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.

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.