结节性多动脉炎的诊断和治疗进展

结节性多动脉炎（PAN）是最早被阐述的系统性血管炎，是累及中等大小动脉或小动脉的一种坏死性血管炎，常有多系统受累。临床表现复杂且缺乏特异性，往往没有肾小球肾炎、肺损害及抗中性粒细胞胞浆抗体（ANCA）。PAN的诊断依赖于临床表现、实验室检查以及病理检查，找出其重要线索如：周围神经病变、胃肠道损害、高血压、HBsAg阳性等才能作出正确诊断。PAN的治疗主要是肾上腺糖皮质激素，对有重要脏器损害的患者使用细胞毒药物和血浆置换也是必要的。     

Cryofibrinogenemia associated with polyarteritis nodosa

We report the case of a 77-year-old woman with biopsy-confirmed polyarteritis nodosa (PAN) associated with cryofibrinogenemia presenting with polyarthralgia and digital gangrene induced by cold exposure. The clinical manifestations and parameters measured by laboratory markers including cryofibrinogen responded well to corticosteroid therapy. To our knowledge, the case of the combination of PAN and cryofibrinogenemia has not been reported. Our case indicates that cryofibrinogenemia might be associated with PAN. The PAN patients with cold-induced symptoms should be screened for cryofibrinogen.     

Acute pachymeningitis mimicking subdural hematoma in a patient with polyarteritis nodosa

Pachymeningitis is a very rare neurologic manifestation of polyarteritis nodosa (PAN). This report describes a case of acute pachymeningitis that was, initially, misdiagnosed as subdural hematoma on the brain CT of a patient with PAN. A 45-year-old man, who had been diagnosed as having PAN 6 months previously, came back to the emergency room with complaints of sudden headache, nausea, vomiting, and diplopia for 3 days before his hospital admission. Initially, the noncontrast enhanced brain CT findings showed high densities in the bilateral tentorial and posterior parafalcial area, which suggested a small amount of subdural hematoma. However, the subsequent MRI findings revealed pachymeningitis of the bilateral tentoria and falx. He was treated with high-dose steroid and cyclophosphamide pulse therapy. Thereafter, his symptoms gradually resolved.     

Outcome of polyarteritis nodosa in northern India

Over the last 10 years, 17 patients (13 males and 4 females) diagnosed as having classical polyarteritis nodosa (PAN) were treated and followed up in the rheumatology clinic of our institute. The median age and duration of symptoms at presentation were 29 years (range 13–59) and 9.5 months, respectively. Patients presented with the typical clinical picture of classical PAN. The diagnosis was established with the help of an aortogram (7/10), sural nerve biopsy (7/8), muscle biopsy (5/7) and renal biopsy (3/4). For reasons not known, none of the 17 patients was HBsAg positive. Patients were treated with a combination of oral prednisolone (1 mg/kg per day) for 6 weeks, which was slowly tapered off over 6 months, and monthly intravenous cyclophosphamide pulses (15 mg/kg) for the first 6 months, followed by 3-monthly pulses for a total of 2 years. Remission was achieved in 14 patients after a median of 5 months of treatment. Remission was stable for a median of 5 years of follow-up. Three patients did not respond well and died within 6 months of diagnosis. The causes of death in these were intracerebral haemorrhage in one patient and gastrointestinal bleeding in two patients. This experience is in accord with the reported literature that classical PAN is mostly a monophasic disease with either an excellent response to the appropriate immunosuppressive therapy and a long remission or a downhill course culminating in death. A chronic course is rare. Received: 8 October 1996 / Accepted: 4 April 1997     

结节性多动脉炎合并心肌梗死8例临床分析

目的总结结节性多动脉炎累及冠状动脉导致心肌梗死患者的临床表现及冠状动脉受累特点。方法对北京协和医院有病案记录的结节性多动脉炎合并心肌梗死的8例患者的临床症状、系统受累、实验室检查、冠状动脉及其他血管造影、超声心动图和病理检查等进行回顾性分析。结果 8例患者中男性5例,女性3例,年龄21～52岁,平均(37.6±11.7)岁。胸痛6例,心力衰竭1例。心电图缺血性ST-T改变5例,除窦性心动过速、房性期前收缩外,未见其他恶性心律失常。左心室射血分数降低(≤50%)3例,节段性室壁运动异常6例,室壁瘤形成2例,心肌病变2例,肺高压1例。4例有冠状动脉影像学检查资料,均累及右冠状动脉,3例为三支病变,1例为两支病变,其造影结果描述为冠状动脉弥漫性病变、冠状动脉扩张、多发动脉瘤以及节段性狭窄、闭塞。这些患者均有其他多部位血管受累的表现和动脉造影检查的异常。结论结节性多动脉炎可累及冠状动脉导致心肌梗死,其冠状动脉受累常为多支病变、多有右冠状动脉受累,动脉瘤伴血栓形成和节段性狭窄。对冠心病低危心肌梗死的患者,需要完善血管检查,警惕结节性多动脉炎累及冠状动脉的情况。     

High-dose intravenous immunoglobulin infusion in polyarteritis nodosa

We describe a 58-year-old Japanese female who developed polyarteritis nodosa (PN). Her skin disease and systemic symptoms were resistant to dapsone (1.5 mg kg⁻¹ day⁻¹), high-dose oral prednisone (1 mg kg⁻¹ day⁻¹) and azathioprine (2 mg kg⁻¹ day⁻¹), and intravenous cyclophosphamide pulse therapy (10 mg kg⁻¹ day⁻¹). She was ultimately treated with infusion of high-dose intravenous immunoglobulin (IVIG) at a dose of 0.1 g kg⁻¹ daily for five consecutive days weekly for a period of 12 weeks, resulting in remission of his cutaneous and systemic symptoms and successful tapering of his prednisone and azathioprine dose. However, 12 months later, relapsing fever and polyarthritis recurred, and eventually, 24 months later, indurated erythema and punched-out ulcers appeared on the lower legs. These symptoms were reduced after increasing the dose of oral prednisone (1 mg kg⁻¹ day⁻¹). Our case indicates that the high-dose IVIG infusion therapy may be useful for controlling PN in certain periods since the long-term observation revealed deterioration of symptoms. We review related articles and discuss its effectiveness in PN.     

Overlap between systemic sclerosis and polyarteritis nodosa: A case report

BackgroundSystemic sclerosis (SSc) is a multiorgan connective tissue disease characterized by vasculopathy, inflammation, autoimmunity, and fibrosis in the skin, lungs and other organs. The occurrence of frank vasculitis is uncommon.Case presentationA 36-year old male patient with limited cutaneous SSc developed multiple necrotic ulcers on both legs and feet and gangrene of several toes, followed by an acute onset of axonal sensorimotor neuropathy affecting both radial and peroneal nerves, severe testicular pain with gangrenous patches over the scrotum. The hepatitis B virus (HBV) core antibody was positive while HB surface antigen and surface antibody, HAV and HCV antibodies were negative. The polymerase chain reaction for HBV and HCV showed no detectable viraemia. Antineutrophil cytoplasmic antibodies, cryoblobulins, anticardiolipin antibodies, lupus anticoagulant, antimitochondrial and anti- liver-kidney microsomal antibodies were negative. Pelvi-abdominal ultrasound and portal vein Doppler study showed a coarse and heterogeneous echo-texture of the liver, splenomegaly, moderate ascites and an enlarged, patent portal vein. Fibroscan revealed grade III liver fibrosis. He had an attack of haematemesis with elevation of the liver enzymes and low serum albumin and prothrombin concentrations. He was diagnosed as a case of polyarteritis nodosa. He was successfully treated by methylprednisolone intravenous pulses, followed by oral prednisone 40 mg/day. Plasmapheresis and six monthly doses of 1000 mg intravenous cyclophosphamide. Prednisone was gradually tapered to 5 mg/day with addition of azathioprine 100 mg/day.ConclusionThe association between systemic sclerosis and polyarteritis nodosa is very rare. The co-existence of SSc and vasculitis necessitates modification of the treatment plan.     

A case of refractory polyarteritis nodosa successfully treated with rituximab

A 59-year-old man who presented with continuous fever, livedo reticularis, and left leg ischemia with multiple tibial artery stenosis and renal artery aneurysm, as demonstrated by arteriography, was diagnosed with polyarteritis nodosa (PAN) 6 years ago. Although he frequently relapsed in spite of intensive immunosuppressive therapies, the disease activity of PAN was controlled with repeated rituximab (RTX) therapies and steroid doses were tapered safely. Peripheral CD19⁺ B-cells disappeared soon after the 1st administration of RTX. Although CD19⁺ B-cells remained absent, 3.1% of CD3⁺CD20⁺ T-cells were observed in the peripheral blood prior to the 2nd administration of RTX. Recent studies have suggested the pathogenic role of CD3⁺CD20⁺ T-cells in autoimmune diseases in the context of RTX therapy; therefore, their roles in the pathogenesis of PAN also need to be considered.     

Occult liver involvement by polyarteritis nodosa

The diagnosis of panarteritis nodosa is usually difficult because of the variability of clinical manifestations. Herein is presented a case of occult liver involvement by polyarteritis nodosa. Several biopsies were performed but the diagnosis was made with the contribution of a hepatic arteriography. Received: 5 March 2001 / Accepted: 15 October 2001     

Cutaneous polyarteritis nodosa associated with Crohn's disease

A 13-year-old white girl with Crohn's colitis developed recurrent erythematous tender cords and nodules in the lower and upper extremities. Histologic examination of subcutaneous nodules of the right arm revealed granulomatous panarteritis of two muscular arteries in the subcutis. The patient's resected colon showed granulomatous transmural colitis without vasculitis. The association between Crohn's disease and cutaneous polyarteritis nodosa is reviewed and emphasis placed on histologic evaluation of suspicious subcutaneous nodules for correct diagnosis.     

Systemic polyarteritis nodosa associated with acute Epstein-Barr virus infection

Polyarteritis nodosa (PAN) is a rare cause of systemic vasculitis in children, affecting medium and small-sized arteries. We report on a patient who presented with prolonged fever, shock, acute renal failure with nephrotic range proteinuria, hypertension, and sudden deterioration of consciousness. Cranial tomography revealed a left extensive hemorrhagic lesion. Renal biopsy revealed a large fibrinoid necrosis lesion consistent with PAN. Epstein-Barr virus (EBV) infection was confirmed by real-time polymerase chain reaction (RT-PCR) detection of EBV DNA. The patient was successfully treated with oral prednisolone and intravenous pulse of cyclophosphamide. To our knowledge, this is the first reported case of c-PAN related to active EBV infection.     

Acute cholecystitis at initial presentation of polyarteritis nodosa

Polyarteritis nodosa (PAN) is a systemic vasculitis of unknown etiology. Although gastrointestinal involvement may be seen in half of the cases of PAN, vasculitis of the gallbladder at the disease onset is a rare presentation. We report a case of a 48-year-old man who was admitted due to acute cholecystitis. He had complained of myalgia, fever and weight loss for about one month prior to admission. At physical examination, mild hypertension and calf pain were noted. He underwent a cholecystectomy; histopathological evaluation disclosed necrotizing vasculitis suggestive of PAN. We emphasize that cholecystitis may be part of the initial presentation of systemic vasculitis.     

Case reports: testicular pain as a manifestation of polyarteritis nodosa

Polyarteritis nodosa (PAN) is a necrotising vasculitis of medium-sized vessels of unknown origin. This type of vasculitis is usually systemic, but restriction to a single organ, for example the testis, the appendix or the gall bladder, can occur. Testicular pain or tenderness are frequent clinical features. In this report, we present three cases of PAN. In every patient, testicular pain was the main symptom or first sign of systemic disease. We state that a thorough history taking, clinical examination and biochemical analyses are obligatory in patients presenting with acute or chronic scrotal pain. Polyarteritis nodosa should always be taken into account, and a search for systemic spread is mandatory. We emphasize that before initiation of systemic therapy with corticosteroids and/or cyclophosphamide, a Five Factor Score should be obtained, which also gives crucial prognostic information.     

新分类标准对结节性多动脉炎诊断影响的研究初探

随着对抗中性粒细胞胞质抗体（ANCA）相关性血管炎的认识及乙型病毒性肝炎疫苗的广泛接种,结节性多动脉炎（PAN）的诊断发生了很大变化。本研究结合2012年Chapel Hill共识会议（CHCC）对PAN的定义和2007年欧洲药品管理局 （EMA）对中小血管炎分类法则,对2002年1月至2018年12月在北京协和医院住...     

Minocycline-induced vasculitis fulfilling the criteria of polyarteritis nodosa

Abstract

A 47-year-old man who had been taking minocycline for palmoplantar pustulosis developed fever, myalgias, polyneuropathy, and testicular pain, with elevated C-reactive protein (CRP). Neither myeloperoxidase- nor proteinase-3-antineutrophil cytoplasmic antibody was positive. These manifestations met the American College of Rheumatology 1990 criteria for the classification of polyarteritis nodosa. Stopping minocycline led to amelioration of symptoms and normalization of CRP level. To our knowledge, this is the second case of minocycline-induced vasculitis satisfying the criteria. Differential diagnosis for drug-induced disease is invaluable even for patients with classical polyarteritis nodosa.     

Minocycline-induced vasculitis fulfilling the criteria of polyarteritis nodosa

A 47-year-old man who had been taking minocycline for palmoplantar pustulosis developed fever, myalgias, polyneuropathy, and testicular pain, with elevated C-reactive protein (CRP). Neither myeloperoxidase- nor proteinase-3-antineutrophil cytoplasmic antibody was positive. These manifestations met the American College of Rheumatology 1990 criteria for the classification of polyarteritis nodosa. Stopping minocycline led to amelioration of symptoms and normalization of CRP level. To our knowledge, this is the second case of minocycline-induced vasculitis satisfying the criteria. Differential diagnosis for drug-induced disease is invaluable even for patients with classical polyarteritis nodosa.     

An autopsy case of hepatitis B (HB) antigen-positive polyarteritis nodosa, with reference to immunopathological studies of vascular lesions

An autopsy case of an 11-year-old boy with polyarteritis nodosa is described in which the onset of the disease was associated with the presence of hepatitis B (HB) antigens (Ag) in the cytoplasm and nuclei of hepatocytes as detected by immunohistological methods. Deposits of HBsAg, HBeAg, IgG, IgM, C3, and C1q were demonstrated in systemic vascular lesions. It is considered that the arteritis was due to deposition in the arteries of immune complexes formed by HBAg and HB antibodies. Received: February 10, 2000 / Accepted: July 13, 2000     

Cyclophosphamide treatment in polyarteritis nodosa

Summary Five male patients with polyarteritis nodosa were treated with cyclophosphamide as follows: 3 mg/Kg/die i.v. up to maximum of 3 g.; subsequently, 200 mg/die per os for two weeks, then 100 mg per os every other day for three months; finally, 100 mg every fourth day until the 18th month. One patient, who also had fever, received 25 mg/die of prednisone for the initial three weeks of treatment. Before treatment ESR, WBC, and circulating immune-complexes were increased, while C_3a, C_3c and C₄ serum complement components levels were normal. Skin ulcers healed within 4 months. A progressive marked improvement of visceral damages in the first months of therapy have been noted (e.g. blood pressure values in normal range after suspension of concomitant antihypertensive treatment, regression of peripheral neuropathy, etc. etc.). No further ischemic lesions occurred during treatment. Significant decreases of ESR and serum immune-complexes levels were detected. No untoward effects due to cyclophosphamide were observed. These findings support the effectiveness of this drug in polyarteritis. The possibility of association with glucocorticoids during the acute phase of disease is also discussed.     

Young male patient diagnosed with cutaneous polyarteritis nodosa successfully treated with etanercept

Abstract

Cutaneous polyarteritis nodosa (CPAN) is a form of necrotizing vasculitis of small and medium-sized arteries. It is limited to the skin and has a recurrent and chronic course, possibly associated with fever, arthralgia, myalgia and neuropathy, but without visceral involvement. We report the clinical case of a 7-year-old male patient with CPAN refractory to treatment with high doses of corticoids and cyclophosphamide, who was successfully treated with the TNF-α (tumor necrosis factor-alpha) inhibitor, etanercept, in monotherapy.     

Successful coil embolization of a ruptured hepatic aneurysm in a patient with polyarteritis nodosa accompanied by angioimmunoblastic T cell lymphoma

Polyarteritis nodosa (PN) occasionally develops in association with malignant disorders. A 71-year-old man suddenly suffered from bleeding due to the rupture of a hepatic artery aneurysm. The ruptured lesion was embolized endovascularly by coiling, and the bleeding was stopped. A biopsy of the right inguinal lymph node demonstrated angioimmunoblastic T cell lymphoma (AITL). He received immunosuppressive treatment with transient response, although he relapsed 4 months later. To our knowledge, this is the first case of which PN was associated with AITL.