BACKGROUND Although fluorodeoxyglucose-positron emission tomography/computed tomography(FDG-PET/CT)is widely used for diagnosis and follow-up of large sized vessel vasculitis,it is still not widely used for small to m...BACKGROUND Although fluorodeoxyglucose-positron emission tomography/computed tomography(FDG-PET/CT)is widely used for diagnosis and follow-up of large sized vessel vasculitis,it is still not widely used for small to medium sized vessel vasculitis.CASE SUMMARY This is the case of a 68-year-old male who presented at the emergency department complaining of fever,myalgia,and bilateral leg pain of over two weeks duration,with elevated levels of C-reactive protein.He was subsequently admitted and despite the absence of clinically significant findings,the patient continued to exhibit recurrent fever.A fever of unknown origin workup,which included imaging studies using FDG-PET/CT,revealed vasculitis involving small to medium-sized vessels of both lower extremities,demonstrated by linear hypermetabolism throughout the leg muscles.The patient was treated with methylprednisolone and methotrexate after diagnosis leading to the gradual resolution of the patient’s symptoms.Three weeks later,a follow-up FDGPET/CT was performed.Previously hypermetabolic vessels were markedly improved.CONCLUSION Our case report demonstrated that FDG-PET/CT has tremendous potential to detect medium-sized vessel inflammation;it can also play a crucial role in prognosticating outcomes and monitoring therapeutic efficacy.展开更多
Here, we present the case of a 74-year-old Japanese man with segmental intestinal necrosis, which developed after treatment with pulsed methylprednisolone for mononeuritis multiplex. The patient was weakly positive fo...Here, we present the case of a 74-year-old Japanese man with segmental intestinal necrosis, which developed after treatment with pulsed methylprednisolone for mononeuritis multiplex. The patient was weakly positive for myeloperoxidase (MPO)-anti-neutrophil cytoplasmic antibody (ANCA). Computed tomography and surgical findings were compatible with nonocclusive mesenteric ischemia (NOMI). He underwent small intestinal resection by emergency surgery and an intestinal fistula was made. Pathologically, necrotizing vasculitis with fibrinoid necrosis was present in medium to small-sized arteries, which was equivalent to Arkin's classification Ⅱ-Ⅳ. Most of the arteries had fibrous intimal thickening, which was considered to obstruct the arteries and thus cause segmental intestinal necrosis. A diagnosis of polyarteritis nodosa (PAN) was made, and intravenous cyclophosphamide pulse therapy was added to the therapeutic regimen. This patient was successfully treated with these multidisciplinary therapies and his stoma was finally closed. This is a very rare and indicative case of PAN weakly positive for MPO-ANCA and clinically mimicking NOMI, which occurred even after treatment with pulsed methylprednisolone.展开更多
The differential diagnosis of acute abdomen is typically extremely broad in range, with vasculitis posing a rare but potentially life-threatening cause of acute abdomen. Here, we report a case of acute abdomen with bo...The differential diagnosis of acute abdomen is typically extremely broad in range, with vasculitis posing a rare but potentially life-threatening cause of acute abdomen. Here, we report a case of acute abdomen with bowel wall thickening limited to jejunum, accompanied by unexplained renal dysfunction. Later, the patient was diagnosed as having polyarteritis nodosa based on surgically resected jejunal necrosis. Despite aggressive treatment, including the use of steroid pulse therapy and continuous hemodiafiltration, the patient died. Although polyarteritis nodosa is extremely rare in patients with acute abdomen, acute abdomen is relatively common manifestation of that. And it is reported that involvement of small intestine suggests poorer prognosis. Our case highlights the importance of vasculitis as a differential diagnosis of patients with atypical acute abdomen. In this report, we not only review possible clues that might have led to an earlier diagnosis in this case, but also attempt to draw some lessons for treating similar cases in the future.展开更多
Classic polyarteritis nodosa(PAN) that targets mediumsized muscular arteries and microscopic polyangiitis(MPA),characterized by inflammation of small-caliber vessels and the presence of circulating myeloperoxidase ant...Classic polyarteritis nodosa(PAN) that targets mediumsized muscular arteries and microscopic polyangiitis(MPA),characterized by inflammation of small-caliber vessels and the presence of circulating myeloperoxidase anti-neutrophil cytoplasmic antibodies(MPO-ANCA),are distinct clinicopathological entities of systemic vasculitis.A 66-year-old woman presented with fever,cholestasis and positive MPO-ANCA.Radiological examination showed a pancreatic mass compressing the bile duct.Therefore,we performed pancreatoduodenectomy.Histopathological examination revealed that necrotizing vasculitis predominantly affecting the medium-sized vessels,spared arterioles or capillaries in the pancreas,a finding consistent with PAN.Unexpectedly,renal biopsy revealed small-caliber vasculitis and glomerulonephritis,supporting MPA.The initial manifestation of a pancreatic mass associated with vasculitis has only been reported in 7 articles.Its diagnosis is challenging because no reliable clinico-radiological findings have been observed.Clinicians should be aware of such cases and early diagnosis followed by immunosuppression is mandatory.Our findings may reflect a polyangiitis overlap syndrome coexisting between pancreatic PAN and renal MPA.展开更多
Chronic hepatitis caused by hepatitis B virus(HBV) is an endemic disease in India. It is associated with extrahepatic manifestations like polyarteritis nodosa(PAN) which is a vasculitis like disorder, presenting in su...Chronic hepatitis caused by hepatitis B virus(HBV) is an endemic disease in India. It is associated with extrahepatic manifestations like polyarteritis nodosa(PAN) which is a vasculitis like disorder, presenting in subacute or chronic phase; involving visceral and systemic vessels. It should always be considered as a possible etiology of hypertension in an underlying setting of hepatitis B. We describe a 56-year-male patient with a history of chronic HBV who presented to the outpatient clinic with history of recent onset hypertension and suspected liver disease. Further work up for the cause of recent hypertension included a contrast computerized tomography of abdomen, which revealed concomitant pathologies of chronic liver disease and multiple aneurysms in bilateral kidneys. This case illustrates the unusual presentation of extrahepatic manifestation of viral hepatitis in the form of PAN of kidneys. PAN as an independent entity may be missed in specialized clinics evaluating liver pathologies, due to its insidious onset, atypical clinical symptoms and multi-systemic manifestations. The knowledge of extrahepatic, renal and vascular manifestations of hepatitis B unrelated to liver disease should be considered by physicians at the time of diagnosis and management of patients with HBV.展开更多
Polyarteritis nodosa(PAN) is one of the systemic vasculitis that affects the media wall of arteries of small and medium diameter. Diagnosis proves difficult due to the unspecific symptoms that dominate the clinical pr...Polyarteritis nodosa(PAN) is one of the systemic vasculitis that affects the media wall of arteries of small and medium diameter. Diagnosis proves difficult due to the unspecific symptoms that dominate the clinical profile. Liver involvement is very diverse, ranging from the development of cirrhotic liver disease to acute abdomen presentation that requires surgery because of liver rupture. The management of these patients requires an expert multidisciplinary team. There are several cases in the literature that describe a sudden liver rupture as the first manifestation of a PAN. In this paper we present the case of a 75 years old patient without any previous disease, who is subjected to major hepatic resection for spontaneous liver rupture.展开更多
Polyarteritis nodosa (PAN) affects mostly medium-sized arteries and sometimes small ones as well. The primary methods used to make the diagnosis are through physical examinations, biopsies of organs that are affected,...Polyarteritis nodosa (PAN) affects mostly medium-sized arteries and sometimes small ones as well. The primary methods used to make the diagnosis are through physical examinations, biopsies of organs that are affected, and/or angiographic studies. Immunosuppressants like glucocorticoids and cyclophosphamide are usually started as soon as possible after a diagnosis. So, it’s not clear if sudden remission of PAN happens or not. Here we present a 42-year-old male who presented with right upper quadrant, right flank pain and fever. CT angiogram Aorta revealed soft tissue rind around the small to medium sized vessel in the abdomen and bilateral cortical renal infarcts of variable age in the right more than the left. A diagnosis of polyarteritis nodosa was made and the patient achieved spontaneous remission with no need for corticosteroids or immunosuppressive therapy.展开更多
Human hepatitis viruses(HHVs)include hepatitis A virus,hepatitis B virus(HBV),hepatitis C virus(HCV),hepatitis delta virus,and hepatitis E virus and can cause liver inflammation in their common human host.Usually,HHV ...Human hepatitis viruses(HHVs)include hepatitis A virus,hepatitis B virus(HBV),hepatitis C virus(HCV),hepatitis delta virus,and hepatitis E virus and can cause liver inflammation in their common human host.Usually,HHV is rapidly cleared by the immune system,following acute HHV invasion.The morbidities associated with hepatitis A virus and hepatitis E virus infection occur shortly after their intrusion,in the acute stage.Nevertheless,the viral infectious process can persist for a long period of time,especially in HBV and HCV infection,leading to chronic hepatitis and further progressing to hepatic cirrhosis and liver cancer.HHV infection brings about complications in other organs,and both acute and chronic hepatitis have been associated with clinical presentations outside the liver.Vascular involvement with cutaneous and systemic vasculitis is a well-known extrahepatic presentation;moreover,there is growing evidence for a possible causal relationship between viral pathogens and vasculitis.Except for hepatitis delta virus,other HHVs have participated in the etiopathogenesis of cutaneous and systemic vasculitis via different mechanisms,including direct viral invasion of vascular endothelial cells,immune complex-mediated vessel wall damage,and autoimmune responses with stimulation of autoreactive B-cells and impaired regulatory T-cells.Cryoglobulinemic vasculitis and polyarteritis nodosa are recognized for their association with chronic HHV infection.Although therapeutic guidelines for HHV-associated vasculitis have not yet been established,antiviral therapy should be initiated in HBV and HCV-related systemic vasculitis in addition to the use of corticosteroids.Plasma exchange and/or combined cyclophosphamide and corticosteroid therapy can be considered in patients with severe life-threatening vasculitis manifestations.展开更多
The primary systemic vasculitides(PSV) are a group of rare inflammatory disorders affecting blood vessels of varying size and multiple organs. Urological manifestations of PSV are uncommon. Testicular vasculitis is th...The primary systemic vasculitides(PSV) are a group of rare inflammatory disorders affecting blood vessels of varying size and multiple organs. Urological manifestations of PSV are uncommon. Testicular vasculitis is the most commonly reported finding and is associated with Polyarteritis Nodosa(PAN), Henoch-Sch?nleinPurpura(HSP), anti-neutrophil cytoplasm antibody associated Vasculitides(AAV), Giant Cell Arteritis(GCA) and Kawasaki disease. Prostatic vasculitis has been reported in association with GCA and AAV. Ureteric involvement has been noted in PAN, HSP and AAV. Other urogenital manifestations of PSV include genital ulceration and bladder dysfunction in Beh?ets Disease and haematuria which is commonly seen in many of the PSV. Finally, therapies used to treat the PSV, especially cyclophosphamide, are associated with urological sideeffects including haemorrhagic cystitis and urothelial malignancy. The aim of this review is to examine how the urological system is involved in the PSV. Each PSV is examined in turn, with a brief clinical description of the disease followed by a description of the urological manifestations and management. Identification of urological manifestations of PSV is important as in many cases symptoms may improve with immunosuppressive therapy, avoiding the need for invasive surgery. Additionally, patients who present with isolated urogenital PSV are at higher risk of developing subsequent systemic vasculitis and will need to be followed up closely.展开更多
文摘BACKGROUND Although fluorodeoxyglucose-positron emission tomography/computed tomography(FDG-PET/CT)is widely used for diagnosis and follow-up of large sized vessel vasculitis,it is still not widely used for small to medium sized vessel vasculitis.CASE SUMMARY This is the case of a 68-year-old male who presented at the emergency department complaining of fever,myalgia,and bilateral leg pain of over two weeks duration,with elevated levels of C-reactive protein.He was subsequently admitted and despite the absence of clinically significant findings,the patient continued to exhibit recurrent fever.A fever of unknown origin workup,which included imaging studies using FDG-PET/CT,revealed vasculitis involving small to medium-sized vessels of both lower extremities,demonstrated by linear hypermetabolism throughout the leg muscles.The patient was treated with methylprednisolone and methotrexate after diagnosis leading to the gradual resolution of the patient’s symptoms.Three weeks later,a follow-up FDGPET/CT was performed.Previously hypermetabolic vessels were markedly improved.CONCLUSION Our case report demonstrated that FDG-PET/CT has tremendous potential to detect medium-sized vessel inflammation;it can also play a crucial role in prognosticating outcomes and monitoring therapeutic efficacy.
文摘Here, we present the case of a 74-year-old Japanese man with segmental intestinal necrosis, which developed after treatment with pulsed methylprednisolone for mononeuritis multiplex. The patient was weakly positive for myeloperoxidase (MPO)-anti-neutrophil cytoplasmic antibody (ANCA). Computed tomography and surgical findings were compatible with nonocclusive mesenteric ischemia (NOMI). He underwent small intestinal resection by emergency surgery and an intestinal fistula was made. Pathologically, necrotizing vasculitis with fibrinoid necrosis was present in medium to small-sized arteries, which was equivalent to Arkin's classification Ⅱ-Ⅳ. Most of the arteries had fibrous intimal thickening, which was considered to obstruct the arteries and thus cause segmental intestinal necrosis. A diagnosis of polyarteritis nodosa (PAN) was made, and intravenous cyclophosphamide pulse therapy was added to the therapeutic regimen. This patient was successfully treated with these multidisciplinary therapies and his stoma was finally closed. This is a very rare and indicative case of PAN weakly positive for MPO-ANCA and clinically mimicking NOMI, which occurred even after treatment with pulsed methylprednisolone.
文摘The differential diagnosis of acute abdomen is typically extremely broad in range, with vasculitis posing a rare but potentially life-threatening cause of acute abdomen. Here, we report a case of acute abdomen with bowel wall thickening limited to jejunum, accompanied by unexplained renal dysfunction. Later, the patient was diagnosed as having polyarteritis nodosa based on surgically resected jejunal necrosis. Despite aggressive treatment, including the use of steroid pulse therapy and continuous hemodiafiltration, the patient died. Although polyarteritis nodosa is extremely rare in patients with acute abdomen, acute abdomen is relatively common manifestation of that. And it is reported that involvement of small intestine suggests poorer prognosis. Our case highlights the importance of vasculitis as a differential diagnosis of patients with atypical acute abdomen. In this report, we not only review possible clues that might have led to an earlier diagnosis in this case, but also attempt to draw some lessons for treating similar cases in the future.
文摘Classic polyarteritis nodosa(PAN) that targets mediumsized muscular arteries and microscopic polyangiitis(MPA),characterized by inflammation of small-caliber vessels and the presence of circulating myeloperoxidase anti-neutrophil cytoplasmic antibodies(MPO-ANCA),are distinct clinicopathological entities of systemic vasculitis.A 66-year-old woman presented with fever,cholestasis and positive MPO-ANCA.Radiological examination showed a pancreatic mass compressing the bile duct.Therefore,we performed pancreatoduodenectomy.Histopathological examination revealed that necrotizing vasculitis predominantly affecting the medium-sized vessels,spared arterioles or capillaries in the pancreas,a finding consistent with PAN.Unexpectedly,renal biopsy revealed small-caliber vasculitis and glomerulonephritis,supporting MPA.The initial manifestation of a pancreatic mass associated with vasculitis has only been reported in 7 articles.Its diagnosis is challenging because no reliable clinico-radiological findings have been observed.Clinicians should be aware of such cases and early diagnosis followed by immunosuppression is mandatory.Our findings may reflect a polyangiitis overlap syndrome coexisting between pancreatic PAN and renal MPA.
文摘Chronic hepatitis caused by hepatitis B virus(HBV) is an endemic disease in India. It is associated with extrahepatic manifestations like polyarteritis nodosa(PAN) which is a vasculitis like disorder, presenting in subacute or chronic phase; involving visceral and systemic vessels. It should always be considered as a possible etiology of hypertension in an underlying setting of hepatitis B. We describe a 56-year-male patient with a history of chronic HBV who presented to the outpatient clinic with history of recent onset hypertension and suspected liver disease. Further work up for the cause of recent hypertension included a contrast computerized tomography of abdomen, which revealed concomitant pathologies of chronic liver disease and multiple aneurysms in bilateral kidneys. This case illustrates the unusual presentation of extrahepatic manifestation of viral hepatitis in the form of PAN of kidneys. PAN as an independent entity may be missed in specialized clinics evaluating liver pathologies, due to its insidious onset, atypical clinical symptoms and multi-systemic manifestations. The knowledge of extrahepatic, renal and vascular manifestations of hepatitis B unrelated to liver disease should be considered by physicians at the time of diagnosis and management of patients with HBV.
基金supported by Reina Sofía University Hospital,14004 Córdoba,Spain(Andalusia Public Health Service)
文摘Polyarteritis nodosa(PAN) is one of the systemic vasculitis that affects the media wall of arteries of small and medium diameter. Diagnosis proves difficult due to the unspecific symptoms that dominate the clinical profile. Liver involvement is very diverse, ranging from the development of cirrhotic liver disease to acute abdomen presentation that requires surgery because of liver rupture. The management of these patients requires an expert multidisciplinary team. There are several cases in the literature that describe a sudden liver rupture as the first manifestation of a PAN. In this paper we present the case of a 75 years old patient without any previous disease, who is subjected to major hepatic resection for spontaneous liver rupture.
文摘Polyarteritis nodosa (PAN) affects mostly medium-sized arteries and sometimes small ones as well. The primary methods used to make the diagnosis are through physical examinations, biopsies of organs that are affected, and/or angiographic studies. Immunosuppressants like glucocorticoids and cyclophosphamide are usually started as soon as possible after a diagnosis. So, it’s not clear if sudden remission of PAN happens or not. Here we present a 42-year-old male who presented with right upper quadrant, right flank pain and fever. CT angiogram Aorta revealed soft tissue rind around the small to medium sized vessel in the abdomen and bilateral cortical renal infarcts of variable age in the right more than the left. A diagnosis of polyarteritis nodosa was made and the patient achieved spontaneous remission with no need for corticosteroids or immunosuppressive therapy.
基金The Institutional Review Board of National Cheng Kung University Hospital approved this study(No.B-ER-105-108).
文摘Human hepatitis viruses(HHVs)include hepatitis A virus,hepatitis B virus(HBV),hepatitis C virus(HCV),hepatitis delta virus,and hepatitis E virus and can cause liver inflammation in their common human host.Usually,HHV is rapidly cleared by the immune system,following acute HHV invasion.The morbidities associated with hepatitis A virus and hepatitis E virus infection occur shortly after their intrusion,in the acute stage.Nevertheless,the viral infectious process can persist for a long period of time,especially in HBV and HCV infection,leading to chronic hepatitis and further progressing to hepatic cirrhosis and liver cancer.HHV infection brings about complications in other organs,and both acute and chronic hepatitis have been associated with clinical presentations outside the liver.Vascular involvement with cutaneous and systemic vasculitis is a well-known extrahepatic presentation;moreover,there is growing evidence for a possible causal relationship between viral pathogens and vasculitis.Except for hepatitis delta virus,other HHVs have participated in the etiopathogenesis of cutaneous and systemic vasculitis via different mechanisms,including direct viral invasion of vascular endothelial cells,immune complex-mediated vessel wall damage,and autoimmune responses with stimulation of autoreactive B-cells and impaired regulatory T-cells.Cryoglobulinemic vasculitis and polyarteritis nodosa are recognized for their association with chronic HHV infection.Although therapeutic guidelines for HHV-associated vasculitis have not yet been established,antiviral therapy should be initiated in HBV and HCV-related systemic vasculitis in addition to the use of corticosteroids.Plasma exchange and/or combined cyclophosphamide and corticosteroid therapy can be considered in patients with severe life-threatening vasculitis manifestations.
文摘The primary systemic vasculitides(PSV) are a group of rare inflammatory disorders affecting blood vessels of varying size and multiple organs. Urological manifestations of PSV are uncommon. Testicular vasculitis is the most commonly reported finding and is associated with Polyarteritis Nodosa(PAN), Henoch-Sch?nleinPurpura(HSP), anti-neutrophil cytoplasm antibody associated Vasculitides(AAV), Giant Cell Arteritis(GCA) and Kawasaki disease. Prostatic vasculitis has been reported in association with GCA and AAV. Ureteric involvement has been noted in PAN, HSP and AAV. Other urogenital manifestations of PSV include genital ulceration and bladder dysfunction in Beh?ets Disease and haematuria which is commonly seen in many of the PSV. Finally, therapies used to treat the PSV, especially cyclophosphamide, are associated with urological sideeffects including haemorrhagic cystitis and urothelial malignancy. The aim of this review is to examine how the urological system is involved in the PSV. Each PSV is examined in turn, with a brief clinical description of the disease followed by a description of the urological manifestations and management. Identification of urological manifestations of PSV is important as in many cases symptoms may improve with immunosuppressive therapy, avoiding the need for invasive surgery. Additionally, patients who present with isolated urogenital PSV are at higher risk of developing subsequent systemic vasculitis and will need to be followed up closely.
