scholarly journals A Rare Case of Eosinophilic Granulomatosis with Polyangiitis Associated with Cryoglobulinemia Presenting with a Bullous Skin Eruption of the Lower Limbs

2018 ◽  
Vol 2018 ◽  
pp. 1-3
Author(s):  
D. D. K. Abeyaratne ◽  
C. Liyanapathirana ◽  
C. L. Fonseka ◽  
P. W. M. C. S. B. Wijekoon
Keyword(s):  
Skin Eruption ◽  
Lower Limb ◽  
Rare Case ◽  
Granulomatosis With Polyangiitis ◽  
Early Recognition ◽  
Lower Limbs ◽  
Small Vessel Vasculitis ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Bilateral Lower Limb ◽  
Eosinophilic Granulomatosis

Background. Eosinophilic granulomatosis with polyangiitis (EGPA) is an antineutrophil cytoplasmic antibody- (ANCA-) associated small vessel vasculitis with multisystem involvement. It is characterized with asthma, eosinophilia, and renal and peripheral nervous system involvement. However, EGPA presenting with bullous skin eruption is an uncommon dermatological manifestation. We report a rare case of EGPA overlapped with mixed essential cryoglobulinemia presenting with a bullous skin eruption. Case Presentation. A 49-year-old female presented with bilateral lower limb erythematous bullous rash with bilateral lower limb numbness. She had bilateral ankle edema with frothyuria and a recent onset wheeze. Blood investigations revealed a marked peripheral eosinophilia with positive P-ANCA. Skin biopsy was suggestive of leukocytoclastic vasculitis. She also had positive cryoglobulins with a high rheumatoid factor titre. The patient was diagnosed of having EGPA with overlapping mixed essential cryoglobulinemia. Her skin eruptions and systemic manifestations improved with prednisolone and cyclophosphamide therapy. Conclusion. EGPA can rarely present with a bullous skin eruption and may rarely associate with secondary cryoglobulinemia. Early recognition of these rare manifestations and prompt treatment would prevent further complications and death.

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.

Pulmonary Vasculitis

Chest Imaging ◽  
2019 ◽  
pp. 355-359
Author(s):  
Felipe Martínez
Keyword(s):  
Granulomatosis With Polyangiitis ◽  
Vascular Wall ◽  
Antineutrophil Cytoplasmic Antibody ◽  
Small Vessel Vasculitis ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Goodpasture Syndrome ◽  
Work Up ◽  
Eosinophilic Granulomatosis ◽  
Common Manifestation

Vasculitis refers to inflammation of blood vessel walls that results in vascular wall destruction and ischemic injury to affected organs. Common vasculitides discussed herein include Takayasu arteritis (TAK), giant cell arteritis (GCA), antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides such as granulomatosis with polyangiitis (GPA) and eosinophilic granulomatosis with polyangiitis (EGPA), and anti-glomerular basement membrane (anti-GBM) disease or Goodpasture syndrome. Vasculitides are further subcategorized depending of the size of the predominantly affected vessels: large, medium and small vessel vasculitis. The affected vessel size strongly influences the clinical and imaging manifestations of the disease. Intrathoracic involvement is more common in small and large vessel vasculitides. Diffuse alveolar hemorrhage (DAH), a common manifestation of vasculitis, is considered a syndrome rather than a specific entity and will be discussed in this chapter. However, it should be noted that DAH may also result from non-vasculitic etiologies. The work up and diagnosis of patients with primary vasculitides is challenging and requires close collaboration between the clinician, the radiologist and the pathologist. Radiographic abnormalities are non specific or may be absent. CT and MRI are the imaging modalities of choice for the evaluation and follow up of these patients, and should be considered despite normal radiographics.

Eosinophilic granulomatosis with polyangiitis: understanding the disease and its management

Rheumatology ◽  
2020 ◽  
Vol 59 (Supplement_3) ◽  
pp. iii84-iii94 ◽  
Author(s):  
Giorgio Trivioli ◽  
Benjamin Terrier ◽  
Augusto Vaglio
Keyword(s):  
B Cells ◽  
Granulomatosis With Polyangiitis ◽  
Clinical Presentation ◽  
Small Vessel ◽  
Small Vessel Vasculitis ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Mononeuritis Multiplex ◽  
Persistent Symptoms ◽  
Tissue Eosinophilia ◽  
Eosinophilic Granulomatosis

Abstract Eosinophilic granulomatosis with polyangiitis is characterized by asthma, blood and tissue eosinophilia and small-vessel vasculitis. The clinical presentation is variable, but two main clinic-pathologic subsets can be distinguished: one hallmarked by positive ANCA and predominant ‘vasculitic’ manifestations (e.g. glomerulonephritis, purpura and mononeuritis multiplex) and the other by negative ANCA and prominent ‘eosinophilic’ manifestations (e.g. lung infiltrates and cardiomyopathy). The pathogenesis is not fully understood but probably results from the interplay between T and B cells and eosinophils. Eosinophilic granulomatosis with polyangiitis must be differentiated from several conditions, including hypereosinophilic syndromes and other small-vessel vasculitides. The overall survival is good; however, patients frequently relapse and have persistent symptoms. The recently developed monoclonal antibodies targeting B cells and eosinophilopoietic cytokines such as IL-5 are emerging as valid alternatives to conventional immunosuppressive therapies. In this review, we discuss the essential features of eosinophilic granulomatosis with polyangiitis, with particular respect to the most relevant issues concerning clinical presentation and management.

P0373MEPOLIZUMAB THERAPY FOR EOSINOPHILIC GRANULOMATOSIS WITH POLYANGIITIS (EGPA) - ONE YEAR FOLLOW-UP STUDY USING ANTI-IL5 AS A STEROID SPARING THERAPEUTIC APPROACH

2020 ◽  
Vol 35 (Supplement_3) ◽  
Author(s):  
Allyson Egan ◽  
Pasupathy Sivasothy ◽  
Robin Gore ◽  
Marcos MartinezDel-Pero ◽  
Lisa Willcocks ◽  
...  
Keyword(s):  
Adjuvant Therapy ◽  
Granulomatosis With Polyangiitis ◽  
Care Pathway ◽  
Response To Therapy ◽  
Small Vessel Vasculitis ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Positive Serology ◽  
Eosinophilic Asthma ◽  
Asthma Control Questionnaire ◽  
Eosinophilic Granulomatosis

Abstract Background and Aims EGPA is a small vessel vasculitis characterised by the presence of tissue eosinophilia, necrotising vasculitis and granulomatous inflammation1. Typically, a prodromal asthmatic phase, leads to an eosinophilic stage, which can evolve to include the presence of vasculitis with renal manifestations. In the recent randomised, placebo-controlled MIRRA trial for relapsing and refractory EGPA, adjuvant therapy with anti-IL5 mAB Mepolizumab [MEPO] at 300mg s/c monthly, accrued longer times in remission, reduced steroid exposure and reduced relapse rates2. The aim of our study was to analyse the response and outcome for EGPA patients who received 100mg s/c of MEPO monthly for a minimum of 52 weeks, with particular focus on the steroid minimisation benefits. Method This retrospective, descriptive study analysed 13 patients with EGPA, who received 100mg s/m monthly MEPO therapy under the eosinophilic asthma care-pathway. Time points of assessment included MEPO commencement [M0] and 12 [M12] months. Results One patient had MEPO switched to Rituximab to treat both EGPA and new onset rheumatoid arthritis Conclusion The relapsing nature of EGPA places a potential dependency of therapy on steroids for asthmatic and vasculitic flares. This underscores the importance of targeted pathway specific biologic therapy to minimise steroid exposure, prevent tissue damage and ensure early response to therapy. This study demonstrates that anti-IL5 serves as a favourable model with steroid minimisation, improvement in asthma control questionnaire, reduction in BVAS and eosinophil counts at the 100mg s/c dosage. ANCA positive serology normalised in all four patients, independent of subtype. Well tolerated, it demonstrated considerable clinical benefit, with 12 patients [92.3%] continuing anti-IL5 therapy beyond 12 months. Adjuvant therapy with conventional immunosuppressants was well tolerated and renal function was preserved. ADDIN Mendeley Bibliography CSL_BIBLIOGRAPHY 1. J.C.Jenette, et al Revised International Chapel Hil Consensus Conference Nomenclature of Vasculitides. 65, 1–11 (2013). 2. Wechsler, M. E. et al. Mepolizumab or Placebo for Eosinophilic Granulomatosis with Polyangiitis. N. Engl. J. Med. 376, 1921–1932 (2017).

scholarly journals A challenging diagnosis of MPO-C-ANCA EGPA

2019 ◽  
Vol 12 (7) ◽  
pp. e228621
Author(s):  
Gareth Lim ◽  
Sheryl Lim ◽  
Shang-Ian Tee ◽  
Chai Yiing Ling
Keyword(s):  
Nasopharyngeal Carcinoma ◽  
Rare Case ◽  
Granulomatosis With Polyangiitis ◽  
Late Onset ◽  
Rare Condition ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Mononeuritis Multiplex ◽  
Atypical Presentations ◽  
Eosinophilic Granulomatosis ◽  
Proximal Myopathy

Eosinophilic granulomatosis with polyangiitis (EGPA) is a multisystemic small-vessel vasculitic disease that can present with positive MPO-P-ANCA (myeloperoxidase–perinuclear–anti-neutrophil cytoplasmic antibody). It is a rare condition that is difficult to diagnose. We present the case of a 64-year-old man with late-onset adult asthma and treated nasopharyngeal carcinoma who initially presented to us with proximal myopathy. Thereafter, he developed a constellation of fleeting symptoms which included rhinosinusitis, mononeuritis multiplex, skin vasculitis and arthritis. Blood investigations showed that he had eosinophilia, and skin biopsy demonstrated dermal vasculitis with eosinophils. He was found to be MPO-C-ANCA positive, and although initially thought to have granulomatosis with polyangiitis, the diagnosis was later revised to EGPA. This case highlights the diagnostic challenges with atypical presentations of EGPA and also presents a rare case of positive MPO-C-ANCA that has never been described in EGPA before.

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