Comparison of characteristics of muscle magnetic resonance imaging findings in patients with antineutrophilic cytoplasmic antibody-associated vasculitis and polyarteritis nodosa.

AIM: This study aimed to analyze the muscle magnetic resonance imaging (MRI) findings of patients with antineutrophilic cytoplasmic antibody-associated vasculitis (AAV) and polyarteritis nodosa (PAN) presenting with clinical symptoms in the extremities. METHODS: Retrospective analysis was conducted on short tau inversion recovery MRI findings, with a focus on intramuscular vessels displaying abnormal perivascular signals, in 22 and eight patients with AAV and PAN, respectively. The number per unit area (4 cm2) and diameter of abnormal vessels on muscle MRI were compared between patients with AAV and those with PAN. Cut-off values, clinical sensitivity, and specificity for these indices were calculated from the receiver operating characteristic curves to distinguish between AAV and PAN, and the relationship between the indices and clinical findings in AAV was analyzed. RESULTS: The number of abnormal vessels per unit area was significantly higher in AAV compared to PAN (p < .05). Additionally, the diameter of the abnormal vessels was significantly higher in PAN than in AAV (p < .05). The presence of >6.44 abnormal vessels per unit area or ≤3.61 mm diameter of abnormal vessels was able to predict AAV (sensitivity, 0.955; specificity, 0.625). AAV patients with peripheral neuropathy exhibited a significantly higher number of abnormal vessels per unit area than those without peripheral neuropathy (p < .05). CONCLUSIONS: Muscle MRI can detect small- to medium-vessel vasculitis and be a valuable tool for distinguishing between patients with AAV and PAN experiencing clinical symptoms in the extremities.

Nailfold videocapillaroscopy in antineutrophil cytoplasmic antibody-associated vasculitis.

OBJECTIVE: Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a group of illnesses that cause inflammation and alterations to small vessels in the body. Some of the most common and detrimental manifestations, including alveolar hemorrhage and glomerulonephritis, are caused by this capillary inflammation. We sought to clarify whether patients with AAV would have abnormal nailfold capillaries when evaluated with nailfold videocapillaroscopy. METHODS: Patients with a current diagnosis of AAV and a control group were identified for enrollment. Nailfold videocapillaroscopy images were used for a semiquantitative analysis on capillary density, morphology, dilation, and microhemorrhage after review by 2 rheumatologists. Disease characteristics, occurrence of recent disease flare, and presence of ANCA were recorded. RESULTS: Thirty-three patients with a diagnosis of AAV and 21 controls were recruited. The AAV group had a median age of 59 and 17 (52%) were women. Granulomatosis with polyangiitis was the most common diagnosis (19 [58%]), followed by eosinophilic granulomatosis with polyangiitis (7 [21%]) and microscopic polyangiitis (7 [21%]). Twenty-seven patients (82%) had positive ANCA tests. After assessment of capillary density, dilation, morphology, microhemorrhages, and disorganization, there were no statistically significant differences between the 2 groups. CONCLUSION: There was no evidence of differences in nailfold capillaroscopy abnormalities between those diagnosed with AAV and the control group. While this cohort was relatively small, we did not find a high enough prevalence or specific phenotype of capillary abnormalities that could aid in diagnosis or prognostication of these diseases in the clinical setting.

Association between computed tomography-assessed sarcopenia and mortality in patients with anti-neutrophil cytoplasmic antibody-associated vasculitis.

AIM: Sarcopenia is frequently observed in patients with autoimmune rheumatic diseases; however, its relationship with patient outcomes has not been well understood. This study evaluated the influence of sarcopenia, especially muscle quality, on outcomes of antineutrophil cytoplasmic antibody-associated vasculitis (AAV). METHODS: Records of patients with AAV at the Severance Hospital with computed tomography (CT) images taken at initial disease diagnosis were retrospectively reviewed. For measures of sarcopenia, normal attenuation muscle area (NAMA), low attenuation muscle area (LAMA), intramuscular adipose tissue (IMAT), and total abdominal muscle area (TAMA) in the axial muscles of the middle third lumbar vertebra level were calculated. Correlations between NAMA, LAMA, IMAT, and baseline patient characteristics, as well as the association between the NAMA/TAMA ratio and clinical outcomes were assessed. RESULTS: A total of 136 patients with CT images at AAV diagnosis were identified. Correlation analyses revealed that age, female sex, total cholesterol, and alanine aminotransferase were significantly associated with NAMA. LAMA was associated with age, body mass index (BMI), five-factor score (FFS), and C-reactive protein, and a relationship between IMAT and age and BMI was observed. During the follow up of 31.2 months, 23 (16.9%) patients died, and Cox-proportional hazard analysis demonstrated that a NAMA/TAMA ≤0.46 (odds ratio [OR] 10.247, p < .001), female sex (OR 0.206, p = .006), dyslipidemia (OR 3.143, p = .027), creatinine (OR 1.342, p = .012), and FFS (OR 1.775, p = .046), were independently associated with patient mortality. CONCLUSION: A higher rate of mortality was observed in patients with AAV with NAMA/TAMA ≤0.46, indicating that careful monitoring is required in these patients.

Image findings of anti-neutrophil cytoplasmic antibody-associated vasculitis involving the skull base.

AIM: To investigate computed tomography (CT) and magnetic resonance imaging (MRI) features of skull bases involving anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV). MATERIALS AND METHODS: A retrospective review was undertaken to identify an institutional historical cohort of 17 patients with confirmed AAV who underwent CT or MRI and had skull base involvement between 2002 and 2021. Two radiologists reviewed the extent and features of the lesions, bone changes, and other MRI findings. RESULTS: A total of 17 patients (12 men; mean age ± standard deviation, 46.5 ± 17.1 years) were selected. AAV presented as infiltrative lesions with involvement at various sites. Most cases involved the paranasal sinuses (PNS; 88%, 15/17), nasopharynx (88%, 15/17), pterygopalatine fossa (82%, 14/17), and parapharyngeal space (82%, 14/17), frequently accompanied by mucosal irregularity of the PNS and nasopharynx (71%, 12/17). Central skull base and temporal bone involvement were seen in 53% (9/17) and 38% (6/16) of cases, respectively. On T1-weighted imaging (WI) and T2WI MRI, all lesions (15/15) showed predominant signal iso-intensity to grey matter. CONCLUSIONS: Although radiological findings of AAV are non-specific and skull base involvement is less common, AAV may be considered if infiltrative lesions predominantly involving the PNS, nasopharynx, pterygopalatine fossa, and parapharyngeal space with combined bone changes of skull base are seen.

Examination of nailfold videocapillaroscopy findings in ANCA-associated vasculitis.

OBJECTIVE: The objective of this study was to evaluate nailfold videocapillaroscopy (NVC) as a useful tool for assessing the disease activity of ANCA-associated vasculitis (AAV). METHODS: This study enrolled 51 patients with AAV and 21 healthy controls. We scored NVC findings semiquantitatively, and compared them between AAV patients and controls. We examined the association of NVC findings with disease activity indicators, histopathological findings of skin biopsies, and high-resolution CT (HRCT) scores in AAV. Additionally, we repeatedly rated the NVC findings 3 months after immunosuppressive therapy. RESULTS: Of the 51 enrolled patients, 36 (70.6%) showed a microangiopathy pattern and 4 (7.8%) showed a scleroderma pattern in AAV. The scores for microhaemorrhage, capillary loss, neoangiogenesis, and tortuosity were significantly higher in the AAV group than in the control group. NVC abnormalities correlated with the severity of skin, lung and kidney involvement. The scores of giant capillaries significantly correlated with the total BVAS and the chest BVAS; the scores of capillary loss correlated with the chest BVAS and the renal BVAS. The scores of microhaemorrhage significantly correlated with perivascular inflammatory cell infiltrations in the upper dermis of the purpura and tended to correlate with the total ground-glass opacity and consolidation scores on HRCT. In addition, capillary loss scores had a significant positive correlation with serum creatinine levels. Additionally, the microhaemorrhage scores were significantly reduced after 3 months of immunosuppressive therapy. CONCLUSION: In AAV patients, NVC abnormalities are significantly associated with disease severity. This result suggests that NVC is a useful tool for assessing the disease activity and treatment response in AAV.

ANCA-associated vasculitis with abdominal aortic aneurysm : Case report and literature review.

INTRODUCTION: ANCA-associated vasculitis is an autoimmune disease that usually involves the small vessel walls. It is difficult to find the presence of ANCA-associated vasculitis in the great arteries, especially the thoracic and abdominal aorta. METHODS: This is an 86-year-old Chinese man with ANCA-associated vasculitis and abdominal aortic aneurysm who presented with epigastric pain. Considering his age and physical condition, the patient was treated with methylprednisolone and mycophenolate mofetil instead of surgery. RESULTS: The patient's epigastric pain symptoms were relieved after 2 months of conservative treatment. Imaging at follow-up 2 years later showed signs of aneurysm enlargement because of irregular medication. CONCLUSIONS: Patients with ANCA-associated vasculitis combined with aortic aneurysm often require surgical management. But for patients with stable disease and poor physical conditions, conservative treatment is also an effective treatment method, which can bring benefits to the patient's survival.

Interstitial lung disease in ANCA-associated vasculitis: associated factors, radiographic features and mortality.

OBJECTIVE: To evaluate the prevalence, clinical and radiographic features, and long-term outcomes of interstitial lung disease (ILD) in a United States-based ANCA-associated vasculitis (AAV) cohort. METHODS: In this retrospective cohort study, we identified cases of ILD within the 2002-2019 Mass General Brigham AAV Cohort, a consecutive inception cohort of PR3- or MPO-ANCA+ AAV patients. ILD diagnosis and classification as fibrotic or non-fibrotic were confirmed by review of available chest imaging by two board-certified radiologists. Cox proportional hazard models, with age as the time scale, were used to estimate the association of AAV-ILD with all-cause mortality. RESULTS: Of 684 patients in the MGB AAV Cohort, 91 (13%) had ILD which preceded the diagnosis of AAV by a mean of 2.2 years. AAV-ILD patients were older (67 vs 60 years, P < 0.001) than patients without ILD but the distribution of sex and race was similar. AAV-ILD patients were more often MPO-ANCA+ (93% vs 65%, P < 0.001); among MPO-ANCA+ patients (n = 470), 85 (18%) had ILD. The majority of ILD was fibrotic (76%) and UIP was the most common ILD pattern (42%). The baseline forced vital capacity (FVC) % predicted among ILD patients was 81 ± 20%. Fibrotic AAV-ILD was associated with a 58% higher risk of death (aHR 1.58, 95% CI 1.06, 2.37) compared with AAV patients without ILD. CONCLUSION: ILD is a frequent complication of AAV, especially MPO-ANCA+ AAV, often preceding recognition of AAV. Fibrotic AAV-ILD is associated with a higher risk of death than AAV without ILD.

Manifestaciones pulmonares al diagnóstico de 170 pacientes con vasculitis asociadas a anticuerpos anticitoplasma de neutrófilos: cohorte retrospectiva de un centro universitario / Pulmonary manifestations at the diagnosis of 170 patients with vasculitis associated with anticytoplasmic neutrophil antibodies: retrospective cohort of a university center

INTRODUCCIÓN: Las Vasculitis Asociadas a Anticuerpos Anticitoplasma de Neutrófilos (VAA) son infrecuentes, pero de amplio espectro de presentación. Si bien el consenso de clasificación de Chapel Hill del año 2012, sigue vigente, la tendencia actual es clasificarlas de acuerdo al marcador inmunológico: anti-Proteinasa 3 (PR3) o anti-mieloperoxidasa (MPO). Las manifestaciones pulmonares clásicas son la hemorragia alveolar y los nódulos pulmonares. En los últimos 10 años se ha descrito la enfermedad pulmonar difusa (EPD). Los estudios epidemiológicos son escasos, y suelen representar en su mayoría poblaciones norteamericanas o europeas. El objetivo es describir las características del compromiso pulmonar al debut en VAA en un centro universitario. PACIENTES Y MÉTODO: De un total de 190 pacientes con diagnóstico de VAA se incluyeron 170 en seguimiento en nuestro centro. Se revisaron aspectos clínicos, demográficos, laboratorio e imagenológicos de los pacientes incluidos. RESULTADOS: De los 170 pacientes, 112 (65,88%) presentaron compromiso pulmonar. 106 (94,64%) de los pacientes fueron anticuerpos anti citoplasma de neutrófilos (ANCA) positivos; de estos, 56 (53,27%) MPO (+) y 39 (36,45%) PR-3 (+). Un tercio de los pacientes de ambos grupos presentó hemorragia alveolar. En los pacientes MPO (+) predomina la EPD (53,5%) y en PR-3 (+) los nódulos pulmonares (69,23%). Destaca la baja frecuencia de patología obstructiva asociada. CONCLUSIONES: El compromiso pulmonar en las VAA es prevalente y heterogéneo. En nuestra serie, destaca la frecuencia de EPD en VAA MPO (+), lo que releva la importancia del estudio con ANCA en paciente con diagnóstico y seguimiento por EPD.

INTRODUCTION: Antineutrophil Cytoplasmic Antibodies (ANCA) associated vasculitis (AAV) are uncommon, but of broad spectrum of presentation. Although the 2012 Chapel Hill classification consensus remains valid, the current trend is to classify them according to the immunological marker: anti-Proteinase 3 antibody (PR-3) or anti-Myeloperoxidase antibody (MPO). The classic pulmonary manifestations are alveolar hemorrhage and pulmonary nodules. Interstitial lung disease (ILD) has been described in the last 10 years. Epidemiological studies are scarce, and they usually represent mostly North American or European populations. The objective is to describe the characteristics of lung involvement upon debut in AAV in a university center. PATIENTS AND METHODS: Of a total of 190 patients diagnosed with AAV, 170 were included in follow-up at our center. Clinical, demographic, laboratory and imaging aspects of the included patients were reviewed. RESULTS: Of the 170 patients, 112 (65.88%) had lung involvement. 106 (94.64%) of the patients were ANCA (+); of these, 56 (53.27%) MPO (+) and 39 (36.45%) PR-3 (+). One third of the patients in both groups had alveolar hemorrhage. In MPO (+) patients, ILD predominates (53.5%) and in PR-3 (+) pulmonary nodules (69.23%). The low frequency of associated obstructive pathology stands out. CONCLUSIONS: Pulmonary manifestations in AAVs are frequent and heterogeneous. Locally, the association of ILD and AAV MPO (+) stands out, which highlights the importance of ANCA study in patients with diagnosis and follow-up by ILD.

Imaging characteristics of hypertrophic pachymeningitis due to ANCA-associated vasculitis.

OBJECTIVE: Hypertrophic pachymeningitis (HP) is a rare disorder that causes localized or diffuse inflammatory fibrosis and thickening of the dura mater. Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV)-related HP is the most frequent form of HP. Otitis media with AAV (OMAAV) patients with HP are associated with higher rates of both ANCA-negative phenotypes and disease-related mortality. However, few studies have reported the imaging characteristics of HP due to AAV/OMAAV. Therefore, we investigated this issue in the present study. METHODS: This retrospective study included patients diagnosed with HP between 2011 and 2020 at our hospital. Age, sex, causative disease, serum C-reactive protein (CRP) level, and MRI data were collected from medical records. We compared the locations of MRI enhancement depending on the causative diseases. RESULTS: Of the 18 included patients with HP (mean age, 64.1 ± 2.6 years; range, 33-77 years), 10 (55.6%) were female, 12 (66.7%) were diagnosed with AAV/OMAAV, four (22.2%) were diagnosed as idiopathic, two (11.1%) were diagnosed with invasive Aspergillus mastoiditis. Eleven (61.1%) had cranial neuropathies. Facial nerve paralysis was common in AAV/OMAAV, while abducent nerve paralysis was common in idiopathic HP. Cranial fossa enhancement was most common presentation in patients with HP, whereas inner acoustic canal (IAC) enhancement was seen only in patients with AAV/OMAAV, while HP involving the cavernous sinus was seen only in patients with idiopathic and mastoiditis. CONCLUSION: HP involving the IAC may be a key factor in diagnosing AAV/OMAAV.

Antineutrophil cytoplasmic antibody-associated vasculitis in diffuse large B cell lymphoma / Vasculitis asociada al anticuerpo citoplasma antineutrófilo en el linfoma difuso de células grandes B

No disponible

Hypertrophic spinal pachymeningitis caused by ANCA-associated vasculitis revealed by 18F-FDG PET/CT: A case report.

RATIONALE: Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) can involve the central nervous system in estimatedly 15% of patients. Hypertrophic pachymeningitis causes inflammatory hypertrophy of the cranial or spinal dura mater and patients present with various neurological deficits. ANCA-associated hypertrophic spinal pachymeningitis has rarely been reported in literature. We report a case of AAV presenting with hypertrophic spinal pachymeningitis detected by 18F-FDG PET/CT. PATIENT CONCERNS: A 66-year-old woman diagnosed with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis developed back pain, bilateral lower limb weakness, dysuria, and dysporia 1 month ago. DIAGNOSIS: Contrast-enhanced MRI showed thickening and enhancement of the dura mater in the thoracic cord. Intraspinal hypermetabolism in the corresponding region was observed on 18F-FDG PET/CT. The patient was finally diagnosed with ANCA-associated hypertrophic spinal pachymeningitis. INTERVENTIONS: The patient was treated with a higher dose of prednisone and cyclophosphamide. OUTCOMES: After 2-week treatment, the patient's neurological symptoms improved rapidly and laboratory findings were ameliorated. A repeated contrast-enhanced MRI showed partial improvement of the disease in the thoracic cord. LESSONS: 18F-FDG PET/CT and contrast-enhanced MRI can aid in the clinical diagnosis and surveillance in AAV-associated hypertrophic spinal pachymeningitis and potentially facilitate early recognition and intervention to prevent irreversible neurological impairment.

Evaluation of body composition using computed tomography in patients with anti-neutrophil cytoplasmic antibody-associated vasculitis.

BACKGROUND/AIMS: Measures of body composition, including visceral adipose tissue (VAT), subcutaneous adipose tissue (SAT), and skeletal muscle area (SMA), are considered important prognostic factors in chronic diseases. The association of these measures with auto-inflammatory disorders, such as anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV), remains unclear. We investigated the clinical significance of VAT, SAT, and SMA in patients with AAV. METHODS: Patients with AAV subjected to chest computed tomography (CT), abdominal CT, or positron emission tomography-CT on diagnosis of AAV were evaluated. Quantitative assessment of VAT, SAT, and SMA was performed at the third lumbar vertebral level and computed by summing the pixel attenuation for tissue-specific Hounsfield units in the corresponding region. Associations of VAT, SAT, and SMA with clinical and laboratory data and clinical outcome measures were evaluated. RESULTS: Of the 117 patients, 61 (52.1%) were classified as having microscopic polyangiitis, 28 (23.9%) as granulomatosis with polyangiitis, and 28 (23.9%) as eosinophilic granulomatosis with polyangiitis. VAT significantly correlated with age, weight, body mass index (BMI), and Birmingham Vasculitis Activity Score, whereas SAT correlated with weight, BMI, and creatinine levels. A significant association was found between SMA and age, height, weight, BMI, and the Five-Factor Score. Cox proportional hazards analysis showed that creatinine levels (odds ratio [OR], 1.346; 95% confidence interval [CI], 1.034 to 1.753; p = 0.027) and high VAT (OR, 7.137; 95% CI, 1.343-37.946; p = 0.021) were independently associated with all-cause mortality during follow-up. CONCLUSION: Evaluation of VAT using CT is useful for estimating disease activity and all-cause mortality in patients with AAV.

Relapsing Mitral Involvement in Antineutrophil Cytoplasmic Antibody-Associated Vasculitis.

Cardiac valvular involvement in antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis is very rare. We report the case of a patient seen in 2019, followed for ANCA-associated vasculitis for 15 years, who had a first relapse with cardiac valvular involvement in 2012, and who underwent a second histologically proven vasculitis relapse involving mitral periprosthetic endocardium. PET/CT imaging showed an intense and focal FDG activity of paramitral bioprosthesis leak site. Mitral bioprosthesis was replaced, and the patient improved with steroids and rituximab. Through this exceptional case, we suggest that FDG PET/CT could be of interest in the follow-up of ANCA-associated vasculitis with cardiac valvular involvement.

Usefulness of PET/CT in ANCA associated renal vasculitis / Utilidad de la PET/TC en la vasculitis renal asociada al ANCA

No disponible