scholarly journals Aortitis with aneurysm formation as a rare complication of Wegener's granulomatosis

2011 ◽  
Vol 54 (5) ◽  
pp. 1485-1487 ◽  
Author(s):  
Çagdas Ünlü ◽  
Martine Willems ◽  
Ineke J.M. Ten Berge ◽  
Dink A. Legemate
2009 ◽  
Vol 2009 ◽  
pp. 1-4 ◽  
Author(s):  
Joanna Rosalind Cunnington ◽  
Ramesh Jois ◽  
Ivan Zammit ◽  
David Scott ◽  
John Isaacs

Wegener's granulomatosis of the pituitary gland resulting in diabetes insipidus is a rare complication of the disease. Standard treatment for Wegener's granulomatosis involves a combination of prednisolone and cylophosphamide, however biologic agents are now being used in refractory cases. We report three cases of patients with diabetes insipidus as a complication of Wegener's granulomatosis who were treated with biologic agents. All three cases showed clinical response to treatment with biologic agents including rituximab and alemtuzumab and two cases demonstrated improvement in pituitary gland abnormalities by MRI. Clinicians should be aware that diabetes insipidus can present as a complication of Wegener's granulomatosis and that biologic therapies may be effective in refractory cases.


2021 ◽  
Vol 15 (1) ◽  
Author(s):  
A. Gravos ◽  
K. Katsifa ◽  
P. Tselioti ◽  
V. Grammatikopoulou ◽  
K. Sakellaridis ◽  
...  

Abstract Background Aneurysm formation is a possible, but rare, complication of granulomatosis with polyangiitis, known as Wegener’s granulomatosis. Urgent diagnosis and therapy is very important because a ruptured aneurysm could be life threatening. Case presentation We, therefore, present the case of a 63-year-old Greek man who was diagnosed with granulomatosis with polyangiitis and retroperitoneal hematoma due to ruptured aneurysm in renal artery and upper pancreaticoduodenal artery. His clinical course was complicated by acute renal failure and acute respiratory failure due to alveolar hemorrhage. Emergency coil embolization was performed. Postembolization recovery was uneventful; no bleeding occurred. The patient underwent mechanical ventilation and continuous veno-venous hemofiltration and received combined immunosuppression and supportive therapy, but eventually died 30 days after admission to hospital from severe septic shock and multiple organ failure. Conclusion Endovascular treatment is the therapy of choice, especially for patients with ruptured aneurysms that are hemodynamically stable. Early diagnosis is very important, as urgent embolization and early initiation of immunosuppression therapy are the treatment of choice.


2015 ◽  
Vol 4 (1) ◽  
pp. 27-29
Author(s):  
Doğan ATAN ◽  
Ayşe Betül TOPAK ◽  
Kürşat Murat ÖZCAN ◽  
Mehmet Ali ÇETİN ◽  
Serdar ENSARİ ◽  
...  

1997 ◽  
Vol 40 (7) ◽  
pp. 1354-1360 ◽  
Author(s):  
Phot Luisiri ◽  
Nancy Joseph Lance ◽  
James J. Curran

1970 ◽  
Vol 5 (3) ◽  
pp. 53-67
Author(s):  
Aline Dos Santos ◽  
Ana Caroline Balducci Scafi ◽  
Luciene Azevedo Morais ◽  
Pablo Girardelli Mendonça Mesquita

RESUMOIntrodução: A Granulomatose de Wegener (GW) é uma vasculite rara e idiopática associada à presença do anticorpo Anticitoplasma de Neutrófilo (ANCA) que acomete, preferencialmente, os pequenos vasos. As manifestações clínicas são diversas, ocorrendo em mais de 90% dos casos, sintomas do trato respiratório. O comprometimento renal é tardio e preditor de mau prognóstico.  Sua morbidade a médio e longo prazo inclui insuficiência renal crônica. A probabilidade de sucesso de manutenção da função renal depende da concentração sérica de creatinina ao início do tratamento, o que indica a importância do diagnóstico e terapêutica adequada precoces. Casuística: Relata-se o caso de uma paciente do sexo feminino, 61 anos, portadora de GW com comprometimento renal avançado à apresentação não precedido por sintomas pulmonares esperados. O tratamento imunossupressor associado a plasmaferese permitiu a melhora da função renal da paciente poupando-a de tornar-se dialítica- dependente. Discussão: A paciente iniciou a doença através de insuficiência renal assintomática, com valores de função renal compatíveis com o estágio mais avançado de doença renal crônica, ultrassonografia dos rins sem alterações compatíveis e sem os sintomas respiratórios esperados. Segundo a literatura, a combinação de imunossupressores e plasmaferese associa-se à recuperação renal em três meses com sobrevivência sem necessidade de diálise por 12 meses, no caso relatado, obteve-se tal resultado em 22 dias sem a necessidade de diálise após um ano. Conclusão: Devido ao diagnóstico precoce, o tratamento adequado foi instalado rapidamente proporcionando à paciente um aumento da expectativa e da qualidade de vida, evitando dependência de terapia renal substitutiva.Palavras-Chave: Granulomatose de Wegener, Plasmaferese, Doença renal crônica.  ABSTRACTIntroduction: The Wegener's Granulomatosis (WG) is a rare and idiopathic vasculitis associated with the presence of Antineutrophil Cytoplasmic Antibody (ANCA), that affects, preferentially, the small vessels. The clinical manifestations are diverse, occurring in over 90% of cases, symptoms in the respiratory tract. Kidney damage is a late and bad prognostic predictor. Morbidity in the medium and long term includes chronic renal failure. The probability of renal function maintenance success depends on serum creatinine concentration at the beginning of treatment that indicates the importance of early diagnosis and deployment of an appropriate therapy. Case Report: We present a case of a 61-year-old female patient, carrier of GW with advanced renal impairment presentation, not preceded by expected pulmonary symptoms. The immunosuppressive treatment associated with plasmapheresis allowed the improvement of the patient’s renal function, saving her from becoming dialysis-dependent Discussion: The patient developed the disease through asymptomatic renal failure, renal function with values that are compatible with the most advanced stage of chronic kidney disease, ultrasound of the kidneys without compatible changes and without the expected respiratory symptoms. According to the literature, the combination of immunosuppressive drugs and plasmapheresis is associated with renal recovery in three months with survival without dialysis for 12 months. In this case, a result was obtained in 22 days without the need for dialysis after one year. Conclusion: Due to the early diagnosis, appropriate treatment was quickly installed giving the patient increased life expectancy and quality, preventing dependence on renal replacement therapy.Keywords: Wegener’s granulomatosis, Plasmapheresis, Chronic renal failure.


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