Cases reported "Polyarteritis Nodosa"

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1/139. Acute pancreatitis as a complication of polyarteritis nodosa.

    CONCLUSIONS: polyarteritis nodosa (PAN) must be considered as one of the rare causes of "idiopathic" acute necrotizing pancreatitis. BACKGROUND: PAN is characterized by panmural inflammation of arterioles causing arteriolar ectasia, aneurysm formation, and thrombosis, resulting in organ ischemia. methods: We report a case of necrotizing pancreatitis associated with segmental necrosis of the liver and spleen due to polyarteritis nodosa. RESULTS: Five previously reported cases of documented acute pancreatitis secondary to PAN have been identified from the English literature. The mechanism through which pancreatic ischemia results in acute pancreatitis is unknown. Although limited pancreatic infarction is common in PAN, necrotizing pancreatitis is rare, and the poor overall prognosis of PAN is owing largely to other organ complications.
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2/139. Isolated periarteritis nodosa of the spermatic cord presenting as a scrotal mass: report of a case.

    We report a 57-year-old man who visited a local hospital because of a fist-sized swelling and pain in his right scrotum. Surgically resected tissue revealed necrotizing arteritis in a small spermatic artery. As systemic symptoms for angiitis were not present, this is thought to be the second case of isolated angiitis occurring in the spermatic cord.
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3/139. Primary polyarteritis nodosa presenting as acute symmetric quadriplegia.

    We report a case of peripheral neuropathy presenting as acute symmetric areflexic quadriplegia in the setting of a well-defined clinical, histopathologic, and angiographic diagnosis of classic polyarteritis nodosa. While it is usually easy to recognize the typical clinical presentation of necrotizing angiopathy-induced peripheral neuropathy as a mononeuritis multiplex or a distal polyneuropathy in association with a collagen vascular disease, clinicians must be equally sensitive to a number of more challenging possibilities. Acute quadriplegia similar to that seen in guillain-barre syndrome can be secondary to primary classic polyarteritis nodosa and the former may be the chief or even the sole manifestation of the latter.
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4/139. Polyarthritis nodosa with mesenteric aneurysms demonstrated by angiography: report of a case and successful treatment of the patient with prednisolone and cyclophosphamide.

    polyarteritis nodosa is a necrotizing angitis that predominantly affects small and medium-sized arteries. The prognosis of untreated polyarteritis nodosa is very poor. Since symptoms are diverse and no serologic test is specific for polyarteritis nodosa, the diagnosis is difficult and often delayed. We describe a patient with polyarteritis nodosa who had gastrointestinal involvement with multiple aneurysms of the inferior mesenteric artery; only abdominal angiography provided a conclusive diagnosis. Alleviation of symptoms and regression of aneurysms were observed after combination therapy of an immunosuppressive agent, cyclophosphamide, and prednisolone. We emphasize the importance of early diagnosis by angiography and aggressive therapy in patients in whom physical signs indicating definite polyarteritis nodosa are not present.
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5/139. Necroqizing enterocolitis with pneumatosis intestinalis in systemic lupus erythematosus and polyarteritis.

    Pneumatosis intestinalis was encountered in association with fatal necrotizing enterocolitis in systemic lupus erythematosus (SLE) and polyarteritis nodosa. The radiologic identification of mottled, bubbly, and linear collections of intramural intestinal gas distinguish this ominous complication from benign pneumatosis cystoides intestinalis. In the setting of intestinal vasculitis due to SLE or polyarteritis nodosa, these characteristic radiologic features indicate necrotizing enterocolitis. Since corticosteroids may mask clinical progression of the intestinal lesion, radiologic evaluation is essential in the overall management of the patient with intestinal vasculitis.
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6/139. Acquired bernard-soulier syndrome: a case with necrotizing vasculitis and thrombosis.

    We describe a patient with positive antinuclear antibodies, polyclonal gammopathy and high level of circulating immunocomplexes, resulting in vascular purpura. In addition, the patient had a slightly prolonged bleeding time and an isolated defect of ristocetin-induced platelet aggregation (RIPA) in platelet-rich plasma (PRP). The patient's plasma also inhibited RIPA in normal PRP and in normal platelet suspension. The activity and multimeric structure of plasmatic von willebrand factor showed no alteration. We could demonstrate an autoantibody against platelet membrane glycoprotein (GP) Ib, using an ELISA-type assay. These data suggest an acquired bernard-soulier syndrome. We suggest that the patient had an immunocomplex-mediated leukocytoclastic vasculitis accompanied by production of antinuclear autoantibodies as well as the presence of an autoantibody against GPIb. The titer of the anti-GPIb antibody, however, was too low to induce significant platelet-type bleeding tendency, only laboratory alterations were found. Moreover, in a later stage of her disease, she developed a severe necrotizing vasculitis which was followed by a deep venous thrombosis.
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7/139. polyarteritis nodosa presenting as massive upper gastrointestinal hemorrhage.

    We report the first case of massive upper gastrointestinal hemorrhage as the initial presentation of polyarteritis nodosa (PAN), which is an uncommon form of systemic necrotizing vasculitis that may involve many organ systems and could affect any age group. abdominal pain is the most common sign of gastrointestinal involvement. Gastrointestinal bleeding occurs less frequently in approximately 6% of cases. Reported cases of gastrointestinal hemorrhage have been in the form of coffee ground emesis, melena, or hematochezia. Such bleeding complications have resulted from ischemic ulceration or perforation of the small or large intestine. However, we are unaware of previous reports showing massive hematemesis to be the initial presentation of PAN.
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8/139. (A)symptomatic necrotizing arteritis of the female genital tract.

    AIMS: The vasculitides represent a heterogenous set of disorders that differ in prognosis and response to therapy. Beside systemic vasculitides, the development of localized forms of arteritis is well known though uncommon and the etiopathogenesis is not yet definitely clear. methods: patients with necrotizing arteritis of the female genital tract proven by histology are studied in a retrospective analysis. RESULTS: Three cases of necrotizing arteritis with histological features of panarteritis nodosa apparently confined to the female genital tract are presented. None of these patients had prior history of systemic vasculitis. The acute necrotizing vasculitis was confined only to the uterine cervix in two patients and involved all the internal genital organs in the third patient. The patients have been observed for up to 4 years without any therapy for these lesions and without any manifestation of systemic vasculitic progression. CONCLUSION: It is to speculate that focal arteritis of the female genital tract is a benign form of panarteritis nodosa or moreover a totally different entity with identical morphogenesis but possibly different pathogenesis. Furthermore it seems to be important to be aware of the specificity of focal arteritis in female genital tract as distinct from the generalized form to prevent unnecessary surgical or chemotherapeutical therapy for this lesion. The benign entity of local arteritis in the female genital tract is discussed in contrast to the severe prognosis of systemic panarteritis nodosa.
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9/139. Juvenile relapsing periarteritis nodosa and streptococcal infection.

    Classic polyarteritis nodosa is a multisystem inflammatory disease associated with necrotizing vasculitis of small and medium arteries. In most cases, the causes of polyarteritis nodosa remain unknown, but viruses (HBV, HCV, hiv) and microbes (especially streptococcus) have been considered as etiologic or contributing factors. A 13-year-old boy was admitted with fever, skin lesions, polyarthritis and muscle involvement. A muscle biopsy demonstrated a necrotizing vasculitis and antistreptolysin titre was tremendously increased. His condition improved following the administration of oral steroids but he experienced relapses 5 and 12 years later when penicillin withdrawal was attempted. The flares were accompanied by a major increase of antistreptolysin titre and response to corticosteroid was obtained. He is currently 38 years old and he remains well on prophylactic penicillin. polyarteritis nodosa in children may occur after a streptococcal infection. It may be prudent to consider penicillin prophylaxis in patients with periarteritis nodosa when a streptococcal etiology is documented or highly suspected.
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10/139. Sensory ataxic dominant neuropathy associated with polyarteritis nodosa.

    A 68-year-old man with sensory ataxic dominant neuropathy associated with polyarteritis nodosa (PAN) had deep sensory disturbance with unsteady gait and absence of the achilles tendon reflex. Examination revealed weight loss, elevated CRP level, negative antineutrophil cytoplasm antibodies, decreased M-wave amplitude in the peroneal motor nerve and absence of action potentials in the sural sensory nerve. sural nerve biopsy revealed a marked loss of myelinated fibers, myelin ovoid formation and necrotizing angiitis of large epineurial arterioles. Renal biopsy revealed global and/or segmental necrotizing angiitis in glomeruli, but not in the arcuate artery. These pathological findings were distinct from those of PAN, particularly microscopic polyangiitis (MPA). Treatment with a steroid improved the deep sensory disturbance, unsteady gait, and CRP level. This case is similar to ataxic neuropathy which can arise from various disorders. This is the first report of a case of sensory ataxic dominant neuropathy associated with MPA in PAN.
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