Polyarteritis nodosa presenting as bilateral Wunderlich syndrome: Rare cause of flank pain in a young woman

Lee, Sun Hwa; Yun, Seong Jong

doi:10.1016/j.ajem.2016.11.032

Cited by 3 publications

(3 citation statements)

References 10 publications

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“…The precise frequency and pathophysiology of spontaneous aneurysm healing in PAN is unknown. Our review of the literature identified 81 reports of PAN patients who presented solely with acute disease limited to the intraabdominal cavity, 43 of which had Wunderlich's syndrome [5][6][7][8][9][10][11][12][13][14][15][16][17]. Therefore, when managing patients with spontaneous, atraumatic intra-abdominal hemorrhage in the absence of a mass or bleeding diathesis, PAN should be placed high on the list of diseases to exclude.…”

Section: Discussionmentioning

confidence: 99%

Acute Retroperitoneal Hemorrhage Due to Multi-Vessel Aneurysmal Bleeding As the Initial Manifestation of Polyarteritis Nodosa in a Previously Healthy Female

Albornoz,

Branca,

Samoyedny

2023

Cureus

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Polyarteritis nodosa (PAN) is a heterogeneous disease characterized by constitutional symptoms, multiorgan dysfunction, and a subacute to chronic mode of onset. We describe a rarely encountered manifestation of PAN in a previously healthy 64-year-old woman who developed acute intra-abdominal bleeding due to multi-vessel, vasculitis-induced retroperitoneal aneurysmal ruptures, followed by the spontaneous cessation of abdominal pain and intra-abdominal bleeding within 24 hours after hospitalization, in the absence of immunosuppressive treatment. Aneurysms regressed and eventually normalized coincident with a moderate-term course of oral corticosteroids and six months of monthly intravenous cyclophosphamide. Our report reveals that patients with life-threatening PAN may present acutely with unexpected historical, physical examination and laboratory features not commensurate with the gravity of the clinical findings. We also highlight that long-term survival lasting greater than 11 years can occur when the correct diagnosis and appropriate treatment are introduced early in the course of the illness.

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Section: Discussionmentioning

confidence: 99%

Acute Retroperitoneal Hemorrhage Due to Multi-Vessel Aneurysmal Bleeding As the Initial Manifestation of Polyarteritis Nodosa in a Previously Healthy Female

Albornoz,

Branca,

Samoyedny

2023

Cureus

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“…(6)(7)(8) La hematuria micro o macroscópica puede o no estar presente (40%) al momento del diagnóstico. (6,9,10) El ultrasonido suele ser el primer estudio realizado en estos pacientes encontrando la imagen característica de hematoma perirrenal.…”

Section: Discussionunclassified

Síndrome de Wünderlich en el embarazo y puerperio, presentación de caso y revisión de la bibliografía

et al. 2019

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El síndrome de Wunderlich también llamado hematoma retroperitoneal espontáneo, es una complicación poco frecuente de etiología variada, En un 50% se asocia a un Angiomiolipoma Renal. El síntoma más común es el dolor abdominal y en un 20% de los pacientes se observa la característica Tríada de Lenk la cual consiste en: dolor abdominal (67%), masa abdominal palpable (54%) y shock hipovolémico (26%). El estudio diagnóstico de elección es la Tomografía Abdominal con contraste Intravenoso, sin embargo la angiografía tiene una utilidad diagnóstica y terapéutica en aquellos pacientes candidatos a angioembolización. El tratamiento de elección depende del estado clínico del paciente, la forma de presentación y en algunos casos la función renal previa, en la mayoría de las ocasiones consiste en Nefrectomía, sin embargo se han descrito casos de manejo exitoso con Nefrectomía parcial o Angioembolización selectiva en pacientes seleccionados: con evolución insidiosa, hemodinámicamente estables, afección en ambos riñones entre otras. Presentamos el caso de una paciente femenino de 19 años de edad, cursando los primeros dias de puerperio fisiológico, quien acude a urgencias por dolor abdominal y hematuria macroscópica, se encuentra en US abdominal una lesión heterogénea en el riñón derecho, se realiza TAC abdomen con contraste IV, se descubre una imagen sugerente de hematoma renal derecho, debido a un abrupto deterioro hemodinamico, se decide relizar nefrectomía derecha, el reporte de patología descarta la presencia de angiomiolipoma u alguna otra neoplasia o condición anormal en la pieza quirúrgica.

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“…Renal neoplasms specifically angiomyolipoma which is a benign neoplasm accounts for 60–65% cases of WS. Other aetiologies include renal cell carcinoma (RCC), vasculitis such as polyarteritis nodosa (PAN) pheochromocytoma and other retroperitoneal tumours [ 1 , 2 , 4 ]. Incidence of WS in pregnancy is extremely rare and there is no clear guidance on how to manage it and it often requires a collaborative approach of several specialists such as obstetricians, internists and urologists [ 5 , 6 ].…”

Section: Introductionmentioning

confidence: 99%

Post-partum occurrence of Wunderlich syndrome and microangiopathic haemolytic anaemia (MAHA): a case report

Swaminathan

Sedhom

Shahzad

et al. 2021

Journal of Community Hospital Internal Medicine Perspectives

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This is a case of a 27-year-old primigravida with monochorionic diamniotic twin gestation who was admitted to the hospital for induction of labour. Her postpartum course was complicated by microangiopathic haemolytic anemia (MAHA). The etiology for the MAHA was initially thought to be secondary to pre-eclampsia and vitamin B12/folate deficiency. However, she had persistent anemia and further workup demonstrated that she had a left renal cell carcinoma (RCC) with perinephric haemorrhage consistent with Wunderlich syndrome. This case was intriguing because of its unusual presentation and the several diagnostic and therapeutic challenges along the way.

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Polyarteritis nodosa presenting as bilateral Wunderlich syndrome: Rare cause of flank pain in a young woman

Cited by 3 publications

References 10 publications

Acute Retroperitoneal Hemorrhage Due to Multi-Vessel Aneurysmal Bleeding As the Initial Manifestation of Polyarteritis Nodosa in a Previously Healthy Female

Acute Retroperitoneal Hemorrhage Due to Multi-Vessel Aneurysmal Bleeding As the Initial Manifestation of Polyarteritis Nodosa in a Previously Healthy Female

Síndrome de Wünderlich en el embarazo y puerperio, presentación de caso y revisión de la bibliografía

Post-partum occurrence of Wunderlich syndrome and microangiopathic haemolytic anaemia (MAHA): a case report

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