The role of split-thickness skin grafting in the treatment of vasculitic and pyoderma gangrenosum ulcers in a multidisciplinary wound centre.

Vasculitic and pyoderma gangrenosum ulcers are traditionally treated with immunosuppressants, and the role of surgery in the treatment of these atypical ulcers remains unclear. This study aimed to investigate the need for surgical intervention as well as the outcome and safety of skin grafting in the treatment of 46 patients with vasculitic ulcers and 34 with pyoderma gangrenosum ulcers using data recorded in the validated Wound Registry. Of the 80 patients with atypical ulcers, 14% (n = 11) were treated surgically; these patients were older (p = 0.039), had lower mobility status (p = 0.002), and more often pulmonary diseases, rheumatoid arthritis, and previous arterial procedures (p = 0.007; p = 0.031; p = 0.031, respectively) than those treated conservatively. Of 181 ulcers, 15% (n = 27) were surgically treated, 78% once and 22% multiple times. During follow-up, 92.3% of both surgically and conservatively treated ulcers with available data healed. Of the surgically treated ulcers, median healing time after first surgical procedure was 96 days, and post-surgical complications were considered mild or unrelated to surgery. Our results suggest that if surgery is indicated, skin grafting is a safe and efficient treatment method provided that multidisciplinary approach is applied.

[Our Experience with ICON Hip Resurfacing System]. / Nase zkusenosti s povrchovou náhradou kycelního kloubu ICON.

PURPOSE OF THE STUDY Our study aimed to assess the mid-term outcomes and complications with the ICON hip resurfacing system and to carry out a detailed analysis of pitfalls and risks associated with pairing the bearing surfaces of metal-on-metal hip implants. MATERIAL AND METHODS A total of 52 hip joints were assessed in 43 patients who received the ICON hip resurfacing system at our department between 2009 and 2013. The cohort included 34 men and 9 women. Their age at the time of primary surgery ranged from 34 to 67 years, with the mean age of 51.8 years. The mean follow-up was 7.6 years. The evaluation focused on the position and osseointegration of both components, bone remodelling around the implant, and signs of potential aseptic loosening. The functional status of the hip was assessed by Harris Hip Score. RESULTS The primary stability of both components was always good, there were no femoral neck fractures reported in our cohort. All the acetabular components were stable, showing appropriate osseointegration, with no radiolucent zones or signs of osteolysis around them. There was not a single case of the femoral component stem being in a biomechanically disadvantageous varus position. In zone I and III according to Beaulé, cancellous bone osteolysis developed in two patients. The narrowing of the femoral neck below the end of the femoral component, compared to postoperative X-rays, achieved the mean value of 1.3% according to Grammatopolouse. The HHS increased from 64 to 95.5 points. An excellent outcome was observed in 48 joints, whereas the outcome of the remaining 4 joints was very good. The mean survival rate of the resurfacing hip implant calculated using the Kaplan-Meier analysis achieved 100%. The cobalt and chromium levels in the blood of patients did not exceed the reference physiological value. DISCUSSION The resurfacing system enables to preserve the bone tissue of the metaphysis and a part of the femoral head. The reduced mechanical endurance of the peripheral part of femoral components smaller in size caused by implant malposition resulted in fatal consequences in the ASR system. Greater range of motion conditioned by the design of the resurfacing system led to a mechanical wear, with a significant increase in the concentration of metal particles in the effective joint space. The elevated levels of cobalt and chromium ions in some patients induced delayed-type hypersensitivity with subsequent development of aseptic lymphocyte-dominated vasculitis associated lesions presented as peri-acetabular changes (pseudotumors to osteolysis) with subsequent failure of implant fixation. We have not observed this complication in the ICON system as yet. In patients suffering from hip pain after the resurfacing hip arthroplasty and simultaneous high chromium and cobalt blood levels, pelvic CT/MRI is indicated with reduction of artefacts around the metal material. Surgical treatment of soft tissue affections, bone defects and reimplantation using conventional or revisioncementless components is a possible treatment option. CONCLUSIONS The ideal patient indicated for hip resurfacing is a physically active man under 60 years of age (with a femoral head size of 54-60 mm), with primary or secondary osteoarthritis, no joint deformity, with a good quality bone tissue in the femoral neck and head region. As for the functional performance, the resurfacing system allows the patients a large range of motion with very good joint stability immediately after surgery. Despite that, the metal-on-metal tribological pairing must be approached with caution. The risk of developing lesions associated with ALVAL is unpredictable. In our cohort of patients with ICON hip resurfacing system, mostly excellent outcomes with minimum complications were reported provided the indication criteria and the correct surgical procedure had been complied with. Key words: hip resurfacing system, metal articulating surfaces, adverse reaction to metal particles, aseptic lymphocytedominated vasculitis associated lesions, pseudotumor.

Cutaneous foreign body microemboli-induced occlusive vasculopathy: A complication of illicit intravenous injection of oral opioid tablets.

Occlusive nonvasculitic vasculopathy is a process characterized clinically by retiform purpura and potential ulceration and necrosis of affected areas, secondary to blockage of small vessels without associated inflammatory vasculitis. Intravascular injection of foreign material is known to cause distal ischemia and necrosis due to thrombosis, local vasoconstriction, or microemboli formation. A 27-year-old male presented with retiform purpura and worsening distal fingertip necrosis of the right upper extremity accompanied by suspicious intravascular polarizable foreign material identified on skin, muscle, and vascular biopsies. We report a case that highlights concerning complications and dermatopathologic findings of intravascular injection of oral opioid tablets.

A rare case of laparoscopy towards SLE with lupus mesenteric vasculitis induced ascites.

BACKGROUND: Diagnosis and management of acute abdomen secondary to systematic lupus erythematosus (SLE) has always been a clinical challenge. CASE PRESENTATION: A 21-year-old lady, with BMI 17.7, presented to our department with acute abdomen. Laparoscopy was carried out to exclude surgical emergency when conservative regimen failed. The patient revealed a history of purpuric changes and lupus test was positive for SLE. CONCLUSION: Based on our experience, early laparoscopy to alleviate acute abdomen has shown to improve the prognosis of the patient.

Five Hundred Fifty-five Retrieved Metal-on-metal Hip Replacements of a Single Design Show a Wide Range of Wear, Surface Features, and Histopathologic Reactions.

BACKGROUND: In 2010, a widely used metal-on-metal hip implant design was voluntarily recalled by the manufacturer because of higher than anticipated failure rates at 5 years. Although there was a large published range of revision rates, numerous studies had reported a higher risk of revision for excessive wear and associated adverse tissue reactions when compared with other metal-on-metal total hips. The reasons for this were suggested by some to be related to cup design features. QUESTIONS/PURPOSES: From retrievals of ASR metal-on-metal implants and tissue samples obtained at revision surgery, we asked the following questions: (1) What were the common and uncommon surface features? (2) What were the common and uncommon linear and volumetric wear characteristics? (3) Were there common taper corrosion characteristics? (4) What aseptic lymphocytic vasculitis-associated lesion (ALVAL) features were present in the tissues? METHODS: Five hundred fifty-five ASRs, including 23 resurfacings, were studied at one academic research center. Features of wear (eg, light and moderate scratching), damage (eg, deposits, gouges), and bone attachment on the porous coating were semiquantitatively ranked from 0 (none) to 3 (> 75%) based on the amount of a feature in each region of interest by the same experienced observer throughout the study. Visible features of head taper corrosion were ranked (Goldberg score) from 1 (none) to 4 (severe) by the same observer using a previously published scoring method. An experienced tribologist measured component wear depth using a coordinate measuring machine and quantified wear volume using previously validated methods. All available tissues were sampled and examined for features of ALVAL and scored from 0 to 10 by a single observer using a method they previously developed and published. A score from 0 to 4 is considered low, 5 to 8 is considered moderate, and 9 or 10 is considered high with regard to the risk of metal hypersensitivity features in the tissues. RESULTS: The most common bearing surface features were light and moderate scratches and removal or postremoval damage. Discoloration and deposits were commonly observed on femoral heads (55% [305 of 553]) and less commonly on cups (30% [165 of 546]). There was no evidence of impingement or dislocation damage. There was typically a small amount of bone attachment in at least one of eight designated regions of interest (84% [460 of 546]); extensive or no bone attachment was uncommon. Edge wear was highly prevalent. The maximum wear of 469 cups (88%) occurred near the edge, whereas the maximum wear of 508 femoral heads (94%) occurred between the pole and 45° from the pole. The median combined head-cup wear volume was 14 mm (range, 1-636 mm). One hundred sixty-nine pairs (32%) had a combined wear volume of < 10 mm, 42 pairs (8%) had volumetric wear of > 100 mm, and 319 pairs (60%) had wear volume between 10 and 100 mm³. Seventy-four percent of tapers (390 of 530) received a Goldberg score of 4, 22% (116 of 530) a score of 3, < 5% (24 of 530) a score of 2, and none received a score of 1. The most frequent ALVAL score was 5 out of 10 (35 of 144 hips [24%]) and ranged from 2 (one hip) to 10 (nine hips); 92 of 144 (64%) had a moderate score, 17 of 144 (12%) had a high score, and 35 (24%) had a low score. CONCLUSIONS: Although edge wear was prevalent, in most cases, this was not associated with high wear. The increased diameter and decreased coverage angle of the ASR design may have resulted in the observed high incidence of edge wear while perhaps decreasing the risk for impingement and dislocation. CLINICAL RELEVANCE: The role of bearing wear in the revisions of metal-on-metal implants is controversial, because it is known that there is a large range of in vivo wear rates even within the same implant type and that patient variability affects local tissue responses to wear debris. The observations from our study of 555 retrieved ASR implant sets indicate that there was a wide range of wear including a subset with very high wear. The results suggested that the failure of the ASR and ASR XL was multifactorial, and the failure of different subgroups such as those with low wear may be the result of mechanisms other than reaction to wear debris.

Oscillatory flow suppression improves inflammation in chronic venous disease.

BACKGROUND: To assess if suppression of the oscillatory component of reflux may improve the inflammatory phenotype in chronic venous disease (CVD). MATERIALS AND METHODS: From 193 CVD patients, we selected 54 (13 males, 41 females, CEAP C2-4EpAsPr) for a blinded, case-control prospective investigation. All of them underwent echo-color-Doppler assessment of reflux parameters. In the same patients a blood systemic assessment of 19 inflammatory cytokines was obtained. Follow-up lasted 6 months. The control group (C) was constituted by 21 homogenous CVD patients, unselected and not operated. RESULTS: Thirty-one of 54 patients were excluded from post-operative evaluation in consequence of reported new other inflammatory episodes. Twenty-three (23) completed the follow up, showing the suppression of the oscillatory component of venous reflux; 4 of the 19 cytokines decreased significantly after the procedure: Tumor Necrosis Factor-α (TNFα), Granulocyte Colony Stimulating Factor (G-CSF), Interferon gamma-induced Protein 10 (IP-10), Interleukin-15 (IL-15). Particularly, TNFα and IP-10 even returned inside a physiological range: 5.3 ± 2.7 to 4.2 ± 2.2 pg/mL (P < 0.003) and from 303.7 ± 168.4 to 254.0 ± 151.6 pg/mL (P < 0.024), respectively. Both cytokines showed a weak but significant correlation with parameters of oscillatory flow correction. Finally, three cytokines implicated in repair and remodeling of tissue, Epidermal Growth Factor, Monocyte Chemoattractant Protein-1 and Platelet Derived Growth Factor-BB (PDGF-BB), significantly increased. Our findings are further reinforced by the significant changes of the same cytokines when compared to C group. CONCLUSIONS: The surgical suppression of the oscillatory component of reflux modulates the inflammatory phenotype, suggesting a pivotal role of flow among factors concurring to inflammation in CVD.

MRI predicts ALVAL and tissue damage in metal-on-metal hip arthroplasty.

BACKGROUND: Adverse local tissue reactions (ALTR) around metal-on-metal (MOM) hip arthroplasties are increasingly being recognized as a cause of failure. These reactions may be associated with intraoperative tissue damage and complication rates as high as 50% after revision. Although MRI can identify ALTR in MOM hips, it is unclear whether the MRI findings predict those at revision surgery. QUESTIONS/PURPOSES: We therefore (1) identified which MRI characteristics correlated with histologically confirmed ALTR (using the aseptic lymphocytic vasculitis-associated lesions [ALVAL] score) and intraoperative tissue damage and (2) developed a predictive model using modified MRI to detect ALVAL and quantify intraoperative tissue damage. METHODS: We retrospectively reviewed 68 patients with failed MOM hip arthroplasties who underwent preoperative MRI and subsequent revision surgery. Images were analyzed to determine synovial volume, osteolysis, and synovial thickness. The ALVAL score was used to grade tissue samples, thus identifying a subset of patients with ALTR. Intraoperative tissue damage was graded using a four-point scale. Random forest analysis determined the sensitivity and specificity of MRI characteristics in detecting ALVAL (score ≥ 5) and intraoperative tissue damage. RESULTS: Maximal synovial thicknesses and synovial volumes as determined on MRI correlated with the ALVAL score and were higher in cases of severe intraoperative tissue damage. Our MRI predictive model showed sensitivity and specificity of 94% and 87%, respectively, for detecting ALVAL and 90% and 86%, respectively, for quantifying intraoperative tissue damage. CONCLUSIONS: MRI is sensitive and specific in detecting ALVAL and tissue damage in patients with MOM hip implants. MRI can be used as a screening tool to guide surgeons toward timely revision surgery.

What causes unexplained pain in patients with metal-on metal hip devices? A retrieval, histologic, and imaging analysis.

BACKGROUND: Adverse tissue reactions associated with metal-on-metal (MOM) hips are common in resurfacing and total hip arthroplasty (THA) designs. The etiology of these reactions in painful, well-positioned arthroplasties is inconsistently described. QUESTIONS/PURPOSES: The purposes of this study were to compare the (1) articular wear rates; (2) histologic findings; (3) synovial response on MRI; and (4) graded intraoperative tissue damage between well-positioned, MOM hips revised for unexplained pain and MOM hips revised for other reasons and to (5) determine whether the presence of a taper junction on a MOM articulation affects these four parameters in unexplained pain. METHODS: We retrospectively studied 88 patients (94 hips) who had undergone revision of either a hip resurfacing or a large-head (> 36 mm) THA. Thirty-five hips revised for unexplained pain were compared with a control group of 59 hips revised for other causes. Articular wear was measured using three-dimensional contactless metrology and histologic analysis was performed using the aseptic lymphocyte-dominated vasculitis-associated lesion (ALVAL) score. Preoperative MRI was performed on 57 patients to determine synovial volumes and thicknesses. Tissue damage was graded from intraoperative reports. RESULTS: Articular wear rates in the unexplained pain group were lower than in the control group (median 2.6 µm/year versus 12.8 µm/year, p < 0.001). Sixty-six percent of patients in the unexplained pain group had histologic confirmation of ALVAL compared with 19% in the control group (p < 0.001). The synovial thickness on MRI was higher in the unexplained pain group (p = 0.04) and was highly predictive of ALVAL. Severe intraoperative tissue damage was noted in more cases in the unexplained pain group (p = 0.01). There were no differences in articular wear, histology, MRI, and tissue damage between resurfacings and THAs revised for unexplained pain. CONCLUSIONS: Unexplained pain in patients with well-positioned MOM hips warrants further investigation with MRI to look for features predictive of ALVAL. Tissue destruction in these cases does not appear to be related to high bearing wear or the presence of a taper.

Silicone oil tamponade induced vasculitis- a rare manifestation of toxic posterior segment syndrome.

AIM: To report silicone oil tamponade induced vasculitis in the early post operative period - a rare manifestation of 'Toxic Posterior Segment Syndrome' after pars plana vitrectomy for rhegmatogenous retinal detachment. CASE DESCRIPTION: A 50-year-old gentleman presented with vasculitis after a pars plana vitrectomy with silicone oil tamponade on the first post-operative day. He was started on oral steroids (1 mg/ kg) tapered sequentially every week. All signs of vasculitis resolved over a period of one month after which the silicone oil was removed. The patient maintained a visual acuity of 20/90 even after 3 months. There was no recurrence of vasculitis observed thereafter. CONCLUSION: Silicone oil induced vasculitis is a rarely described entity, especially as a manifestation of toxic posterior segment syndrome. This differential must be kept in mind even on the first post-operative day in cases where silicone oil has been used for tamponade.

Rare Case of Abdominal Aortic and Multiple Visceral Aneurysms in a Pediatric Patient With PIK3CA Mutation and Vasculitis.

Abdominal aortic aneurysms (AAA) are most commonly observed in elderly male patients and are particularly rare in children. Among the pediatric population, they are usually diagnosed in the context of connective tissue disorders, genetic mutations, or vasculitis. The same is true of visceral arteries aneurysms. This case report describes the staged management of an 11-year-old patient presenting PIK3CA mutation and a 5.8 cm infrarenal AAA associated with bilateral common iliac arteries and multiple visceral aneurysms, the largest observed in the superior mesenteric artery (SMA = 3.2 cm). After careful evaluation, decision was made to first approach the most life-threatening lesion (the infrarenal AAA due to the large diameter) and the remaining aneurysms in secondary procedures, with special attention to the SMA aneurysm. The patient underwent a staged repair, with the first phase consisting of an aortobi-iliac graft with the distal anastomosis made at the left common iliac artery and right external iliac artery. The right hypogastric artery was ligated. The second procedure consisted of SMA aneurysm repair with a plication technique, as 7 branches were visualized coming off the aneurysm sac. Postoperative pathology analysis of the aortic and SMA aneurysms sac revealed vasculitis with a mixed inflammatory pattern and a COL3A1 gene heterozygote variant. He is currently in his 18th month after the last surgical intervention, receiving immunomodulatory therapy, with a planned follow-up by the interdisciplinary team to monitor the medications' side effects and the diameter of the remaining visceral aneurysms.

Presumed reactive polyarthritis and granulomatous vasculitis in a Mississippi sandhill crane (Grus canadensis pulla).

A 1.5-year-old female Mississippi sandhill crane (Grus canadensis pulla) was presented and managed for a polyarthritis of the intertarsal and tarsophalangeal articulations. Results of aerobic bacterial cultures, Mycoplasma species culture, and polymerase chain reaction testing of articular fluid did not identify any causative organisms. Results of radiographs and cytologic examination of articular fluid were consistent with an inflammatory, nonerosive polyarthritis. The arthritis did not improve with systemic anti-inflammatory and antibiotic treatment and with joint lavage. A large necrotic granulomatous mass was detected on the right shoulder area from which Staphylococcus aureus and Enterococcus species were isolated as opportunistic pathogens. Two days after surgical resection of the mass, the distal polyarthritis resolved. Histopathologic examination of the mass was consistent with granulomatous vasculitis with abscess formation of unknown origin. In this crane, the unresponsiveness to standard therapy, the presence of an infected and inflammatory mass, and the resolution of the polyarthritis after the resection of the mass strongly supported a diagnosis of reactive immune-mediated nonerosive polyarthritis. Analysis of this case suggests that immune-mediated idiopathic arthritis should be a differential diagnosis of distal polyarthritis in cranes and that an inciting source remote from the joints should be investigated in case of lack of response to standard therapy.

Clinical study of profile of retinal detachment in vasculitis and its management at tertiary eye care hospital.

Purpose: The aim of the study was to understand the clinical profile and anatomical and visual outcome in Tractional/Combined (Tractional + Rhegmatogenous) Retinal Detachment due to vasculitis after surgical intervention. Methods: It was an interventional retrospective study of all cases who underwent surgery for RD with vasculitis at a single tertiary eye care center for over a period of 6 years. Patients with retinal detachment due to vasculitis were included in the study. All patients underwent the following surgical procedures: 240 belt buckle with three-port pars plana vitrectomy with membrane dissection and peeling with fluid gas exchange/with endolaser with silicon oil/C3 F8 gas injection. Results: In our study, 83.33% had preoperative vision of less than 6/60, whereas postoperatively 66.66% had vision of less than 6/60. Postoperatively 33.33% patients had vision better than 6/36. Of the six eyes operated for vasculitis with RD, retina was attached in five eyes following surgery. One patient had recurrent retinal detachment due to extensive proliferative vitreoretinopathy changes, and he was advised re-procedure but was lost for follow-up. The anatomical success was 83.33% on the first surgery. Conclusion: The overall anatomic success rate of retina reattachment surgery in vasculitis patient was good, and the visual outcome following the surgery can improve in majority of the cases. Hence, timely intervention is advocated.

[The importance of early surgical intervention in the treatment of necrotizing vasculitis]. / Význam vcasné chirurgické intervence v terapii nekrotizující vaskulitidy.

Vasculitides are an etiologically heterogeneous group of vascular affections leading to vascular wall damage followed by ischaemia of the tissues supplied by the affected vessels. Damage to the vessels of lower extremities is frequent; in serious cases a complete destruction of soft tissue as well as skeletal lesions may occur. This then results in a situation which, without a well-timed surgical intervention, often requires an amputation of the limb. We are presenting a case of a 61-year-old female patient with a large plantar defect of the right foot and gangrene of three toes of the same limb which developed as a result of necrotizing vasculitis.

Multimodal Approach of Isolated Pulmonary Vasculitis: A Single-Institution Experience.

BACKGROUND: Isolated pulmonary vasculitis (IPV) is a single-organ vasculitis of unknown etiology and may mimic chronic thromboembolic pulmonary hypertension (CTEPH). The aim of this study was to review our clinical experience with pulmonary endarterectomy in patients with CTEPH secondary to IPV. METHODS: Data were collected prospectively for consecutive patients who underwent pulmonary endarterectomy and had a diagnosis of IPV at or after surgery. RESULTS: We identified 9 patients (6 women; median age, 48 years [range, 23-55]) with IPV. The diagnosis was confirmed after histopathologic examination of all surgical materials. The mean duration of disease before surgery was 88.0 ± 70.2 months. Exercise-induced dyspnea was the presenting symptom in all patients. Pulmonary endarterectomy was bilateral in 6 patients and unilateral in 3. No deaths occurred; however 1 patient had pulmonary artery stenosis, and stent implantation was performed. All patients received immunosuppressive therapies after surgery. Mean pulmonary artery pressure decreased significantly from 30 mm Hg (range, 19-67) to 21 mm Hg (range, 15-49) after surgery (P < .05). Pulmonary vascular resistance also improved significantly from 270 dyn/s/cm-5 (range, 160-1600) to 153 dyn/s/cm-5 (range, 94-548; P < .05). After a median follow-up of 41 months, all but 1 patient had improved to the New York Heart Association functional class I. CONCLUSIONS: IPV can mimic CTEPH, and these patients can be diagnosed with pulmonary endarterectomy. Furthermore surgery has not only diagnostic but also therapeutic value for IPV when stenotic and/or thrombotic lesions are surgically accessible. A multidisciplinary experienced CTEPH team is critical for management of these unique patients.

Primary testicular necrotizing vasculitis clinically presented as neoplasm of the testicle: a case report.

We present a case of necrotizing vasculitis with the testicle as the isolated affected organ. A 25-year-old man, pretreated for epididymo-orchitis, presented with a presumed testicular neoplasm. Radical orchiectomy was performed and diagnosis of necrotizing vasculitis was established. In the absence of any other sign of systemic disease, the diagnosis of isolated necrotizing vasculitis of the testis was confirmed. Two years after the operation, the patient showed no symptoms of systemic disease.

[Eosinophilic angiocentric fibrosis and granuloma facial with extra facial presentation, the same pathology?]. / Fibrose angiocentrique à éosinophiles et granulome facial à forme extrafaciale, une même entité ?

Eosinophilic angiocentric fibrosis is a rare fibro inflammatory lesion of unknown etiology which occurs usually in the upper respiratory tract mucosa of middle-aged adults. The histologic features show an eosinophilic vasculitis and an angiocentric fibrosis with onion-skin pattern. Firstly described as a mucosal variant of the granuloma facial, which is a rare cutaneous vasculitis with eosinophils, it is considerated by some authors as separated entities. Four cases have been described in the orbit and three of them were in fact an extension of a sinusal lesion. We report the first case affecting a 69-years-old male patient who showed an isolated orbital involvement in association with granuloma facial, extra facial. This observation illustrates the relationship between these two pathologies and consolidates the first hypothesis of a single disease with cutaneous or mucosal involvement.

Clinical presentations and long term prognosis of childhood onset polyarteritis nodosa in single centre of Korea.

Childhood-onset polyarteritis nodosa (PAN) is a rare and systemic necrotising vasculitis in children affecting small- to medium-sized arteries. To date, there have been only a few reports because of its rarity. Thus, we aimed to investigate the clinical manifestations, laboratory findings, treatment, and long-term outcomes in patients with childhood-onset PAN and to evaluate the usefulness of the paediatric vasculitis activity score (PVAS). We retrospectively analysed the data of nine patients with childhood-onset PAN from March 2003 to February 2020. The median ages at symptom onset, diagnosis, and follow-up duration were 7.6 (3-17.5), 7.7 (3.5-17.6), and 7.0 (1.6-16.3) years, respectively. All patients had constitutional symptoms and skin manifestations, while five exhibited Raynaud's phenomenon. Organ involvement was observed in one patient. The median PVAS at diagnosis was 7 (range: 2-32). Prednisolone was initially used for induction in all patients, and other drugs were added in cases refractory to prednisolone. All patients survived, but three patients with high PVAS at diagnosis experienced irreversible sequelae, including intracranial haemorrhage and digital amputation. In conclusion, early diagnosis and treatment may minimise sequelae in patients with childhood-onset PAN. This study suggests that high PVAS score at diagnosis may be associated with poor prognosis.