1/60. polyarteritis nodosa involving the hepatobiliary system in an eight-year-old girl with a previous diagnosis of familial mediterranean fever.polyarteritis nodosa (PAN) is a vasculitis of small- and medium-sized muscular arteries with deposition of immune complex in the vessel wall. Although gastrointestinal involvement is common, the symptomatic involvement of the hepatobiliary system is rare. An eight-year old female patient with a previous diagnosis of familial mediterranean fever (FMF) was hospitalized for right upper quadrant pain and fever. The thickened gall bladder wall by ultrasonography, called for exploration. Histopathological evaluations of the liver biopsy and gall bladder revealed PAN. Corticosteroid therapy was initiated and the patient recovered fully. This case represents one of the rarest forms of PAN in childhood.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
2/60. Unusual presentation of polyarteritis nodosa.We describe an unusual presentation of a localized form of polyarteritis nodosa (PAN) manifested by acute onset of severe calf pain. Biopsies of the gastrocnemius muscle and fascia revealed an acute necrotizing arteritis with fasciitis. The lumens of affected vessels were occluded by thrombi. PAN localized to calf muscles is extremely rare. To our knowledge this is the first report of evidence of fascial involvement believed to contribute to the severity of the clinical features of PAN. The occurrence of multiple intraluminal thrombi in conjunction with anticardiolipin antibodies suggested the possibility of a coexisting coagulopathy, and they were also likely contributors to the severity of the pain.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
3/60. Pancreaticobiliary arteriovenous malformation with common bile duct dilation in a patient with hemobilia.Arteriovenous malformation of the pancreas is a very rare entity. We describe a case involving the head of the pancreas associated with progressive hemobilia bleeding from the lower part of the common bile duct. The patient was a 50-year-old man with acute epigastralgia. Endoscopic retrograde cholangiopancreatography revealed hemobilia and cystic dilation of the common bile duct. Angiography demonstrated increased blood volume in the head of the pancreas and early filling of the superior mesenteric and portal veins. abdominal pain and progressive anemia caused by hemobilia required surgical treatment. Histologic examination of the resected specimen revealed marked proliferation of the blood vessels in the pericholedochal area and the exact point of bleeding from the pancreaticobiliary arteriovenous malformation.- - - - - - - - - - ranking = 4.3518508492626keywords = blood vessel, vessel (Clic here for more details about this article) |
4/60. Cutaneous polyarteritis nodosa after streptococcal necrotizing fasciitis.polyarteritis nodosa (PAN) is a necrotizing arteritis of small and medium-sized vessels. It may present with hypertension and/or renal insufficiency. Peripheral neuropathy, myopathy, joint pains, testicular pain, and ischemic myalgias may also be seen. Gastrointestinal involvement may lead to gangrene of the bowel, peritonitis, perforation, intra-abdominal hemorrhage, and pancreatitis. The cutaneous manifestations include tender subcutaneous nodules grouped along the course of superficial arteries of the lower extremities, with or without an overlying livedo reticularis. Although multisystem involvement is characteristic, sometimes only one organ or system may be involved. Associations with viral hepatitis (both B and C) and streptococcal infection have been established for PAN. Recurrent strep infections of the upper respiratory tract, streptococcal glomerulonephritis and rheumatic fever have previously been linked to PAN. This report extends the spectrum of associated streptococcal infections to include necrotizing fasciitis.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
5/60. Exudative retinal detachment and scleritis in polyarteritis.A 64-year-old white man, treated with systemic corticosteroids for five years, developed polyarteritis. He then developed a severe scleritis with an exudative retinal detachment in the right eye, which became blind and painful and was enucleated 23 months after onset of the scleritis. Histopathologic examination of the enucleated eye revealed granulomatous scleritis, chronic nongranulomatous uveitis, exudative retinal detachment, and perivasculitis of intrascleral, iris, ciliary body, and retinal blood vessels. Systemic findings were minimal and limited to elevated sedimentation rate, weight loss, mild anemia, and microscopic hematuria. Respiratory disease, severe kidney disease, hypertension, and arthritis were notably absent. A muscle biopsy established the diagnosis.- - - - - - - - - - ranking = 4.3518508492626keywords = blood vessel, vessel (Clic here for more details about this article) |
6/60. Systemic lupus erythematosus presented as non-inflammatory necrotizing vasculopathy-induced ischemic glomerulopathy and small vessels-related ischemic cardiomyopathy.The clinical significance of lupus non-inflammatory necrotizing vasculopathy (NINV) is not well established. For example, since lupus renal NINV is usually reported to coexist with proliferative and active glomerulonephritis, it is difficult to demonstrate the role of NINV on renal pathophysiology. Here we report a 16-year-old SLE boy with renal NINV presenting as ischemic glomerulopathy and small vessels-related ischemic heart failure. The renal biopsy demonstrated mild proliferative glomerulonephritis and NINV initially, and one month later repeated renal biopsy showed NINV with ischemic glomerulopathy. These findings established that NINV, but not proliferative glomerulonephritis, was responsive for his acute renal failure (ARF). Another interesting question is about the pathophysiology of his myocardial dysfunction. This patient presented typical angina and congestive heart failure (CHF). Echocardiograms and ventriculography revealed dilatation of four chambers and low ejection fraction. Serial electrocardiograms demonstrated evolutionary ischemic changes. coronary angiography revealed no abnormality of large vessels. These findings suggested small vascular lesions-induced myocardial ischemia was the underlying mechanism of dilated cardiomyopathy. As myocardial biopsy was not done in our case, we could only speculate, but not prove, that the NINV observed in renal biopsy may also involve in cardiac microvascular beds. Nevertheless, this interesting case emphasized the role of obliterative small vascular lesions in the pathophysiology of ARF and myocardial dysfunction. The patient was treated with high-dose corticosteroid, plasma infusion and hemodialysis. His cardiac function improved gradually, however the renal function did not recover.- - - - - - - - - - ranking = 6keywords = vessel (Clic here for more details about this article) |
7/60. Extracranial head and neck circulation aneurysms in a case of polyarteritis nodosa.polyarteritis nodosa (PAN) is a rare systemic necrotising medium vessel vasculitis. The hallmark of this condition is aneurysms which are seen in visceral arteries. Aneurysms of extracranial neck and head vessels are rare. We describe a case of PAN who had such aneurysms together with characteristic aneurysms in the mesenteric circulation.- - - - - - - - - - ranking = 2keywords = vessel (Clic here for more details about this article) |
8/60. central nervous system involvement in a child with polyarteritis nodosa and severe atopic dermatitis.polyarteritis nodosa is a diffuse vasculitis of small- and medium-sized muscular arteries that can involve the vessels of one or several organ systems. We report an 11-year-old boy with severe atopic dermatitis further complicated with erythroderma and cerebellar symptoms. Laboratory studies showed negative antinuclear antibodies and antineutrophil cytoplasmic antibodies; increased serum complement, IgG cryoglobulins 96.8 mg/dL (0-80) and serum IgG 2,160 mg/dL (613-1,295), and positive alpha-cardiolipin test. The IgE value was high. skin prick tests and RAST were positive for dust mites and casein. The viral profile was negative. Imaging studies disclosed tortuousity in the vessels and ischemic infarction in the cerebellum and thalamus. A diagnosis of polyarteritis nodosa with central nervous system involvement was made. The patient improved with immunosuppressive therapy and thalidomide. Currently, the patient is controlled and in a rehabilitation program. Other cases of polyarteritis nodosa associated with atopic dermatitis have not been reported.- - - - - - - - - - ranking = 2keywords = vessel (Clic here for more details about this article) |
9/60. Microscopic polyarteritis presenting with chest infections and acute appendicitis.We describe a 38-year-old male with antineutrophil cytoplasmic auto-antibody (ANCA) positive microscopic polyarteritis who presented with recurrent chest infections, lung haemorrhage, renal insufficiency and acute appendicitis. appendectomy was followed by resolution of abdominal symptoms and the surgical specimen revealed vasculitis of the serosal vessels. A renal biopsy was performed because of impaired renal function and this revealed focal necrotising glomerulonephritis with absence of immune deposits. Chest infections were treated with antibiotics resulting in partial clinical response, but pulmonary symptoms relapsed and a complete resolution was achieved only after plasma exchange and the administration of cyclophosphamide. Our observation emphasises the protean manifestations of microscopic polyarteritis and the relationship between ANCA and disease activity.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
10/60. N-butyl cyanoacrylate glue embolization of splenic artery aneurysms.A patient with polyarteritis nodosa presented with abdominal pain and low hematocrit level. Abdominal computed tomography (CT) depicted the presence of free blood and CT angiography revealed two aneurysms in the inferior branch of the splenic artery that were subsequently treated by endovascular transarterial embolization with N-Butyl Cyanoacrylate. Post embolization splenic arteriography demonstrated complete embolization of both aneurysms, including the inflow and outflow vessels.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
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