The significance of antineutrophil cytoplasmic antibody in microscopic polyangitis and classic polyarteritis nodosa.

AIMS: To describe the distribution and features of classic polyarteritis nodosa (PAN) and microscopic polyarteritis (MPA) and the importance of antineutrophil cytoplasmic antibody (ANCA) in childhood PAN. METHODS: Classic PAN was diagnosed in 15 patients based on the presence of aneurysms on angiography in 10 patients and of necrotising vasculitis in medium sized arteries in five. MPA was diagnosed in 10 patients, based on characteristic findings at renal biopsy in six and by the presence of small sized necrotising arteritis in four. Serum ANCA was detected initially by indirect immunofluorescence (IIF) followed by an immunoassay for myeloperoxidase (MPO) in each case. RESULTS: The median age of the patients with classic PAN and MPA was 12 (range 8-17) and 9.5 (range 5-14) respectively. None of the patients with classic PAN had renal failure. Six of the patients with MPA presented with renal failure; four progressed to chronic renal failure. Clinically evident pulmonary-renal syndrome was present in three of the 10 patients with MPA. IIF for ANCA in classic PAN was negative in nine, showed mild staining patterns in six, and in one MPO-ELISA was mildly increased. IIF for ANCA in MPA revealed very strong perinuclear ANCA staining in nine and atypical staining in one. In MPA, median MPO-ELISA level was 42.5 EU/ml (range 20-250). Treatment of childhood PAN was satisfactory with effective treatment; however relapses did occur. CONCLUSION: ANCA is useful in the diagnosis and follow up of MPA.     

Anti-neutrophil cytoplasmic antibody for proteinase 3 in a child with polyarteritis nodosa

We report a case of a 15 year old boy with polyarteritis nodosa associated with antineutrophil cytoplasmic antibody (ANCA) against proteinase 3 (PR3). After months of steroid and immunosuppressant therapy, the symptoms subsided and the polyaneurysms almost disappeared. The levels of anti-PR3 antibody and of cytokines also decreased. The results indicate that ANCA is a good indicator of this disease activity, and may play some pathogenic role in the disease.     

儿童抗中性粒细胞胞浆抗体相关性小血管炎的临床病理特点分析

目的探讨儿童抗中性粒细胞胞浆抗体(ANCA)相关性小血管炎的临床表现、血清ANCA和肾脏病理检查特征以及其治疗转归情况.方法采用间接免疫荧光定性检查血清ANCA,以酶联免疫吸附分析(ELISA)定量测定患儿血清抗蛋白酶3(PR3)和抗髓过氧化物酶(MPO)抗体活性,并同时进行肾穿刺病理检查,对5例ANCA相关性小血管炎(ASV)患儿的临床表现、血清ANCA与肾活检结果及治疗转归情况进行分析.结果 (1) 5例ASV患儿占同期肾脏疾病住院患儿的0.25%,发病年龄在8～12岁之间,平均10岁6个月,均为女孩.(2) 5例ASV患儿血清胞浆型ANCA阴性,抗PR3定量都在正常范围内,环核型ANCA均阳性,抗MPO 98～242 kEU/L,平均154.5 kEU/L(正常<12.7 kEU/L).(3) 5例患儿均为ANCA相关性小血管炎中的显微镜下多动脉炎,肾脏病理改变均为广泛新月体形成,并有毛细血管袢纤维素样坏死,免疫荧光检查有不同程度的C3沉积,除1例IgA和IgM沉积较强外,免疫球蛋白沉积均较弱.电镜下显示沉积部位主要在内皮下,1例在GBM内也有沉积.(4) 5例患儿中有3例在确诊后1年内死亡,2例经甲泼尼龙和环磷酰胺冲击后,肾功能恢复至正常,分别随访1年和5年,留有血尿和少量蛋白尿.结论儿童ASV女孩占绝大多数,以环核型ANCA为主,较成人更易出现肾功能衰竭,预后也更为严峻,血清ANCA定性定量检查与肾活检是诊断儿童ASV的关键.     

Inferior suprarenal artery aneurysm in polyarteritis nodosa

A 16-year-old boy with the complaints of fever, abdominal pain, arthralgia and hypertension was found to have polyarteritis nodosa. Selective renal angiography disclosed a 5-mm microaneurysm of the right inferior suprarenal artery, which has not been previously reported in polyarteritis nodosa. Aside from the diagnostic viewpoint, some of the perirenal and retroperitoneal hemorrhages of unknown origin seen in polyarteritis nodosa might be due to the rupture of suprarenal artery aneurysms. Received: 6 December 1995 Accepted: 8 February 1996     

伴抗中性粒细胞胞浆抗体阳性狼疮性肾炎患儿临床病理特征分析

目的 分析伴抗中性粒细胞胞浆抗体（ANCA）阳性狼疮性肾炎（LN）患儿的临床及病理特征。方法 回顾性选择2003年1月至2019年12月于中山大学附属第一医院诊断的LN患儿,依据血清ANCA结果分为ANCA阳性组（n=59）和ANCA阴性组（n=454）,比较两组患儿临床表现、组织病理学、治疗缓解与预后情况。结果 ANCA阳性组白细胞降低并伴红细胞沉降率增快（P < 0.05）。两组患儿血肌酐、尿蛋白、尿红细胞差异无统计学意义（P > 0.05）。308例患儿行肾脏穿刺活检,光镜结果提示ANCA阳性组细胞纤维性新月体比例高于ANCA阴性组（P < 0.05）,免疫复合物沉积比例低于ANCA阴性组（P < 0.05）。两组患儿治疗缓解率、生存情况差异无统计学意义（P > 0.05）。结论 ANCA阳性LN患儿肾脏病理损害更重,与临床表现不完全对应平行,及时行肾脏穿刺活检至关重要。     

Cutaneous polyarteritis nodosa in a young child.

A 5 1/2-year-old girl presented with an acute febrile illness associated with limb and facial swelling, and a skin eruption. She was diagnosed as suffering from cutaneous polyarteritis nodosa. She was told to stay in bed and was given soluble aspirin. Now, more than 3 years later, she is well and symptom-free. The important diagnostic feature of this benign condition, which is distinct from the systemic disease, is the presence of skin nodules showing the histology of a necrotising arteritis at the junction of dermis and subcutaneous tissue.     

Isolated polyarteritis nodosa of the gallbladder in older children

A 10-year-old-boy with acute acalculous cholecystitis due to polyarteritis nodosa of the gallbladder with limited involvement of this organ is reported. It is suggested that a search for other lesions is indicated when polyarteritis nodosa of the gallbladder has been diagnosed, but that no treatment is indicated when additional lesions are not found.     

Abdominal manifestations of polyarteritis nodosa demonstrated with CT

We report a rare case of polyarteritis nodosa (PAN) presenting in childhood. The child had multiple visceral aneurysms and later developed ascending colitis and jejunitis. The diagnosis was established with multidetector CT and CT angiography.     

Fatal polyarteritis nodosa with gastrointestinal involvement in a child

Polyarteritis nodosa (PAN) is a necrotizing angiitis that predominantly affects small- and medium-sized arteries. Polyarteritis nodosa occurs rarely during childhood. Boys and girls seem to be equally affected, with a peak at the age of 10 years. Rarely, severe and fatal gastrointestinal involvement is seen in PAN. Here, we report a 15-year-old boy with PAN, who had gastrointestinal involvement with multiple aneurysms of the hepatic and superior mesenteric arteries. This involvement could be demonstrated with conventional angiography and gastrointestinal bleeding scintigraphy. The progression of the symptoms and the decrease in the size of the aneurysms were noted after combination treatment with cyclophosphamide and prednisolone, but there was severe bleeding from small bowel, and it was taken under control by resection of jejunum. However, the patient died because of sepsis. In conclusion, severe gastrointestinal involvement in PAN is usually fatal despite aggressive therapy, as is the case in our patient.     

Cutaneous polyarteritis nodosa in a patient with ulcerative colitis

Skin disease is common in patients with inflammatory bowel disease. Described herein is a child with ulcerative colitis and cutaneous polyarteritis nodosa. Review of the literature suggests cutaneous polyarteritis must be considered as another skin lesion associated with inflammatory bowel disease. Cutaneous polyarteritis tends to run a chronic relapsing course independent of bowel disease, however.     

Paediatric enteritis as a presentation of infantile polyarteritis nodosa

Multisystem arteritis is a rare condition in paediatric practice. We describe a 4-month-old boy presenting with an acute abdomen. At laparotomy the small bowel had the appearance of necrotising enterocolitis. The child died shortly after surgery from unsuspected cardiac involvement. The clinical features, diagnosis, and management are reviewed. Correspondence to: D. Talbot     

Cutaneous polyarteritis nodosa in a child and a review of the literature

The cutaneous form of polyarteritis nodosa in children is extremely rare. Findings are usually limited to the skin, muscles and joints. It has a benign but often chronic course. We describe an 8-y-old girl with cutaneous PAN, with extensive livedo reticularis on lower and upper extremities, tender subcutaneous nodules, arthralgia and right ankle swelling. Skin biopsy revealed vasculitis of small and medium-sized blood vessels characterized by fibrinoid necrosis. The use of prednisolone resulted in clinical improvement initially, but recurrence occurred during tapering. She showed marked improvement with additional high dose methyl prednisolone monthly.     

Clinical observation of 14 cases of childhood polyarteritis nodosa in Japan

An epidemiological survey of childhood polyarteritis nodosa was conducted at 1290 hospitals followed by a secondary survey of hospitals that had therapeutic experience with the disease. Fourteen cases obtained from these surveys were studied clinically. The male-female ratio was 4:3. The mean age at onset was 10.6 years. To established the diagnosis, skin biopsy was performed in 43%, retrograde aortography in 21%, and kidney biopsy in 14% of cases. Non-specific symptoms such as fever (86%), skin eruption (50%), arthritis (50%), myalgia (50%), abdominal pain (43%) and hypertension (36%) were often noted as clinical findings at the initial visit. Throughout the clinical course, symptoms considered to be caused by local angiopathy, including interrupted blood circulation, were frequently noted. On blood tests, no specific findings other than acute inflammatory reaction were observed. Steroid preparations were used for treatment in all cases but one, and about half of them are presently free from steroids. Immunosuppressants were administered in 10 cases. As for prognosis, all patients are alive, but untoward sequelae were noted in 36% of cases. The results of the present survey reinforced the difficulty of establishing a final differential diagnosis because specific findings were scarce for this disease, as stated in previous reports. Establishing a method for early diagnosis by gathering and analyzing more data in detail will be necessary in the future.