Cases reported "Polyarteritis Nodosa"

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1/81. Cutaneous polyarteritis nodosa: a case report and literature review.

    Cutaneous polyarteritis nodosa (CPN) is an uncommon form of vasculitis. It exists as a separate entity, though bearing similar name with polyarteritis nodosa (PAN) which is an aggressive systemic vasculitis with multi-organ involvement. CPN runs a chronic but benign course. Its aetiology is unknown and it usually presents with painful nodules on the legs with mild constitutional symptoms, and extracutaneous features of arthralgias, arthritis, neuropathy and myopathy. No mortality has been reported thus far. It is therefore important to distinguish CPN apart from PAN. Symptomatic treatment with judicial use of systemic steroids and anti-inflammatory agents will suffice in most cases.
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2/81. 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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3/81. Endoneurial vasculitis and tubuloreticular inclusions in peripheral nerve biopsy.

    We describe 3 patients in whom nerve biopsy revealed endothelial tubuloreticular inclusions in association with peripheral nerve endoneurial vasculitis. Two of the patients had systemic lupus erythematosus (SLE), while the third was hiv-positive. review of our biopsy material featuring the much more common finding of epineurial vasculitis failed to disclose any instances in which endothelial tubuloreticular inclusions (TRIs) were present. We conclude that TRIs and endoneurial vasculitis are closely associated. Moreover, if detected on a nerve biopsy specimen, TRIs are very suggestive of SLE or hiv infection. Finally, literature evidence is cited to suggest that an "acid-labile" alpha-interferon may be pathogenically related to the vasculitic process in these patients, perhaps through a process mediated by tumor necrosis factor.
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4/81. 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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5/81. (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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6/81. 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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7/81. cytomegalovirus infection in systemic necrotizing vasculitis: causative agent or opportunistic infection?

    We report on a 69-year-old woman who presented with myalgia, hearing impairment, fever, night sweats, weight loss, muscular weakness, paresthesia, hypesthesia, and hypalgesia. sural nerve biopsy showed demyelinative and axonal polyneuropathy due to necrotizing vasculitis with fibrinoid necrosis. A positive test for antineutrophil cytoplasmic antibodies (ANCA) with a perinuclear immunofluorescence pattern directed against myeloperoxidase was more suggestive of microscopic polyangiitis (MPA) than of polyarteritis nodosa (PAN), the possible differential diagnoses. In addition, positive tests for cytomegalovirus (CMV) antibodies (immunoglobulin (Ig)M and IgG) and the detection of CMV-dna in sputum specimens by polymerase chain reaction (PCR) were indicative of active CMV infection. Treatment with ganciclovir and anti-CMV immunoglobulin in addition to prednisolone medication for 6 months resulted in rapid improvement of the clinical symptoms without relapse. CMV infection has been described to be related to ANCA-associated vasculitis in non-immunocompromized patients and may be either a causative agent or an opportunistic infection. Identification of a viral etiology in patients with atypical ANCA-associated vasculitides may lead to different, less aggressive treatment approaches, including antiviral therapy.
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8/81. central nervous system involvement of polyarteritis nodosa: a case report.

    polyarteritis nodosa (PAN) is a necrotizing vasculitis involving small and medium-sized arteries and it affects multiple organ systems in the body central nervous system (CNS) involvement appears less frequently, and usually develops after the disease is established. Although aneurysms are common in visceral arteries in PAN, intracranial aneurysms are uncommon and have been documented rarely. This case is reported to raise awareness among radiologists as it has characteristic and rare, if not specific, imaging findings of CNS involvement of PAN.
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9/81. Choroidal infarction, anterior ischemic optic neuropathy, and central retinal artery occlusion from polyarteritis nodosa.

    PURPOSE: Ocular ischemia from polyarteritis nodosa (PAN) is rare. The authors present a case of multifocal ocular infarction from PAN. methods AND RESULTS: A 70-year-old woman developed hand and foot numbness followed by intermittent blurred vision and binocular horizontal diplopia. Two weeks later, she suddenly lost vision in the right eye from a central retinal artery occlusion and then developed a left anterior ischemic optic neuropathy and bilateral triangular choroidal abnormalities consistent with infarction. Her erythrocyte sedimentation rate and c-reactive protein were elevated. Although giant cell arteritis was suspected, a multiple mononeuropathy was demonstrated by electromyogram and nerve conduction velocity studies. biopsy specimens from her sural nerve and biceps muscle showed a necrotizing vasculitis with fibrinoid necrosis, consistent with PAN. CONCLUSIONS: polyarteritis nodosa can produce ischemia of a variety of ocular structures, including the retina, choroid, and optic nerve. In our patient, all three structures were affected. To our knowledge, this is the first reported case of the triangular sign of Amalric in PAN.
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10/81. 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.
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