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The global outbreak of coronavirus disease (COVID-19) caused by severe acute respiratory syndrome coronavirus type 2 has prompted the rapid spread and development of vaccines to prevent the spread of the disease. COVID-19 vaccine has demonstrated excellent efficacy in reducing morbidity and severity of the disease, and most adverse reactions are very minor. However, some patients have been reported to develop autoimmune diseases, such as rheumatoid arthritis, myocarditis, Guillain-Barre syndrome, and vasculitis, following COVID-19 vaccination. Herein, we present a case of polyarteritis nodosa with epididymitis following COVID-19 mRNA vaccination. The patient's initial symptoms were fever and testicular pain, and magnetic resonance imaging showed epididymitis. He was diagnosed as having polyarteritis nodosa with epididymitis and treated with high-dose prednisolone, with a good clinical outcome.
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Objectives: Patients with immune-mediated rheumatic diseases (IMRDs) develop more severe outcomes of Coronavirus disease 2019 (COVID-19). Recent studies have contributed to understand the safety and efficacy of COVID-19 vaccines in IMRDs, suggesting that different diseases and therapies may interfere on immunization efficacy. In this study we analyze the immunogenicity of COVID-19 vaccines in patients with Systemic Vasculitides (VASC), the rate of COVID-19 and the frequency of disease relapse following immunization. Method(s): We included patients with VASC (n = 73), a subgroup of the SAFER study (Safety and Efficacy on COVID-19 Vaccine in Rheumatic Disease), a longitudinal, multicenter, Brazilian cohort.We analyzed the geometric means of IgG antibody against receptor-biding domain of protein spike of SARS-CoV-2 (anti-RBD) after two shots of CoronaVac (Inactivated vaccine), ChadOx-1 (AstraZeneca) or BNT162b2 (Pfizer-BioNTech). IgG anti-RBD was measured by chemiluminescence test. We assessed new-onset COVID-19 episodes, adverse events (AE) and disease activity for each VASC. Result(s): The sample included Behcet's disease (BD) (n = 41), Takayasu arteritis (TAK) (n = 15), antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) (n = 14), polyarteritis nodosa (n = 7) and other small vessel VASC(n = 6). The majority of patients were female (69%) without comorbidities (49%) and a median age of 37 years. The most common medication was conventional synthetic disease-modifying anti-rheumatic drugs, followed by biologic drugs. No patient received rituximab at baseline. Most patients received CoronaVac (n = 25) or ChadOx-1 (n = 36), while four received BNT162b2. Baseline IgG-RBD means were 1.34 BAU/mL. They increased to 3.89 and 5.29 BAU/mL after the 1st and 2nd vaccine dose, respectively. ChadOx-1 had higher antibody titers than CoronaVac (p = 0.002). There were no differences between different VASC. There were 3 cases of COVID-19 after immunization with CoronaVac. BD patients had a tendency for more cutaneous-articular activity following ChadOx-1. There were no severe relapses and no serious adverse events. Conclusion(s): Our results show the safety of different SARS-CoV-2 vaccines in VASC population. A progressive increase of IgG-RBD antibodies was observed after each dose. ChadOx-1 led to higher IgG-RBDgeometricmeans compared toCoronaVac. Finally, even though ChadOx-1 presented a tendency of triggering mild disease activity, there were no significant disease activity following vaccination in VASC patients. .
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Background/Aims A 72-year-old lady presented in primary care with complaints of generalised body aches, bilateral leg weakness and constitutional symptoms following a first dose of COVID-19 vaccine. Blood tests showed slightly raised inflammatory markers. She was initially diagnosed with polymyalgia rheumatica and was started on 40mg prednisolone with minimal improvement. Methods The examination in the rheumatology clinic was unremarkable. Investigations revealed raised white cell count, consistent with high dose steroid treatment, and elevated monocytes. There was mild improvement in inflammatory markers. The working diagnosis was of self-limiting viral illness. Further testing discovered strongly positive MPO ANCA (115 IU/ml), and the patient received three pulses of 500mg methylprednisolone for suspected vasculitis arranged by the medical team. There was no evidence of renal involvement. The diagnosis made at this point was autoimmune inflammatory disorder with unclear aetiology. At the subsequent clinic visit she reported mild shortness of breath, but no other symptoms suggestive of either vasculitis or connective tissue disease. Repeat ANCA showed significant reduction in MPO titre following pulse steroid treatment. CT of chest, abdomen and pelvis demonstrated a localised lobular/ nodular deformity of the liver. Viral hepatitis screen was negative. CA19-9 was raised at 100 U/ml. Liver biopsy was reported as poorly differentiated carcinoma without specific localising immunohistochemical features. Results The patient underwent hemi-hepatectomy for histologically confirmed pT2pNXM0R0 liver cholangiocarcinoma in a tertiary centre followed by adjuvant chemotherapy with capecitabine. With treatment, her MPO ANCA and CA19-9 levels declined. An interval CT scan of chest, abdomen and pelvis performed ten months after the surgery, showed no recurrence of malignancy. Given the fact that the patient's MPO ANCA fell following the treatment of cholangiocarcinoma, it is likely that positive MPO ANCA is associated with underlying malignancy rather than an active vasculitis. Conclusion This unusual case describes an evolution of the diagnostic process guided by non-specific symptoms and ANCA positivity, arriving at an unexpected diagnosis of malignancy. Although ANCA is a sensitive and specific marker of vasculitides, it can be positive in other conditions particularly hepatitis B, inflammatory bowel disease and autoimmune liver disorders. Malignancy can also be associated with ANCA in the absence of vasculitis. In one study, of 118 ANCA positive patients without ANCA-associated vasculitis, four were found to have malignancy. In a study of 1024 patients who had ANCA tested, 61 patients were found to have malignancy, predominantly haematological and lung cancers. However, after adjustment for sex, age and time of blood draw, no association was found between ANCA status and incidence of cancer. Interestingly, paraneoplastic vasculitis such as polyarteritis nodosa (PAN) has been described in the context of underlying cholangiocarcinoma, and is associated with ANCA rise. Moreover, patients with raised ANCA and PAN also have raised CA 19- 9.
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Background: Viral infections including SARS-CoV-2 may trigger autoimmune disease through T-cell-mediated autoimmune response through molecular mimicry-cross-reactive T-cell recognition or bystander T-cell activation. Autoantibodies have been detected in patients with COVID-19 and some human proteins have homologous regions with SARS-CoV-2 peptides that could function as autoantigens. While there are scattered reports of various autoimmune diseases diagnosed after COVID-19, the risk is not known. Method(s): TriNetX (a global federated health research network providing access to electronic medical records across 72 large healthcare organizations) was utilized to define a cohort of adults 18 years or older seen on or after January 1, 2020 with at least one follow-up visit after an index date. Exposure was defined as COVID-19 diagnosis by ICD10 code or positive laboratory test. Controls did not have COVID-19 (by the same criteria) and were propensity score-matched to patients who had COVID-19 by age and female sex. Index date was the date of COVID-19 diagnosis or first provider visit for any reason during the study period for controls. Outcomes (see table) were assessed starting one month after index date (to exclude prior undiagnosed autoimmune disease) until one year after. Patients with a specific outcome prior to the index date or within one month after the index date were excluded from the analysis for that outcome. Incidence by COVID-19 exposure status and risk ratios for each outcome were assessed. Result(s): 4,016,472 patients were included (2,008,236 in both groups). Overall, mean (SD) age was 49.2 (17.9) and 57.7% were female. Patients who had COVID-19 were more likely to be white (63 vs 56.9%;p< 0.001). Rheumatoid arthritis, psoriasis and type 1 diabetes mellitus had the highest incidence after COVID-19 (0.24, 0.22 and 0.19%, respectively). While the incidence of most of the autoimmune diseases assessed were low in both groups, the risk ratios for all but one condition (Grave's) showed statistically significant higher risk in patients after COVID-19 than in those without COVID-19 (see table). Risk ratios were highest for polyarteritis nodosa (4.43, 3.27-6.01), reactive arthritis (3.56, 2.05-6.2) and ANCA-associated vasculitis (3.36, 2.6-4.34). Conclusion(s): Autoimmune diseases were more likely to be diagnosed within the first year after COVID-19 than in age-, sex-matched controls. Future work will assess the validity of autoantibodies in predicting autoimmune disease after COVID-19. (Table Presented).
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Polyarteritis nodosa (PAN) is a rare autoimmune disease that affects medium-sized arteries and causes inflammation and damage to the blood vessel walls. Testicular pain is an uncommon symptom of PAN but can occur in rare cases. This specific symptom may be useful in diagnosing older patients with limited tissue access because of their vulnerability and high risk for biopsy complications. We report the case of a 78-year-old male patient with progressive fatigue and walking difficulty. After ruling out various forms of vasculitis and malignancy, we diagnosed the patient with PAN and intensively treated him with rituximab, which successfully cured his symptoms. This case report highlights the importance of intensively ruling out possible diagnoses mimicking vasculitis and treating vasculitis with a tentative diagnosis of PAN in older patients in rural hospitals. The progressive clinical course of vasculitis may devastate older patients' activities of daily living (ADLs). PAN may particularly affect older patients with possible hepatitis B infections. Thus, shared decision-making and prompt intensive treatment should be considered.
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BACKGROUND: Several cases of skin and central nervous system vasculopathy associated with COVID-19 in children have been published, but the information is rather limited. Our study aimed to describe these cases of vasculitis associated with COVID-19 in children. METHODS: In the retrospective-prospective case series study we included information regarding four children with COVID-19-associated vasculitis. In every case, we had a morphological description and the etiology was confirmed via real-time polymerase chain reaction during a tissue biopsy. RESULTS: The most involved systems were skin (4/4), respiratory (3/4), cardiovascular (2/4), nervous (1/4), eye (1/4), kidney (1/4), and inner year (1/4). All patients had increased inflammatory markers and thrombotic parameters (D-dimer). No patient met the criteria for multisystem inflammatory syndrome in children. Two patients met polyarteritis nodosa criteria, one met Henoch-Schonlein purpura criteria, and one met unclassified vasculitis criteria. All patients were treated with systemic glucocorticosteroids (two-pulse therapy). Non-biologic DMARDs were prescribed in all cases; 1/4 patients (25%) was treated with intravenous immunoglobuline, and 3/4 (75%) were treated with biologics (etanercept, tocilizumab, and adalimumab). CONCLUSIONS: Vasculitis associated with COVID-19 could be a life-threatening condition; SARS-CoV-2 might be a new trigger or etiological agent for vasculitis and other immune-mediated diseases. Further research and collection of similar cases are required.
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Numerous post-vaccine complications have been reported secondary to the COVID-19 vaccine. Many of these complications are believed to be due to a hyperactive immune system. A 59-year-old woman developed diffuse abdominal pain two days after receiving the mRNA-1273 COVID-19 vaccine (Moderna). A computerized tomography (CT) angiogram of the abdomen and pelvis revealed the presence of numerous vascular irregularities in the celiac axis, bilateral renal arteries, and inferior mesenteric artery consistent with polyarteritis nodosa (PAN), a medium-vessel vasculitis. The patient was managed with intravenous methylprednisolone 500 mg daily for three days and was then placed on oral methotrexate (MTX) 12.5 mg daily for immunosuppressive maintenance treatment. Until now, a limited number of cases of polyarteritis nodosa secondary to the COVID-19 vaccine have been reported. Major mechanisms of post-vaccine autoimmunity are molecular mimicry and autoantibody production. Although rare adverse events from COVID-19 vaccination are possible, there remains an immense benefit to vaccination in preventing COVID-19-related morbidity and mortality.
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Although histological assessment can assist in many dermatological diagnoses, there has been a recent trend to add imaging modalities to improve diagnostic accuracy. Ultrasound uses a large array of frequencies that correspond to various depths in the skin: the subcutaneous layer can be viewed with various frequencies in high resolution. Subcutaneous nodules, deep-seated lesions in the deep dermis and subcutaneous layers, are attractive targets for ultrasound imaging because they often show minimal visual changes on the skin surface. The aim of this work is to review the expanding body of literature regarding the use of ultrasound for the visualization of subcutaneous nodules. A review of the literature was conducted with the Covidence review managing using the electronic databases PubMed, Medline (ovid), Web of Science, and Google Scholar with the search terms 'ultrasound,' 'dermatology,' 'subcutaneous nodule', 'panniculitis,' 'sarcoidosis,' 'granuloma annular' and 'polyarteritis nodosa.' The search yielded 474 results that were screened. A total of 57 articles were included that were written in English and involved the use of ultrasound for the subcutaneous nodules of human patients. These case reports and case series characterized the sonographic features of the subcutaneous nodules in sarcoidosis (irregular hypoechoic nodule with perilesional hyperechoic portion), granuloma annulare (irregular ill-defined hypoechoic nodule with hyperechoic halo) or panniculitis (septal: hypoechoic thickening of septa between the hyperechoic fatty lobules;lobular: diffuse increase in echogenicity of the fatty lobules). Sonography was shown to be a non-invasive and cost-effective technique to capture images in real-time without ionizing radiation. It can help dermatological diagnoses, direct biopsies, facilitate appropriate treatment, and monitor disease progression. More research is required to understand the full potential of ultrasound for a larger variety of dermatologic concerns. Copyright © 2022
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Autoimmune vasculitis is an autoimmune disease that causes various systemic symptoms, such as fever, fatigue, joint pain, and night sweats. Its clinical course depends on the severity of the inflammation, which can cause acute clinical progression of symptoms. Moreover, when the inflammation of the arteries occurs in the deeper parts of the body, a biopsy may be difficult to perform. Here, we report a case of autoimmune vasculitis in an elderly man who visited our hospital with a chief complaint of muscle pain and fever triggered by a rapid paralysis of both lower limbs. Autoimmune vasculitis can cause a variety of systemic symptoms depending on the size of involved arteries, and its clinical course depends on the severity of the inflammation. Prompt diagnosis and simultaneous treatment of symptoms, excluding other likely diseases, prevent the development of severe and long-term complications of autoimmune vasculitis.
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Background: Vacuoles, E1 enzyme, X-linked, autoinfammatory, somatic (VEXAS) syndrome is a recently identifed disorder caused by somatic mutations in the UBA1 gene of myeloid cells. Various manifestations of pulmonary involvement have been reported, but a detailed description of lung involvement and radiologic fndings is lacking. Objectives: To describe lung involvement in VEXAS syndrome. Methods: A retrospective cohort study was conducted of all patients iden-tifed at the Mayo Clinic with VEXAS syndrome since October 2020. Clinical records and chest high resolution computed tomography (HRCT) scans were reviewed. Results: Our cohort comprised 22 white men with a median age of 69 years (IQR 62-74, range 57-84). Hematologic disorders including multiple myeloma, myelodysplastic syndrome and pancytopenia were present in 10 patients (45%), rheumatologic diseases including granulomatosis with poly-angiitis, IgG4-related disease, polyarteritis nodosa, relapsing polychondritis, and rheumatoid arthritis were found in 10 patients (45%), and 4 patients had dermatologic presentations including Sweet syndrome, Schnitzer-like syndrome or drug rash with eosinophilia skin syndrome (DRESS). VEXAS syndrome-related features included fever (18, 82%), skin lesions (20, 91%), lung infiltrates (12, 55%), chondritis (10, 45%), venous thromboembolism (12, 55%), macrocytic anemia (21, 96%), and bone marrow vacuoles (21, 96%). Other manifestations observed were arthritis, scleritis, hoarseness and hearing loss. Median erythrocyte sedimentation rate (ESR) was 69 mm/1st hour (IQR 34.3-118.8) and median C-reactive protein (CRP) of 55.5 mg/dL (IQR 11.4-98.8). The somatic mutations affecting methionine-41 (p.Met41) in UBA1 gene were: 11 (50%) p.Met41Thr, 7 (32%) p.Met41Val, 2 (9%) p.Met41Leu, and 2 (9%) in the splice site. All patients received glu-cocorticoids (GC) (median duration of treatment was 2.6 years);21 (96%) received conventional immunosuppressive agents (methotrexate, aza-thioprine, mycophenolate, leflunomide, cyclosporin, hydroxychloroquine, tofacitinib, ruxolitinib) and 9 (41%) received biologic agents (rituximab, tocilizumab, infliximab, etanercept, adalimumab, golimumab, abatacept). Respiratory symptoms included dyspnea and cough present in 21 (95%) and 12 (55%), respectively, and were documented prior to VEXAS diagnosis. Most of the patients were non-smokers (14, 64%) and obstructive sleep apnea (OSA) was present in 11 patients (50%). Seven patients (32%) used non-invasive ventilation, 6 used C-PAP, and 1 used Bi-PAP. Bronchoalveolar lavage (BAL) was available in 4 patients, and the findings were compatible with neutrophilic alveolitis in 3. Two patients had lung biopsies (2 transbronchial and 1 surgical) that showed ATTR amyloidosis and organizing pneumonia with lymphoid interstitial pneumonia, respectively. Pulmonary function tests were available in 9 (41%) patients and showed normal results in 5;3 patients had isolated reduction in DLCO and 1 with mild restriction. On chest HRCT, 16 patients (73%) had parenchymal changes including ground-glass opacities in 9, septal thickening in 4, and nodules in 3;pleural effusions were present in 3 patients, air-trapping in 3 patients and tracheomalacia in 1 patient. Follow-up chest HRCT was available for 8 patients (36%), the ground-glass opacities resolved in 5 patients, 3 patients manifested new or increased ground-glass opacities, and 1 patient had increased interlobular septal thickening. After 1 year of follow-up, 4 patients (17%) had died;3 due to pneumonia (2 COVID-19,1 bacterial) and 1 due to heart failure. VEXAS flares occurred in 18 patients (82%), the maximum number of relapses was 7, and they were mainly managed with GC and with changes in the immuno-suppressive regimen. Conclusion: Pulmonary involvement was documented by chest HRCT in most patients with VEXAS syndrome. Respiratory symptoms occurred in over one half of patients and about 20% had PFT abnormalities. The pulmonary manifestations of VEXAS are nonspecifc and characterized predominantly by infamma-tory parenchymal involvement.
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Background: Vaccinations against SARS-CoV-2 represent a fundamental tool in controlling the pandemic. To date, data on the safety of anti-SARS-CoV-2 vaccines in patients with rare rheumatic diseases, such as systemic vasculitis, are limited. Objectives: In this study we aimed at evaluating the safety of anti-SARS-CoV-2 vaccines in a multicentric cohort of patients with systemic vasculitis. Methods: Patients with systemic vasculitis from two Rheumatology centres who had received anti-SARS-CoV-2 vaccine were retrospectively examined. The primary outcome was to evaluate, in this multi-centric cohort, the occurence of a disease fare after the administration of the vaccine, defned as development of clinical manifestations related to vasculitis with a concomitant increase in serum infammatory markers. As a secondary outcome we aimed at evaluating, in a monocentric cohort of patients with vasculitis, the occurrence of adverse events (AEs) following vaccine administration compared to healthy controls (HC). Results: We examined 111 patients with systemic vasculitis (n=69 female, n=42 male), with a mean age of 64.3 (± 13) years. Sixty had ANCA-associated vas-culitis (AAV), fourty-two had Giant-Cell Arterities (GCA), five had Periarteritis Nodosa, four had Takayasu's arteritis. One-hundred and five patients received a mRNA vaccine and six a viral vector one. A disease fare occurred in only 2 patients (1.8%) after the frst dose of a mRNA vaccine: both had AAV (microscopic poliangioitis) and developed a pulmunary disease fare (respiratory failure requiring hospitalization and treatment with high-dose glucocorticoids). Of note, one of these patients had multiple previous comorbidities, including a severe COPD. Multivaried analysis, adjusted for age and sex, performed in a single monocentric cohort of patients with systemic vasculitis [n=60 (39 AAV, 21 GCA), 37 female, 23 male, mean age 71 (± 12.5) years] demonstrated a statistically sig-nifcant higher frequency of AEs in vasculitis patients compared to HC (p=0.015) after the frst dose of vaccination. No signifcant differences in the frequency of AEs in vasculitis patients compared to HC after the second dose were detected. All the AEs were mild in both groups (malaise was the most frequently reported);no serious AEs were reported. Conclusion: Our data show a very low incidence of disease fares after the administration of anti-SARS-CoV-2 vaccines in patients with systemic vasculitis. Patients with systemic vasculitis seem more prone to develop mild AEs after the frst dose of the vaccine. Taken together, this data suggest a good risk profile for anti-SARS-CoV-2 vaccine in patients with systemic vasculitis.
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Lower extremity ulcers have significant morbidity, with treatment determined by the underlying disorder. Reported is a 32-year-old female presenting with small skin nodules and bruises across her legs 4 weeks following her second COVID vaccination. These lesions progressed into large, necrotic ulcers over several months. Initial work-up showed widespread pannicular thrombotic vasculopathy with ischemic skin necrosis. The tissue was negative for calcification on Von Kossa histochemistry, and a working diagnosis of subcutaneous thrombotic vasculopathy was suggested. The ulcers progressed despite treatments with corticosteroids, therapeutic anticoagulation, intravenous immunoglobulin, plasmapheresis, sodium thiosulfate, wound care, and repeat debridement. Later debridement specimens demonstrated rare vascular and pannicular calcifications. This finding supports the hypothesis that subcutaneous thrombotic vasculopathy is a precursor to calciphylaxis, the patient's current working diagnosis. However, based on the patient's entire clinical picture, a definitive diagnosis has yet to be found. This report highlights the challenges of working with rare diseases and the importance of multidisciplinary cooperation.
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Introduction: Polyarteritis nodosa (PAN) is a necrotizing vasculitis of medium/small arteries. It is a rare condition, especially in the pediatric age group. Cutaneous PAN (cPAN) is recognized as a separate entity. It is characterized by disease affecting the skin with no major organ system involvement. Objectives: Chronic polyarthritis is much less observed and can simulate juvenile idiopathic arthritis, which can delay diagnosis, as was the case with this patient. Methods: We report a 14-year-old boy, who presented to us with polyarthritis and myalgia as a first manifestation of cPAN. Results: This 14-year-old male adolescent presented with a history of arthritis in right shoulder, ankles, knees, elbows, fingers and toes, associated with myalgia, with partial improvement after the use of non-hormonal anti-inflammatory drugs. Two months later, he was diagnosed with juvenile idiopathic arthritis. Corticosteroids were prescribed in a dose of 20 mg/day, methotrexate 7.5 mg/week and folic acid. There was no clinical improvement. At this time, he start with fever and the appearance of a painful erythematous lesions in the lower limbs and arms, difficulty in walking, which were progressively getting worse, anorexia and weight loss during this period. He also had a history of oropharyngeal infection prior to beginning of these symptoms. At that time, he was treated with azithromycin for 5 days. As there was no improvement in his clinical condition, he sought the Emergency Room where he was admitted for investigation 3 months after the beginning of his symptoms. On physical examination, we found arthritis in the elbows, knees, ankles and joints of the hands, maculopapular rash in the ankles, muscle atrophy in the arms and legs, palpable and painful nodules on the right leg and right foot. Several laboratory tests were requested. Blood investigations showed anemia, leucocytosis, elevated CRP, ESR and ASLO titres, with normal liver and kidney functions, and normal urine 1. Syphilis, HIV, serology for Mycoplasma pneumoniae, leishmaniasis, Paracoccidioides brasiliensis, Epstein-Barr virus, cytomegalovirus, Zika and Chikungunya viruses, dengue, and parvovirus B19 were negative. HBsAg, hep c were negative. CRP and serology was negative for Coronavirus 19 (Covid-19). Chest Xray, echocardiogram, and ultrasound of the abdomen were normal. Lupus anticoagulant, and FR were negative. Other autoantibodies were negative. p-ANCA and c-ANCA were negative. Myelogram with normal immunophenotyping was found. A skin biopsy was suggestive of PAN showing perivascular lymphomononuclear inflammatory infiltrate, sometimes permeating the vascular wall. The presence of eosinophils and neutrophils with outbreaks of leukocytoclasia was noted, an absence of malignancy was also shown. With infectious, hematological and oncological causes aside, pulse therapy with methylprednisolone was started. After the first infusion, a significant improvement in myalgia and arthritis was observed, in addition to the disappearance of febrile peaks. Then, he started a monthly pulse therapy with cyclophosphamide and methylprednisolone. Four months after start this therapy, we observed improvement of skin lesions and in laboratory exams. Conclusion: Cutaneous PAN is a rare disease, especially in the pediatric age group. Its clinical manifestations are quite varied, making early diagnosis difficult. Joint involvement, when it occurs, is characterized by an acute and oligoarticular pattern in the knees and ankles. Chronic polyarthritis is much less observed and can simulate juvenile idiopathic arthritis, which can delay diagnosis, as was the case with this patient. We must consider the diagnosis of PAN in those patients with chronic polyarthritis, associated with cutaneous vasculitic manifestations and increased ASLO.