polyangiitis (GPA) patients according to blood eosinophils count (Eos) at vasculitis diagnosis. Methods Data from newly diagnosed GPA patients registered in the French Vasculitis Study Group database with available eosinophil count at diagnosis were reviewed. Disease characteristics, rate and type of relapses and overall survival were analyzed according to Eos, categorized as normal (<500/mm3), mild-to-moderate hypereosinophilia (HE) between 500 and 1500/mm3 and severe HE > 1500/mm3. Results Three hundred and fifty-four patients were included. At diagnosis, 90 (25.4%) patients had HE ≥ 500/mm3; they were more likely male (73% vs 56%, p= 0.006) and had more frequent cutaneous manifestations (49% vs 33%, p= 0.01), peripheral neuropathy (32% vs 17%, p= 0.004) and higher BVAS (21 vs 18, p= 0.01), compared with those with Eos <500/mm3. Patients with severe HE (n = 28; median Eos 2355, range 1500–9114) had more frequent renal function worsening at presentation (p= 0.008). After a median follow-up of 3.95 (IQR 1.95–6.76) years, no difference was found in overall relapse rates according to baseline Eos, but those with HE experienced more neurologic (p= 0.013) and skin (p= 0.024) relapses and had more frequently peripheral neuropathy as damage at last follow-up (p= 0.02). Overall survival difference was not significantly different in patients with normal Eos or HE at diagnosis. (p= 0.08). Conclusions Blood HE at diagnosis, observed in about one quarter of GPA patients, identifies a subgroup of patients with a more severe disease and higher rate of skin and neurological involvement both at presentation and during follow-up.

show abstract

Section: Discussionsupporting

confidence: 60%

Section: Discussionmentioning

confidence: 95%

Significance of eosinophilia in granulomatosis with polyangiitis: data from the French Vasculitis Study Group Registry

et al. 2021

View full text Add to dashboard Cite

show abstract

“…The current treatment recommendation for EF is combination therapy using high‐dose systemic corticosteroids plus immunosuppressive therapy, with preference for inclusion of low‐dose methotrexate as part of maintenance therapy . Therapies which have shown efficacy in managing EF include high‐dose pulsed intravenous (IV) methotrexate, infliximab, azathioprine, sulfasalazine, sirolimus, mycophenolate mofetil, cyclophosphamide, eculizumab, cyclosporine, tocilizumab, PUVA, immunoglobulins, and bone marrow transplantation, among others.…”

mentioning

confidence: 99%