FRI0210 ORBITAL PSEUDOTUMOR AMONG PATIENTS WITH GRANULOMATOSIS WITH POLYANGIITIS – DATA FROM THE POLISH REGISTRY POLVAS

Background:Orbital inflammatory masses have been described as the common manifestation of granulomatosis with polyangiitis (GPA) occuring in 7- 45% of patients.Objectives:Identification and characterization of patients with orbital pseudotumor among Polish patients based on the national vasculitis registry, POLVAS.Methods:Clinical presentation and management of all GPA patients fulfilling ACR criteria or Chapel Hill Consensus Conference definition included to the Polish registry POLVAS who developed orbital masses in the course of GPA were evaluated.Results:Ocular involvement was found in 114 (27%) of 417 GPA patients registered in POLVAS, 34 (8%) developed orbital masses. Mean patients’ age was 47.8 (range from 19-75) yrs., 23 (67%) were women. Forty four per cent of the patients developed tumor at the beginning of the disease, 56% during relapse. Patients’ characteristics on diagnosis of orbital mass: 24 cANCA, 2 pANCA, and 8 ANCA negative, 9% active smokers and 31% past smokers, 29% had localized disease, 21% early systemic and 50% systemic with organ involvement, 29% had other type of ophthalmological involvement before pseudotumor occurred, 88% had active paranasal sinus involvement, 41% lungs, 15% CNS, 15% skin and 6% heart manifestations. Thirty seven per cent of patients had positive nasal swabs cultures, 50% of which were positive for Staphylococcus aureus. In 65%, tumor occurred during steroid therapy (46% had prednisone more than 5mg/d) and 45% on immunosuppressive treatment (19% when treated with AZA, 16% MTX, 6,5% MMF and 3,5% CYC). Due to orbital mass 86.5% were treated with CYC and 13.5% with RTX. Twenty one per cent had complete remission of the pseudotumor, 76% partial remission and in 3% patients there was no response to the treatment; 43% developed visual impairment, 20% suffered from blindness.Conclusion:Orbital inflammatory mass was not common manifestation of GPA among our patients. The mass developed at the beginning or in the course of the disease, even during immunosuppressive treatment. Orbital masses have been resistant to therapeutic interventions and were accompanied by high risk of visual impairment.Disclosure of Interests:Anna Masiak: None declared, Marcin Ziętkiewicz: None declared, Krzysztof Wójcik: None declared, Katarzyna Wawrzycka-Adamczyk: None declared, Radoslaw Jeleniewicz: None declared, Marta Madej: None declared, Joanna Kur-Zalewska: None declared, Katarzyna Jakuszko: None declared, Małgorzata Wisłowska: None declared, Hanna Storoniak: None declared, Michał Komorniczak: None declared, Barbara Bułło-Piontecka: None declared, Iwona Brzosko: None declared, Małgorzata Stasiek: None declared, Eugeniusz Kucharz: None declared, Alicja Dębska-Ślizień: None declared, Maria Majdan Consultant of: Roche, Amgen, Speakers bureau: Roche, Amgen, Jacek Musiał: None declared, Zbigniew Zdrojewski: None declared

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Orbital mass in ANCA-associated vasculitides: data on clinical, biological, radiological and histological presentation, therapeutic management, and outcome from 59 patients

Rheumatology ◽

10.1093/rheumatology/kez071 ◽

2019 ◽

Vol 58 (9) ◽

pp. 1565-1573 ◽

Cited By ~ 4

Author(s):

Cécile-Audrey Durel ◽

Arnaud Hot ◽

Ludovic Trefond ◽

Olivier Aumaitre ◽

Gregory Pugnet ◽

...

Keyword(s):

Visual Loss ◽

Granulomatosis With Polyangiitis ◽

Immunosuppressive Agents ◽

Consensus Conference ◽

Response To Therapy ◽

Therapeutic Management ◽

Eosinophilic Granulomatosis With Polyangiitis ◽

Orbital Mass ◽

First Line Therapy ◽

Line Therapy

Abstract Objective Orbital mass is a rare and sight-threatening manifestation of ANCA-associated vasculitides, which remains a therapeutic challenge. We aimed to describe the presentation, therapeutic management and outcome of ANCA-associated vasculitides-related orbital mass. Methods We conducted a French nationwide retrospective study of patients with orbital mass in the setting of ANCA-associated vasculitides according to ACR criteria and/or Chapel Hill Consensus Conference definitions. Results Fifty-nine patients [33 women, median age 46 (range 7–90) years] were included. Fifty-six (95%) patients had granulomatosis with polyangiitis, two eosinophilic granulomatosis with polyangiitis and one microscopic polyangiitis. Orbital mass was unilateral in 47 (80%) cases, and seemed to develop from ENT involvement in most cases. Orbital mass biopsy was available in 32 (54%) patients, showing lymphoplasmacytic infiltration in 65%, fibrosis in 55%, granulomas in 48% and vasculitis in 36%. All patients but one received glucocorticoids as first-line therapy associated with immunosuppressive agents in 82%, mainly cyclophosphamide. Response to therapy was noted in 52% of patients treated with cyclophosphamide compared with 91% of those treated with rituximab. Twenty-seven (46%) patients required a second-line therapy because of relapse (59%) or refractory course (41%). Sequelae included visual impairment in 28%, with definitive blindness in 17%. Refractory course was associated with PR3-ANCA positivity, visual loss and contiguous pachymeningitis. Conclusion Orbital mass is associated with refractory course and high frequency of sequelae, especially blindness. Refractory course is associated with PR3-ANCA positivity, visual loss and contiguous pachymeningitis.

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Pulmonary Vasculitis

Chest Imaging ◽

10.1093/med/9780199858064.003.0061 ◽

2019 ◽

pp. 355-359

Author(s):

Felipe Martínez

Keyword(s):

Granulomatosis With Polyangiitis ◽

Vascular Wall ◽

Antineutrophil Cytoplasmic Antibody ◽

Small Vessel Vasculitis ◽

Eosinophilic Granulomatosis With Polyangiitis ◽

Goodpasture Syndrome ◽

Work Up ◽

Eosinophilic Granulomatosis ◽

Common Manifestation

Vasculitis refers to inflammation of blood vessel walls that results in vascular wall destruction and ischemic injury to affected organs. Common vasculitides discussed herein include Takayasu arteritis (TAK), giant cell arteritis (GCA), antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides such as granulomatosis with polyangiitis (GPA) and eosinophilic granulomatosis with polyangiitis (EGPA), and anti-glomerular basement membrane (anti-GBM) disease or Goodpasture syndrome. Vasculitides are further subcategorized depending of the size of the predominantly affected vessels: large, medium and small vessel vasculitis. The affected vessel size strongly influences the clinical and imaging manifestations of the disease. Intrathoracic involvement is more common in small and large vessel vasculitides. Diffuse alveolar hemorrhage (DAH), a common manifestation of vasculitis, is considered a syndrome rather than a specific entity and will be discussed in this chapter. However, it should be noted that DAH may also result from non-vasculitic etiologies. The work up and diagnosis of patients with primary vasculitides is challenging and requires close collaboration between the clinician, the radiologist and the pathologist. Radiographic abnormalities are non specific or may be absent. CT and MRI are the imaging modalities of choice for the evaluation and follow up of these patients, and should be considered despite normal radiographics.

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P0212IMMUNOSUPPRESSIVE TREATMENT OF PATIENTS WITH ANCA ASSOCIATED VASCULITIS (AAV) IN CHRONIC DIALYSIS PROGRAM

Nephrology Dialysis Transplantation ◽

10.1093/ndt/gfaa142.p0212 ◽

2020 ◽

Vol 35 (Supplement_3) ◽

Author(s):

Ibolya File ◽

László Ujhelyi ◽

Jozsef Balla ◽

László Bidiga ◽

Trinn Csilla ◽

...

Keyword(s):

Maintenance Therapy ◽

Relapse Rate ◽

Granulomatosis With Polyangiitis ◽

Combined Therapy ◽

Immunosuppressive Treatment ◽

Chronic Dialysis ◽

Eosinophilic Granulomatosis With Polyangiitis ◽

Time To Death ◽

Serious Infection ◽

The Mean

Abstract Background and Aims There are numerous studies on AAV, however, data describing the use of immunosuppressive treatment in AAV patients (pts) on chronic dialysis are limited. Most studies found that the incidence of infection is much higher than that of relapses (0.05-0.1 pt per year), suggesting that immunosuppression should be stopped after 3 months in pts with end-stage renal failure. Material, Method Retrospective analysis of immunosuppressive treatment in pts on chronic dialysis due to AAV. From 1995 to 2019 139 pts were diagnosed with AAV. Among them 38 patients (26 female, 12 male, mean age 58 years) needed chronic dialysis (>90 days) due to severe renal AAV (29), relapsing renal disease (6) or progressive CKD without active vasculitis (3). Results Microscopic polyangiitis was diagnosed in 20, granulomatosis with polyangiitis in 14, eosinophilic granulomatosis with polyangiitis in 4 cases. MPO-ANCA was more frequent than PR3-ANCA (23 vs 12), in one case both ANCAs were positive, and in two cases both were negative. The mean dialysis duration was 55 months (4–180 months). 18 pts (47%) had at least one relapse during the observation period. The mean age, the clinical diagnosis, the type of ANCA did not cause any difference, the dialysis time was longer in the relapsed pts group (72+/-38 vs. 40+/-29 months) compared to pts without relapse. High relapse rate was seen in 6 cases. Three patient had continuously high level of PR3-ANCA without relapse or any sign of disease. Relapses were treated mostly with combined therapy of corticosteroids and oral/intravenous cyclophosphamide followed by azathioprin maintenance therapy. Cortocosteroid alone was used in 7 pts, rituximab in five pts as maintenance therapy. Serious infection (needing hospital care) rate during any immunosuppressive treatment was 0.12 per patient-years. There were 12 deaths on dialysis, the median time to death was 61 months (range 3–132). The deaths rate was higher (9/18 vs. 4/20) in relapsed pts, 4 occurring during immunosuppressive treatment. Causes of deaths were cardiovascular (8), sepsis (3), malignancy (2 pts). Seven pts received kidney grafts, 15 pts remained on dialysis, but in three case dialysis could be discontinued after 16, 35 and 36 months. Conclusion Our study points to higher relapse rate and lower serious infection rate in AAV pts on chronic dialysis compared to previous studies. It seems reasonable to continue immunosuppression above 3 months in pts remaining on chronic dialysis. This approach may decrease the relapse rate preventing damage of other organs, allowing transplantation and in some pts recovery of renal function could be achieved.

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Orbital pseudotumor can be a localized form of granulomatosis with polyangiitis as revealed by gene expression profiling

Experimental and Molecular Pathology ◽

10.1016/j.yexmp.2015.07.002 ◽

2015 ◽

Vol 99 (2) ◽

pp. 271-278 ◽

Cited By ~ 18

Author(s):

James T. Rosenbaum ◽

Dongseok Choi ◽

David J. Wilson ◽

Hans E. Grossniklaus ◽

Christina A. Harrington ◽

...

Keyword(s):

Gene Expression ◽

Gene Expression Profiling ◽

Expression Profiling ◽

Granulomatosis With Polyangiitis ◽

Orbital Pseudotumor ◽

Localized Form

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Retrospective review of tertiary and neurosyphilis cases in Alberta, 1973–2017

BMJ Open ◽

10.1136/bmjopen-2018-025995 ◽

2019 ◽

Vol 9 (6) ◽

pp. e025995 ◽

Cited By ~ 6

Author(s):

Takaaki Landry ◽

Petra Smyczek ◽

Ryan Cooper ◽

Jennifer Gratrix ◽

Lindsay Bertholet ◽

...

Keyword(s):

Retrospective Review ◽

Hiv Positive ◽

Ocular Involvement ◽

Clinical Parameters ◽

Sex Partners ◽

Hiv Status ◽

Same Sex ◽

Notification Rate ◽

Tertiary Syphilis ◽

Common Manifestation

ObjectivesTo review the notification rate and characteristics of tertiary and neurosyphilis cases in Alberta, Canada in the postantibiotic era.MethodsA retrospective review of all neurosyphilis and tertiary syphilis cases reported in Alberta from 1973 to March 2017 was undertaken and cases classified into early neurosyphilis, late neurosyphilis and cardiovascular (CV) syphilis. Variables collected included demographics, sexual partners, HIV status, clinical parameters, symptoms and treatment and distributions were compared between early versus late neurosyphilis and asymptomatic versus symptomatic cases (stratified by early versus late stage). Data were analysed using IBM SPSS Statistics V.19.0.Results254 cases were identified; 251 were neurosyphilis and 3 were CV. No cases of gummatous syphilis were reported. Early neurosyphilis accounted for 52.4% (n=133) and 46.1% (n=117) were late neurosyphilis cases; one (0.4%) case with unknown duration. Three outbreaks of infectious syphilis were identified during the study period and a concurrent rise in both early and late neurosyphilis was observed during the outbreak periods. The most common manifestation of symptomatic neurosyphilis was ocular involvement which was more likely in early neurosyphilis. Relative to late neurosyphilis cases, early neurosyphilis cases were more likely to be younger, Caucasian, born in Canada, HIV positive and reporting same sex partners.ConclusionsOur review of tertiary and neurosyphilis cases found that early and late neurosyphilis cases continue to occur in the context of cycling syphilis outbreaks. CV syphilis cases were extremely rare. Ongoing identification of new cases of syphilis and clinical evaluation of cases for complications continues to be important in the context of global resurgence of syphilis.

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264. OCULAR INVOLVEMENT IN GRANULOMATOSIS WITH POLYANGIITIS: A SINGLE-CENTER COHORT STUDY ON 63 PATIENTS

Rheumatology ◽

10.1093/rheumatology/kez062.038 ◽

2019 ◽

Vol 58 (Supplement_2) ◽

Cited By ~ 1

Author(s):

María Asunción Pérez-Jacoiste Asín ◽

Pierre Charles ◽

Pierre-Raphaël Rothschild ◽

Benjamin Terrier ◽

Antoine Brézin ◽

...

Keyword(s):

Cohort Study ◽

Granulomatosis With Polyangiitis ◽

Ocular Involvement ◽

Single Center

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Anesthesia and Intensive Care Management in a Pregnant Woman with PRES: A Case Report

Case Reports in Anesthesiology ◽

10.1155/2012/745939 ◽

2012 ◽

Vol 2012 ◽

pp. 1-5 ◽

Cited By ~ 3

Author(s):

Ismail Demirel ◽

Ayse Belin Ozer ◽

Mustafa K. Bayar ◽

Salih Burcin Kavak

Keyword(s):

Intensive Care ◽

Pregnant Woman ◽

Visual Impairment ◽

Care Management ◽

Relevant Literature ◽

Immunosuppressive Treatment ◽

Acute Hypertension ◽

Cortical Visual Impairment ◽

The Right ◽

Reversible Encephalopathy

Posterior reversible encephalopathy syndrome (PRES) is a temporary condition that is diagnosed clinically, neurologically, and radiologically. Its symptoms vary, and nonspecific headaches, confusion, impairment of consciousness, nausea, vomiting, and visual impairment may occur. Acute hypertension often accompanies these symptoms. Patients can also suffer from convulsions, cortical visual impairment, and coma. Diagnosis can be difficult due to focal neurologic signs. Nevertheless, knowing the clinical risk factors can lead to the right diagnosis. It has been reported that this condition may also occur during organ transplantation, immunosuppressive treatment, and autoimmune diseases and chemotherapy, and also patients with eclampsia. In this paper, a 21-year-old, 31-week pregnant woman, who had been diagnosed with PRES and thanks to early diagnosis and treatment had fully recovered and discharged from the intensive care unit, is presented, and the relevant literature is discussed.

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Granulomatosis with Polyangiitis Presenting as Pyrexia of Unknown Origin, Leukocytosis, and Microangiopathic Haemolytic Anemia

Case Reports in Rheumatology ◽

10.1155/2017/6484092 ◽

2017 ◽

Vol 2017 ◽

pp. 1-6

Author(s):

Sima Terebelo ◽

Iona Chen

Keyword(s):

Granulomatosis With Polyangiitis ◽

Immunosuppressive Treatment ◽

Kidney Injury ◽

Unknown Origin ◽

Antineutrophil Cytoplasmic Antibodies ◽

High Dose ◽

Normal Range ◽

Pyrexia Of Unknown Origin ◽

History Of ◽

Haemolytic Anemia

A 66-year-old woman presented to the Emergency Department with a florid sepsis-like picture, a two-week history of fever, relative hypotension with end organ ischemia (unexplained liver enzyme and troponin elevations), and nonspecific constitutional symptoms. She was initially found to have a urinary tract infection but, despite appropriate treatment, her fever persisted and her white blood cell count continued to rise. During her hospitalization the patient manifested leukocytosis to 47,000 WBC/μL, ESR 67 mm/hr (normal range 0–42 mm/hr), CRP 17.5 mg/dL (normal range 0.02–1.20 mg/dL), and microangiopathic haemolytic anemia, with declining haemoglobin and haematocrit. An infectious aetiology was not found despite extensive bacteriologic studies and radiographic imaging. The patient progressed to acute kidney injury with “active” urinary sediment and proteinuria. Kidney biopsy results and serological titres of myeloperoxidase positive perinuclear-antineutrophil cytoplasmic antibodies (MPO+ p-ANCA) led to a diagnosis of granulomatosis with polyangiitis. Immunosuppressive treatment with high dose methylprednisolone and rituximab led to resolution of the leukocytosis and return of the haemoglobin and haematocrit values toward normal without further signs of hemolysis.

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Breast involvement in granulomatosis with polyangiitis: a case report

Egyptian Journal of Radiology and Nuclear Medicine ◽

10.1186/s43055-021-00569-z ◽

2021 ◽

Vol 52 (1) ◽

Author(s):

Bhavya Basetti ◽

Gokulakrishnan Periakaruppan ◽

Arunan Murali ◽

Bhawna Dev ◽

Prabhu Radhan Radhakrishnan ◽

...

Keyword(s):

Breast Lesion ◽

Granulomatosis With Polyangiitis ◽

Immunosuppressive Treatment ◽

Imaging Features ◽

Multisystem Disorder ◽

Multisystem Disease ◽

Histopathological Correlation ◽

Multisystem Involvement ◽

Immune Mediated ◽

The Right

Abstract Background Granulomatosis with polyangiitis (GPA) previously known as Wegener's granulomatosis is a rare complex immune-mediated disease and a multisystem disorder with varying presentations where many of the imaging features of GPA overlap with those of other diseases, and hence its diagnosis can be challenging. Case presentation Here we report a case of a 48-year-old female who presented with complaints of hemoptysis, difficulty in speech, bilateral progressive hearing loss and, a palpable lump in the right breast. The case was initially misdiagnosed as breast carcinoma but biopsy proved to be GPA. Here we present the multisystem manifestations of GPA involving the head and neck, breast, lungs, spleen with involvement of the breast being very rare. The breast lesion showed improvement with steroids on follow-up. Conclusions GPA is a multisystem disease and though breast lesions in GPA are rare, a thorough knowledge about this uncommon manifestation is required and clinical, radiological, and histopathological correlation is of utmost importance in the evaluation of a breast lesion in cases of multisystem involvement to avoid unnecessary surgeries. The differential diagnosis of breast tumors should include such rare conditions that can sometimes mimic breast cancer. Early diagnosis and initiation of the immunosuppressive treatment help in a better prognosis of the disease.

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Bioluminescent Ross River Virus Allows Live Monitoring of Acute and Long-Term Alphaviral Infection by In Vivo Imaging

Viruses ◽

10.3390/v11070584 ◽

2019 ◽

Vol 11 (7) ◽

pp. 584 ◽

Cited By ~ 6

Author(s):

Essia Belarbi ◽

Vincent Legros ◽

Justine Basset ◽

Philippe Desprès ◽

Pierre Roques ◽

...

Keyword(s):

Immunosuppressive Treatment ◽

Treatment Strategies ◽

Chronic Phase ◽

Therapeutic Interventions ◽

In Vivo Evaluation ◽

Viral Spread ◽

Bioluminescent Signal

Arboviruses like chikungunya and Ross River (RRV) are responsible for massive outbreaks of viral polyarthritis. There is no effective treatment or vaccine available against these viruses that induce prolonged and disabling arthritis. To explore the physiopathological mechanisms of alphaviral arthritis, we engineered a recombinant RRV expressing a NanoLuc reporter (RRV-NLuc), which exhibited high stability, near native replication kinetics and allowed real time monitoring of viral spread in an albino mouse strain. During the acute phase of the disease, we observed a high bioluminescent signal reflecting viral replication and dissemination in the infected mice. Using Bindarit, an anti-inflammatory drug that inhibits monocyte recruitment, we observed a reduction in viral dissemination demonstrating the important role of monocytes in the propagation of the virus and the adaptation of this model to the in vivo evaluation of treatment strategies. After resolution of the acute symptoms, we observed an increase in the bioluminescent signal in mice subjected to an immunosuppressive treatment 30 days post infection, thus showing active in vivo replication of remnant virus. We show here that this novel reporter virus is suitable to study the alphaviral disease up to the chronic phase, opening new perspectives for the evaluation of therapeutic interventions.

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