Pulmonary Involvement in Antineutrophil Cytoplasmic Antibodies (ANCA)-associated Vasculitis: The Influence of ANCA Subtype

2017 ◽  
Vol 44 (10) ◽  
pp. 1458-1467 ◽  
Author(s):  
Aladdin J. Mohammad ◽  
Kristian H. Mortensen ◽  
Judith Babar ◽  
Rona Smith ◽  
Rachel B. Jones ◽  
...  

Objective.To describe pulmonary involvement at time of diagnosis in antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV), as defined by computed tomography (CT).Methods.Patients with thoracic CT performed on or after the onset of AAV (n = 140; 75 women; granulomatosis with polyangiitis, n = 79; microscopic polyangiitis MPA, n = 61) followed at a tertiary referral center vasculitis clinic were studied. Radiological patterns of pulmonary involvement were evaluated from the CT studies using a predefined protocol, and compared to proteinase 3 (PR3)-ANCA and myeloperoxidase (MPO)-ANCA specificity.Results.Of the patients, 77% had an abnormal thoracic CT study. The most common abnormality was nodular disease (24%), of which the majority were peribronchial nodules, followed by bronchiectasis and pleural effusion (19%, each), pulmonary hemorrhage and lymph node enlargement (14%, each), emphysema (13%), and cavitating lesions (11%). Central airways disease and a nodular pattern of pulmonary involvement were more common in PR3-ANCA–positive patients (p < 0.05). Usual interstitial pneumonitis (UIP) and bronchiectasis were more prevalent in MPO-ANCA–positive patients (p < 0.05). Alveolar hemorrhage, pleural effusion, lymph node enlargement, and pulmonary venous congestion were more frequent in MPO-ANCA–positive patients.Conclusion.Pulmonary involvement is frequent and among 140 patients with AAV who underwent a thoracic CT study, almost 80% have pulmonary abnormalities on thoracic CT. Central airway disease occurs exclusively among patients with PR3-ANCA while UIP were mainly seen in those with MPO-ANCA. These findings may have important implications for the investigation, management, and pathogenesis of AAV.

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1561-1562
Author(s):  
G. Cassone ◽  
G. Dei ◽  
G. Sambataro ◽  
A. Manfredi ◽  
S. Cerri ◽  
...  

Background:Prevalence of anti-neutrophil cytoplasmic antibody (ANCA) in patient with idiopathic pulmonary fibrosis (IPF) ranges from 1 to 35%, mainly anti-MPO. The presence of ANCA positivity seems to be a poorer prognostic factor in patient with IPF, and some of these patients will develop clinical vasculitis (7-23%).Unfortunately, the majority of the available studies on this topic are retrospective and the real natural history of the disease remains poorly understood.Objectives:Aim of the study was to investigate the clinical, serological and radiologic features of patients with interstitial lung disease (ILD) and positivity for anti-MPO, and to evaluate the survival of this population compared with IPF patients.Methods:We retrospectively analysed 30 patients with ILD and anti-MPO antibodies, without diagnosis of vasculitis, from 3 different rheumatology-pulmonology Italian Center.For each patient, clinical, radiologic and serological data were evaluated. Treatments were also collected, both immunosuppressants or antifibrotic agents.Finally, survival of ILD-MPO patients and of 90 unselected idiopathic pulmonary fibrosis (IPF) patients was compared.Results:Thirty patients were enrolled in the study (see table for the characteristics of the patients).Fibrosing pneumonia was described in 73.3% of patients (usual interstitial pneumonia [UIP] in 19 patients), and 10 patients (33.3%) received antifibrotic drugs, all with UIP pattern. Of interest, 7 patients were treated with immunosuppressants (azathioprine, cyclophosphamide, mycophenolate mofetil), independently by the ILD pattern and 21 (70%) low dosage of steroids.After a median period of 23.5 months (range 11-111), 7 patients developed an ANCA associated vasculitis, while other 3 developed other rheumatic diseases.Finally, when compared with IPF, ILD-MPO patients had a better survival (81.2%±0.9 vs 54.7±0.7 for ILD-MPO and IPF, respectively; p=0.045)Conclusion:ILD positive for anti-MPO antibodies are still a not definite condition. We need larger population to identify possible markers for the evolution in an ANCA associated vasculitis, to define the prognosis of disease and the better therapeutic approach.References: :[1]Mohammad AJ, et al. Pulmonary Involvement in Antineutrophil Cytoplasmic Antibodies (ANCA)-associated Vasculitis: The Influence of ANCA Subtype. J Rheumatol. 2017;44:1458-67Table.Serological, clinical and radiological features of anti-MPO + interstitial lung diseaseNumber30Males/female15/15Median age (years + IQR)68 (17)Median follow-up (months + IQR)39.5 (61)Smoke36.70%ILD pattern Usual interstitial pneumonia63.30% Nonspecific interstitial pneumonia16.70% Hipersensitivity pneumonia10% Other fibrosing pneumonia10%Median FVC (% + IQR)83 (23)Median DLCO (% + IQR)53 (28)Clinical features Raynaud’s phenomenon7.70% Sicca syndrome0 Arthralgias20% Arthritis3.40%Serology Antinuclear antibodies30.80% Anti-extractable nuclear antibodies (ENA)8% Anti-SSA4% Rheumatoid factor21.40%Therapy Immusuppressants23.30% Anti-fibrotic drugs33.30%Disclosure of Interests:None declared


2020 ◽  
Vol 3 (1) ◽  

Rationale and Objectives: Subcutaneous panniculitic T-cell lymphoma (SPTCL) is a rare neoplasm accounting for less than 1% of pediatric Non-Hodgkin Lymphomas. It is most common in young adults with predominance of female cases at 0.5. Symptoms include multiple nodules involving the subcutaneous tissues of the extremities and trunk, neck and face. B symptoms such as fever, chills, night sweats and weight loss, have been reported. Rare extra-cutaneous manifestations include edema, involvement of the bone marrow, lymph nodes, liver, spleen, lungs and viscera. Laboratory abnormalities such as cytopenias and elevated lactate dehydrogenase have been recorded. Hemophagocytic syndrome (HPS) occurs in 33% of cases, which were correlated with fatal outcome. Case Report: We present a rare case of a 17 year-old male diagnosed with subcutaneous pannicultic T-cell lymphoma manifesting with prolonged fever, weight loss, and multiple subcutaneous nodules. He also presented with extra-cutaneous manifestations of facial edema, pleural effusion and ascites, lymph node enlargement, hepatosplenomegaly with jaundice and pancytopenia. Conclusion and Summary: Subcutaneous Panniculitic T-Cell Lymphoma may present with an unusual finding of facial edema, ascites and pleural effusion, lymph node enlargement, hepatosplenomegaly with jaundice and pancytopenia alongside skin manifestations of subcutaneous panniculitis. HPS may complicate the course, treatment decisions and outcome of the disease.


Diagnostics ◽  
2020 ◽  
Vol 10 (12) ◽  
pp. 1041
Author(s):  
Dongjun Lee ◽  
Min Ji Son ◽  
Seung Min Yoo ◽  
Hwa Yeon Lee ◽  
Charles S. White

This study investigated the potential role of paracardiac fat stranding (FS) interspersed with multiple fluid collections (FC) as a clue to differentiate between pleural tuberculosis (pleural TB) and malignant pleural effusion (MPE). The authors retrospectively analyzed chest computed tomography (CT) findings of 428 patients, 351 with pleural TB and 77 with MPE, focusing on the paracardiac fat, and level of pleural adenosine deaminase (ADA) and blood C-reactive protein (CRP). Two radiologists independently evaluated the chest CT findings regarding the paracardiac fat pad ipsilateral to the effusion, including FS, FC, phlegmonous appearance (a combination of the FS and multiple FC), and the presence of lymph node enlargement (>1 cm in short axis diameter). There were significant differences between patients with pleural TB and those with MPE with respect to the prevalence of phlegmonous appearance in the ipsilateral paracardiac fat (47.6% and 10.4%, p < 0.001, OR = 7.8; 95% CI 3.7–16.8) and paracardiac lymph node enlargement (1.4% and 19.5%, p < 0.001, OR = 0.06; 95% CI 0.02–0.2) on CT. In contrast, there was no difference in the prevalence of isolated FS or multiple FC within the ipsilateral paracardiac fat between the two groups. Median pleural ADA and serum CRP level were higher in patients with pleural TB accompanied by phlegmonous appearance in paracardiac fat compared to those without that appearance (ADA: median 104 IU/L versus 90 IU/L, p < 0.001; CRP: 6.5 mg/dL versus 4.2 mg/dL, p < 0.001). In conclusion, phlegmonous appearance in the ipsilateral paracardiac fat without paracardiac lymph node enlargement on chest CT favors a diagnosis of pleural TB over MPE.


2021 ◽  
Vol 2021 ◽  
pp. 1-10
Author(s):  
Jing Wang ◽  
Chen Xia ◽  
Ashutosh Sharma ◽  
Gurjot Singh Gaba ◽  
Mohammad Shabaz

Background. In this day and age, 17% of children less than 5 years of age died of pneumonia; it is the common cause of children death. It is one of the main children respiratory infectious diseases, i.e., mycoplasma pneumonia (MP). The imaging examination can be adopted to quickly observe the morphology and scope of the pulmonary lesions and know the effect of disease treatment and subsequent changes in the disease in order to provide a basis for treatment. Therefore, the most commonly applied technology for detecting pneumonia in children is imaging technology, including chest X-ray and CT. Objectives. The main objective of the work is to investigate the chest computed tomography (CT) findings of children patients with Mycoplasma pneumoniae pneumonia (MPP) and MP combined with streptococcal pneumonia (SP). The mixed infection of MP and SP is very common clinically, and the diagnosis of this type of mixed pneumonia is a critical research topic faced by pediatric respiratory physicians. The comparison is done on the incidence of bronchial and pulmonary interstitial lesions, the degree of lymph node enlargement, the volume and depth of pleural effusion, and the location and morphology of the pulmonary lesions in the chest CT images of children patients from the two groups. Methods. There were comparisons on the incidence of bronchial and pulmonary interstitial lesions, the degree of lymph node enlargement, the volume and depth of pleural effusion, and the location and morphology of the pulmonary lesions in the chest CT images of children patients from the two groups. All the experiments are done in the MATLAB. Results. The results showed that the proportions of reticular shadow, ground glass shadow, bronchial inflation phase, tube wall thickening, and vascular bundle thickening on the CT images of children patients from the MPP group were dramatically higher than those of the MP + SP group ( P < 0.05 ). The maximum transverse diameter of enlarged lymph node in children patients from the MPP group was obviously larger than the diameter of the MP + SP group ( P < 0.05 ). The number of children patients with pleural effusion was 22 in the MP + SP group, which was greatly higher than the MPP group ( P < 0.05 ). Conclusion. In conclusion, the chest CT images of children patients from the MPP group were mainly pulmonary interstitial changes. Furthermore, the alveolar inflammation could be observed on the CT images shown when children patients were combined with SP infection. The more obvious manifestations were that the flaky shadows appeared in the lungs, the pleural effusion became thicker, and the transverse diameters of enlarged lymph nodes were bigger.


2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1557.1-1557
Author(s):  
C. Pena ◽  
F. Savy ◽  
A. C. Costi ◽  
L. Garcia ◽  
M. García

Background:The lung in ANCA associated vasculitis (AAV) is one of the most frequently compromised organs (20-80%). The clinical features of pulmonary involvement vary according to the type of vasculitis and some studies have shown its association with the ANCa subtype or antigenic specifity (MPO-ANCA), (PR3-ANCA).Objectives:A-Describe the clinical features and tomographic findings of pulmonary involvement in vasculitis associated with ANCA and its association according to the ANCA subtypes.B-Evaluate outcome, relapses and associated mortality.Methods:Observational, analytical, retrospective study. Data was collected from the medical records and tomographic image files of patient evaluated in rheumatology department in a tertiary level hospital (2007-2019).Patients diagnosed with AAV, who met criteria for ACR 1990 classification or according to nomenclature of Chapel Hill 2012, with thoracic CT performed and dosage PR3 an MPO antibodies by ELISA technique by a pulmonologist and radiologist.Demographic data, subtype of vasculitis, concomitant organic involvement, disease activity evaluated by Birmingham Vasculitis Activity Score v 3 (BVAS v3), time of evolution of pulmonary involvement, ERS – PCR, serum creatinine, ANCA determined by ELISA were collected. The following findings in parenchyma were evaluated by thorax CT: Consolidation, Ground glass opacities, Reticulation, Honeycomb, cavitated or not nodules, Central airway compromise (thickening or stenosis), Bronchiectasis,Peribronchial thickening, Pleural effusion and the following patterns of disease:NIU (Usual Interstitial Pneumonia), NINE (Unspecified Interstitial Pneumonia),HAD (Diffuse Alveolar Hemorrhage)Results:66/87 patients were included, 59% female, with a mean age of 51 (14 SD) years. GPA 46.9%, MPA 39.4%, EGPA13.6%, median follow-up time of 36 months (RIC 12-77).According to antigenic specificity: 40.9% PR3 positive, 47.6% MPO positive and ANCA negative 11.5%. 74% of the GPA were positive for PR3, and 58% PAM at MPO.BVAS basal: 17.8 + 7.5 DS.Frequency of organic involvement: 81.8% pulmonar, 77% systemic, 57.6% renal, 43% ENT.54% of patients with pulmonary involvement was present at the onset of their disease.Table 1.shows the main findings in lung parenchyma.Parenchymal Findings%Consolidations37Ground glass opacities72Reticulation15Honeycomb9No cavitated nodules41Cavitated nodules20.7Central airways comp (stenosis)9.4Peribronchial thickening11.3Bronchiectasis9.4Pleural effusion7.7According to the pattern of tomographic condition: 36.5 HAD %, 9.6% NIU, 7.8% NINE and, 1.5% Bronchiolitis obliterans.The presence of positive MPO was significantly associated with the presence of honeycomb (p 0.017) and NIU (p 0.018).There were no significant associations with the presence of PR3.Mortality was 17%.No association was found in relation to mortality or relapse frequency among PR3 or MPO positive patients.Conclusion:The frequency of pulmonary involvement in this cohort of patients was 82%, similar to that reported in the literature and was presented at the beginning of the disease in half of the cases. The presence of positive MPO was associated with a higher frequency of usual interstitial pneumonia.References:[1]Sophia Lionaki. Classification of ANCA vasculitides: The role of anti-neutrophil cytoplasmic autoantibody specificity for MPO or PR3 in disease recognition and prognosis. Arthritis Rheum. 2012 October; 64(10): 3452–3462. doi:10.1002/art.34562.[2]Kouichi Hirayama. Pulmonary involvements of anti-neutrophil cytoplasmic autoantibody-associated renal vasculitis in Japan Nephrol Dial Transplant (2015) 0: 1–11 doi: 10.1093/ndt/gfu385 .3-Beatrice Feragalli.The Lung in Systemic Vasculitis: Radiological Patterns and Differential Diagnosis. British Institute of RadiologyDisclosure of Interests:None declared


2019 ◽  
Vol 46 (10) ◽  
pp. 1415-1420 ◽  
Author(s):  
Nataliya Milman ◽  
Eilish McConville ◽  
Joanna C. Robson ◽  
Annelies Boonen ◽  
Peter Tugwell ◽  
...  

Objective.Aspects of antineutrophil cytoplasmic antibodies–associated vasculitis (AAV) prioritized by patients with AAV were described using the International Classification of Function, Disability, and Health (ICF).Methods.Items identified during 14 individual interviews were incorporated into an ICF-based questionnaire administered to participants of 2 vasculitis patient symposia: 36 in the United Kingdom and 63 in the United States.Results.Categories identified as at least “moderately relevant” by ≥ 5% of subjects included 44 body functions, 14 body structures, 35 activities and participation, 31 environmental factors, and 38 personal factors.Conclusion.Identified categories differ from those identified by the current Outcome Measures in Rheumatology (OMERACT) core set and those prioritized by vasculitis experts.


2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Tong-Hui Xie ◽  
Peng Su ◽  
Jian-Guo Hong ◽  
Hui Zhang

Abstract Background Colorectal cancer is a very common malignant tumor worldwide. The clinical manifestations of advanced colorectal cancer include the changes in bowel habits, hematochezia, diarrhea, local abdominal pain and other symptoms. However, the colorectal cancer with an initial symptom of cervical lymph node enlargement is extremely rare. In this article, we report a case of rectal cancer presenting with cervical lymph nodes enlargement as the initial symptom. Case presentation A 57-year-old woman was admitted to our hospital for cervical lymph node enlargement which was accidentally detected during physical examination. Computed tomography scan revealed multiple enlarged lymph nodes in the neck. Cervical ultrasound showed normal thyroid gland and multiple left supraclavicular lymph nodes enlargement. The patient underwent lymph nodes biopsy and pathologic results showed metastatic adenocarcinoma. The subsequent lower gastrointestinal endoscopy revealed a mucosal bulge lesion located at rectus and biopsy revealed adenocarcinoma. The patient underwent rectal cancer resection. She is alive with no evidence of recurrence or new tumors 2 years after surgery. Conclusions Cervical lymph node metastasis is a rare metastatic way in colorectal cancer. This is the first case of rectal cancer presenting with cervical lymph nodes metastases as the initial symptom. Surgical resection combined with postoperative chemotherapy improved long-term prognosis of the patient. This rare metastatic way of rectal cancer should be paid attention for clinicians.


2020 ◽  
Vol 11 ◽  
Author(s):  
Gwen E. Thompson ◽  
Lynn A. Fussner ◽  
Amber M. Hummel ◽  
Darrell R. Schroeder ◽  
Francisco Silva ◽  
...  

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