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

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.

Peripheral Neuropathy in ANCA Vasculitis

Antineutrophil cytoplasmic antibodies (ANCA)-associated diseases are necrotizing systemic vasculitides that affect small blood vessels (arterioles, capillaries and venules). This entity represents three main systemic vasculitides: granulomatosis with polyangiitis (GPA; formerly Wegener’s granulomatosis), microscopic polyangiitis (MPA) and eosinophilic granulomatosis with polyangiitis (EGPA; formerly known as Churg-Strauss’ syndrome). Their clinical manifestations are polymorphous, being the most frequent respiratory, oto-laryngo-pharyngeal and renal involvement. Peripheral neuropathy (PN) is reported in almost 50% of the patients. The aim of this chapter is to discuss the prevalence, clinical presentation, treatment and prognosis of PN in ANCA-associated vasculitis.

Atividade Física para Mulher Portadora de Síndrome de Churg Strauss: Relato de Caso

Introdução: A síndrome de Churg Strauss (SCS) é uma doença autoimune de etiologia indeterminada. Seu diagnóstico é difícil, não somente pela raridade, mas também pela sobreposição clínica e anatomopatológica que pode haver entre diferentes vasculites; por este motivo, pode ter sua prevalência subestimada. Manifestações sistêmicas incluem mialgia, febre, perda de peso, artralgia, alterações cutâneas, neuropatia periférica, envolvimento pulmonar, de trato gastrintestinal e cardiomiopatia. Comumente são utilizados glicocorticoides para combater os efeitos da doença, entretanto, apresentam efeitos colaterais importantes, como obesidade centrípeta, face em lua cheia, giba ou corcunda de búfalo, osteoporose, fraqueza, miopatia, atrofia muscular; necrose asséptica da cabeça do fêmur e úmero, além da depressão do sistema imunológico. Objetivo: Relatar um caso singular de uma paciente diagnosticada com SCS por 10 anos, que realiza tratamento com corticosteroides e treinamento físico para combater os efeitos colaterais do tratamento farmacológico. Relato de caso: Para combater os efeitos colaterais desse tratamento, foi adotado o treinamento de força (TF) e corrida. A paciente realizou TF três a cinco vezes por semana durante todo o período de tratamento até o momento. A carga utilizada em seus treinamentos variou entre 50 a 70% de 1 Repetição Máxima, de acordo com a periodização, além de corridas duas vezes por semana com percepção subjetiva de esforço entre 4 e 6. Regularmente foram realizados exames de sangue para análise de eosinófilos, densitometria óssea e avaliações antropométricas. O treinamento mostrou indícios de ser útil na manutenção da composição corporal e da densidade mineral óssea (DMO). Conclusão: O TF e o treinamento aeróbio são estratégias importantes no combate aos efeitos da SCS e do tratamento com corticosteroides.

Nonasthmatic Churg–Strauss syndrome superimposed on chronic pyelonephritis: a case report

Churg–Strauss syndrome (CSS) is a granulomatous small-vessel vasculitis. Asthma is seen in the majority of patients with CSS, but atypical nonasthmatic forms of CSS are also being recognized. We herein describe a 67-year-old woman with a history of chronic pyelonephritis and drug allergy reactions who was admitted to our hospital because of worsening renal function preceded by fever, purpura, sinusitis, and a positive urine culture that confirmed a urinary infection. She was initially treated with pipemidic acid for 7 days, followed by clarithromycin for sinusitis. Laboratory tests on admission showed an absolute eosinophil count of 1750 cells/µL and serum creatinine concentration of 4.72 mg/dL. Urine and blood cultures showed no growth. Kidney biopsy revealed crescent formations with diffuse interstitial fibrosis and foci of eosinophil infiltration. An atypical form of CSS was diagnosed based on tissue eosinophilia, peripheral eosinophilia, and sinusitis. Intravenous methylprednisolone and cyclophosphamide pulse therapy together with hemodialysis treatment improved the patient’s clinical condition but did not resolve the kidney damage. The onset of an atypical form of CSS in our patient manifested as symptoms and signs mimicking those of chronic pyelonephritis and drug allergy reactions. The patient’s chronic kidney disease finally progressed to dialysis dependence.

Pleural Effusion in Churg-Strauss Syndrome Detected and Successfully Treated at a Tertiary Care Institute: Case Report

Churg-Strauss syndrome is a disorder of hypereosinophilia and systemic vasculitis in subjects with asthma and allergic rhinitis. Patient was admitted with chief complaints of cough without expectoration for a duration of three weeks without accompanying fever. X ray revealed pleural effusion ‘right side’. Exudative pleural fluid with eosinophil predominance and peripheral eosinophilia. The patient was further investigated. No history suggestive of any drug use. stool routine microscopy show no parasites. ANA investigated found to be positive in 1: 80 titre in homogenous pattern. Patient was then found to be P-ANCA positive by immunofluorescence microscopy. Patient was successfully treated with oral prednisolone 40 mg for three months and subsequently improved with disappearance of symptoms. Keywords: Churg-Strauss syndrome, Pleural effusion, hypereosinophilia.