scholarly journals Recalcitrant steroid-resistant EGPA with multisystem involvement

2017 ◽  
Vol 4 (3) ◽  
pp. 12
Author(s):  
Andrea Cervi ◽  
Gerard Cox ◽  
Nader Khalidi ◽  
Parameswaran Nair ◽  
Nathan Hambly
Keyword(s):  
Granulomatosis With Polyangiitis ◽  
Clinical Course ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Significant Weight Loss ◽  
Intravenous Cyclophosphamide ◽  
Multisystem Disorder ◽  
Steroid Resistant ◽  
Multisystem Involvement ◽  
Heterogeneous Nature ◽  
Eosinophilic Granulomatosis

Eosinophilic granulomatosis with polyangiitis (EGPA) is a multisystem disorder classically characterized by sequential histopathologic and clinical disease phases. An eosinophilic tissue infiltration phase typically precedes the development of a systemic small-vessel vasculitic phase. The contribution of these two pathogenic mechanisms to disease course and the boundaries between them are often blurred. We report the case of a 49-year-old man with established p-ANCA positive EGPA presenting acutely with central and peripheral nervous system dysfunction, diffuse alveolar hemorrhage, biopsy-proven myositis, and myocarditis resistant to steroids. The patient’s clinical course was marked by unremitting abdominal pain associated with significant weight loss. Although eosinophilic infiltrates were not observed on gastrointestinal mucosal biopsy, samples stained heavily for eosinophil peroxidase, indicating prior degranulation of eosinophils. Complete remission of eosinophil-mediated tissue infiltration and vasculitic phases was achieved only after the addition of intravenous cyclophosphamide to corticosteroid therapy. This case report demonstrates both: a) the importance of recognizing eosinophilic degranulation in addition to intact eosinophils in tissue and b) a unique presentation of a rare disease, underscoring the multifaceted and heterogeneous nature of EGPA and its overlapping disease phases.

scholarly journals Hypertrophic pachymeningitis in eosinophilic granulomatosis with polyangiitis

2021 ◽  
Author(s):  
Shinji Izuka ◽  
Hiroyuki Yamashita ◽  
Yuko Takahashi ◽  
Hiroshi Kaneko
Keyword(s):  
Dura Mater ◽  
Steroid Therapy ◽  
Inflammatory Disease ◽  
Granulomatosis With Polyangiitis ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Hypertrophic Pachymeningitis ◽  
Intravenous Cyclophosphamide ◽  
Blurred Vision ◽  
Small Vessels ◽  
Eosinophilic Granulomatosis

ABSTRACT Eosinophilic granulomatosis with polyangiitis (EGPA) is one of the anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV), which is characterised by systemic small vessels vasculitis with associated eosinophilia. Hypertrophic pachymeningitis (HP) is an inflammatory disease in which the cerebral or spinal dura mater is thickened. AAV other than EGPA may sometimes develop HP; however, patients with EGPA rarely develop HP. This is the case of myeloperoxidase-ANCA-positive EGPA that presented with headache and blurred vision and was diagnosed with HP. It was successfully treated with pulsed steroid therapy and intravenous cyclophosphamide without any relapse for more than 4 years.

scholarly journals Improvement of Chronic Rhinosinusitis and Reduction of the Myeloperoxidase-Antineutrophil Cytoplasmic Antibody Titer in a Patient with Eosinophilic Granulomatosis with Polyangiitis by Additional Mepolizumab

2021 ◽  
Vol 2021 ◽  
pp. 1-4
Author(s):  
Shin-ya Tamechika ◽  
Shuntaro Isogai ◽  
Shinji Maeda ◽  
Taio Naniwa ◽  
Akio Niimi
Keyword(s):  
Side Effects ◽  
Chronic Rhinosinusitis ◽  
Eosinophil Count ◽  
Granulomatosis With Polyangiitis ◽  
Clinical Course ◽  
Antineutrophil Cytoplasmic Antibody ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Inflammatory Cascade ◽  
Myeloperoxidase Antineutrophil Cytoplasmic Antibody ◽  
Eosinophilic Granulomatosis

A case of eosinophilic granulomatosis with polyangiitis (EGPA) in which chronic rhinosinusitis (CRS) was improved with a reduction in the myeloperoxidase-antineutrophil cytoplasmic antibody (MPO-ANCA) titer after the addition of mepolizumab is reported. A 55-year-old woman with EGPA receiving prednisolone 5 mg/day developed CRS with increases in the eosinophil count and the MPO-ANCA titer. Although it improved with prednisolone 15 mg/day in addition to mizoribine 150 mg/day, because azathioprine could not be taken orally due to side effects, it relapsed after prednisolone was tapered to 5 mg/day. There was no exacerbation of other vasculitis symptoms such as mononeuropathy multiplex. The patient was treated with additional mepolizumab 300 mg every 4 weeks, which resulted in the improvement of CRS and marked reductions of the eosinophil count and MPO-ANCA titer, and the reduction of prednisolone to 2 mg/day. Furthermore, even after tapering mepolizumab to 200 mg every 4 weeks, her condition remained stable without relapse of EGPA and without increases in the eosinophil count and MPO-ANCA titer. The clinical course of mepolizumab treatment in this patient suggests that the IL5-dependent inflammatory cascade is one of the factors contributing to the increase in MPO-ANCA in EGPA.

scholarly journals A Case of Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss, EGPA) Presenting With Relapsed Mononeuritis Multiplex

2020 ◽  
Vol 13 (1) ◽  
Author(s):  
Hilmi BA ◽  
Ainon MM
Keyword(s):  
Risk Stratification ◽  
Treatment Strategy ◽  
Granulomatosis With Polyangiitis ◽  
Factor Score ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Mononeuritis Multiplex ◽  
Multisystem Disorder ◽  
Control Asthma ◽  
Eosinophilic Granulomatosis ◽  
Churg Strauss

We report a case of eosinophilic granulomatosis with polyangiitis (EGPA), a rare multisystem disorder characterized by difficult-to-control asthma, hypereosinophilia and polyneuropathy. We also discuss the Five Factor Score (FFS) risk stratification strategy, which is used to quantitate the extent of the disease and guide treatment strategy.

scholarly journals Mepolizumab -- therapeutic strategy for a paediatric patient with eosinophilic granulomatosis with polyangiitis

2022 ◽  
Author(s):  
Lota Ozola ◽  
Elīna Aleksejeva ◽  
Diāna Stoldere ◽  
Ineta Grantiņa ◽  
Zane Dāvidsone ◽  
...  
Keyword(s):  
Paediatric Patient ◽  
Granulomatosis With Polyangiitis ◽  
Case Reports ◽  
Small Vessel Vasculitis ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Multisystem Disorder ◽  
Paediatric Patients ◽  
Strauss Syndrome ◽  
Eosinophilic Granulomatosis ◽  
Churg Strauss

Eosinophilic granulomatosis with polyangiitis (EGPA; formerly known as Churg-Strauss syndrome) is classified as an anti-neutrophil cytoplasmic antibody (ANCA)-associated small vessel vasculitis. It is a multisystem disorder and can affect every organ system. EGPA is a rare disease, with an estimated prevalence of 1/70,000–100,000 in Europe. As its onset usually occurs in adulthood, data from paediatric patients are limited. We present here a very rare practical EGPA clinical case involving a paediatric patient. Presently, data on mepolizumab usage in paediatric patients are limited, with only a few case reports published.

scholarly journals Eosinophilic Granulomatosis with Polyangiitis Presenting with Myocarditis as an Initial Symptom: A Case Report and Review of the Literature

2021 ◽  
pp. 329-333
Author(s):  
Kanako Kurihara ◽  
Jun Tsugawa ◽  
Shinji Ouma ◽  
Toshiyasu Ogata ◽  
Mikiko Aoki ◽  
...  
Keyword(s):  
Granulomatosis With Polyangiitis ◽  
Nerve Biopsy ◽  
Pulse Therapy ◽  
Sural Nerve Biopsy ◽  
Lower Limbs ◽  
Rapid Progression ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Initial Symptom ◽  
Steroid Pulse ◽  
Eosinophilic Granulomatosis

A 66-year-old woman with a history of bronchial asthma had shortness of breath and fatigue upon mild exercise. She was diagnosed as congestive heart failure. A blood test showed eosinophilia without the presence of anti-neutrophil cytoplasmic antibody (ANCA), and a myocardial biopsy specimen revealed eosinophilic infiltration in the myocardium. Eosinophilia was improved when she was administered short-term methylprednisolone. After that, she had numbness and pain in her lower limbs with re-elevation of eosinophils. She had dysesthesia and hypalgesia in the distal part of the limbs. Sural nerve biopsy revealed axonal degeneration and thickness of the arterial wall, indicating a diagnosis of eosinophilic granulomatosis with polyangiitis (EGPA). Two courses of steroid pulse therapy were performed, resulting in marked improvement of her sensory symptoms. ANCA-negative EGPA might be associated with myocarditis and peripheral neuropathy. A sufficient immunotherapy should have been considered to prevent rapid progression.

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