Treatment of Wegener's granulomatosis with cyclosporin.

Borleffs, Jan C. C.; Derksen, Rwhm; Hené, R. J.

doi:10.1136/ard.46.2.175

Cited by 29 publications

(2 citation statements)

References 3 publications

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“…Although no guidelines were published at the time, cyclophosphamide and prednisolone was selected because these were widely used. In addition, cyclosporin A was selected because there were positive reports for ANCA-associated vasculitis at that time [15][16][17]. Nowadays, cycloporin A is known to suppress T cells and T cells are reported to have a pathophysiological role in ANCA-associated vasculitis [18].…”

Section: Case Presentationmentioning

confidence: 99%

Microscopic polyangiitis necrotizing glomerulonephritis associated with pregnancy: case with a 20-year clinical course and review of the literature

et al. 2018

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A 29-year-old woman with past medical history of hypertension was referred to our hospital for the evaluation of kidney dysfunction (serum creatinine 1.0 mg/dL), proteinuria (0.54 g/gCre), and microscopic hematuria. Renal biopsy before the first pregnancy was supportive for benign nephrosclerosis with no evidence of vasculitis. After her second pregnancy and delivery when she was 32 years old, she developed proteinuria of 3.2 g/gCre, hematuria, and elevated serum creatinine level of 2.6 mg/dL. Second renal biopsy revealed necrotizing glomerulonephritis and her serum MPO-ANCA was positive, leading to the diagnosis of MPA/renal-limited vasculitis (RLV). Interestingly, frozen preserved serum from 4 years earlier also tested positive for MPO-ANCA. Despite intensive treatment, hemodialysis was required 10 years later due to progressive deterioration of renal function. At that time, she developed pericarditis, bloody cardiac tamponade, and pulmonary alveolar hemorrhage, resulting in a diagnosis of systemic vasculitis MPA. She received living donor kidney transplantation at the age of 44 years, after which serum creatinine has been stable around 1.1 mg/dL without proteinuria or hematuria and MPO-ANCA has remained negative. The association of vasculitis with pregnancy and delivery is not well documented, especially in patients with MPA. Here, we report this MPO-ANCA positive woman developing MPA necrotizing glomerulonephritis after her second pregnancy and a 20-year clinical course.

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Section: Case Presentationmentioning

confidence: 99%

Microscopic polyangiitis necrotizing glomerulonephritis associated with pregnancy: case with a 20-year clinical course and review of the literature

et al. 2018

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“…(13) In resistant cases, cyclosporine may be added to this treatment regimen. (14) An effective medical treatment programme can result in the resolution of the disease in the salivary gland without surgical intervention and healing of both the organ and tissue manifestations. Early commencement of treatment may also prevent irreversible organ damage.…”

Section: A S E R E P O R T E198mentioning

confidence: 99%

Parotid gland involvement as a presenting feature of Wegener’s granulomatosis

Ceylan¹,

Asal²,

Çelenk³

et al. 2013

smedj

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C a s e R e p o r t e196 I NTRO D U C TIO NWegener's granulomatosis (WG) is a systemic disease characterised by necrotising granulomas that involve the upper and/or lower respiratory tract, with vasculitis in small arterioles and venules, and focal glomerulonephritis.(1) At onset, the disease is localised to a region without any systemic involvement. The head and neck are involved in nearly 90% of cases, while the respiratory tract, including the nose and paranasal sinuses, is usually the first site of WG.The presenting symptoms of WG that involve the upper respiratory tract include epistaxis, sinusitis, saddle nose deformity, nasal obstruction, severe rhinorrhoea and necrotising lesions.Other commonly involved sites of WG are the lungs and kidneys. In this case report, we present the case of a patient whose initial symptom involved the parotid gland. CA S E R EPO RTA 37-year-old woman presented to a medical centre with bilateral painful parotid swelling that failed to respond to systemic antibiotic treatment three months prior to her admission to our department. At the time of referral to our department, the patient had been suffering from epistaxis, nasal obstruction and a weight loss of 10 kg over the past two months. She also complained of a nonproductive cough that had persisted for the past one month. There was evidence of arthralgia of the knee, wrist and elbow, as well as fatigue and malaise. Physical examination at admission revealed diffuse enlargement and cutaneous fistulas on the bilateral parotid glands (Fig. 1

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