Diagnosis Of Wegener's Granulomatosis Research Articles

A Wegner's Granulomatosis Risk Prediction Model Based on Machine Learning Algorithms

Introduction: Prediction of Wegener's granulomatosis diagnosis and relapse is a complex process. In this study, we applied machine learning algorithms to predict Wegener's granulomatosis relapse.
 Methods: In this research, 189 patients admitted to Amiralam Hospital were studied and followed for approximately 2 years. Patient features included demographics, organ involvement, symptoms, and other clinical data. Different popular machine learning algorithms were applied for predicting Wegener's granulomatosis relapse, including Support Vector Machines, Random Forest, Gradient Boosting, and XGBoost algorithms. The prediction model performance was measured for the different candidate prediction algorithms using accuracy, precision, recall, and F1-measure. The selected prediction model performance was calculated based on different relapse rates and major relapse occurrence according to Birmingham Vasculitis Activity Score (BVAS) fields.
 Results: Applying different machine learning algorithms, the XGBoost algorithm performed the best. The results indicated that the prediction model's performance increased when calculating higher relapse rate possibilities. The XGBoost model had 82% accuracy while predicting more than one relapse rate and 92% accuracy in predicting more than twice the relapse rate. We also calculated the SHAP value for the prediction model. The results indicated that Cr, BVAS, lymphocyte percentage, vitamin D, nose involvement, alkaline phosphatase, diagnosis age, white blood cell count, erythrocyte sedimentation rate, and initial nose presentation are the 10 most important features according to SHAP value.
 Conclusion: In this study, we have developed Wegener's granulomatosis relapse prediction model using machine learning algorithms. We achieved reasonable precision and recall for early prediction and decision- making regarding Wegener's granulomatosis relapse.

Поражение органа зрения при болезни Вегенера. Клинический случай

The article provides a description of clinical case of patient 53 years old with Wegener's granulomatosis, manifested with necrotizing scleritis of the left eyeball against the background of malignant nose granuloma. The patient underwent sclera plastic surgery twice in order to save the eye. Due to intensive corticosteroid and cytostatic therapy clinical activity of vasculitis was reduced. At the same time was observed further growth of nose granoluma with damage of inferior orbital wall and spread of the tumor to the orbital cavity with the subsequent destruction of the eye. It was performed an enucleation of the left eye with removal of the tumor of the left orbit. The Wegener's granulomatosis diagnosis was confirmed histologically. The problem of treating systemic vasculitis and its complications is very relevant. During 5-year observation period the patient there were noted another complications: septal perforation, oroantral fistula formation, formation of bilateral hearing loss, disintegration of the left lung granuloma. Right eye stayed intact during whole observation period. Prognosis is unfavorable not only for eyesight, but also for patient's life. Keywords: Wegener's disease, granulomatosis with polyangiitis, necrotizing sleritis

Значение рентгеновской компьютерной томографии и ультразвукового исследования орбит в диагностике гранулематоза Вегенера

«Российская офтальмология онлайн» - электронное информационное издание, создается с 1 марта 2010 года под эгидой Российского общества офтальмологов. Основная задача проекта - научно-информационная поддержка специалистов в области офтальмологии.

Strawberry gingivitis – First sign of Wegener's granulomatosis

Wegener's Granulomatosis (WG) is an immunologically mediated rare multisystem disease characterized by necrotizing granulomatous inflammation affecting the upper and lower respiratory tracts, disseminated vasculitis and glomerulonephritis. WG oral lesions are reported to occur in 6%–13% of patients; they were an initial presenting feature in only 2% of cases. A case of strawberry gingivitis as the first sign of WG presented with erythematous and painful localized gingival enlargement affecting the maxillary anterior region of jaw in a 17-year-old boy is reported here. Correlation of histopathology with routine hematoxylin and eosin and special stains such as Grocott-Gomori's methenamine-silver nitrate and periodic acid–Schiff, peripheral blood smear, chest radiographs, Mantoux test and clinical presentation were established in diagnosing this rare entity. By the above-mentioned procedures, we arrived at the diagnosis of WG. The aim of reporting this case was to emphasize that, the dental surgeon often being the first person to examine the oral cavity, should be familiar with the typical appearance of gingiva as “strawberry gingivitis,” its clinical course, diagnostic parameters and adequate management of WG.

ORAL MANIFESTATION OF WEGENER GRANULOMATOSIS: CASE REPORT

An 86-year-old man was evaluated complaining a painful lesion situated in the tongue with 45-day duration. Medical history revealed anemia, heart disease, vasculitis, and glomerulitis associated with the previous diagnosis of Wegener granulomatosis. Positive reaction for antineutrophil cytoplasmic autoantibodies (ANCA) was reported. Intraoral examination revealed an asymptomatic ulceration on the dorsum of the tongue surrounded by a whitish area that measures its 2.0 × 1.0 cm in its largest diameters. Clinical hypotheses were Wegener granulomatosis (GW), fungal infection, or bacterial infection. Incisional biopsy was performed, and the histopathologic diagnosis was an unspecific ulceration. The study of BAAR (alcohol - resistant acid bacillus) and fungal infection were negative. The treatment consisted of topical corticosteroids (clobetasol), oral hygiene guidelines, and photobiomodulation with the diode laser. Lesion completely regressed in 60 days.

Wegener's granulomatosis causing subglottic stenosis: Experiences at a tertiary care hospital of the Eastern India

Abstract Objectives To study the clinical profile and management of Wegener's granulomatosis (WG) with subglottic stenosis (SGS) at a tertiary teaching hospital in Eastern India. Methods This prospective study incorporated a case series of six patients treated between 2007 and 2015 for WG with SGS. The demographic details of the patients, such as age, sex, clinical presentation, laryngeal endoscopy, imaging, laboratory tests, and medical and surgical options, are described. Results Of the six patients, five had laryngeal symptoms, such as hoarseness or breathing difficulty, at the time of presentation. There were four female and two male patients ranging in age from 14 to 62 years. The diagnosis of all six patients was confirmed via histopathological examination. Of the six patients, one had isolated subglottic involvement, and four had a positive antineutrophilic cytoplasmic autoantibody (C-ANCA) test on presentation. All of the patients received immunosuppressant and steroid therapies at the time of diagnosis. Five patients required tracheostomy with subglottic dilatation with cold steel instruments followed by the local injection of steroids and mitomycin-C application. Four patients have shown clinical improvement. Conclusion Although WG is a rare clinical condition, it is often confused with common ailments, which delays diagnosis, and it may involve the subglottis. The subglottis is a vital part of the laryngotracheal airway, and mild obstruction can be life threatening. The accurate and timely diagnosis of WG helps to prevent life-threatening complications, such as SGS.

Mastoiditis and facial paralysis as initial manifestations of Wegener's Granulomatosis

Wegener's Granulomatosis (WG) is characterized by necrotizing granulomas and vasculitis. If left untreated, the prognosis is poor – a 90% mortality rate within 2 years. Several authors have described the otologic manifestations of WG; these authors, however, have not mentioned the stage of the disease in which these findings present – whether as initial manifestations or subsequent to other findings. AimTo describe three confirmed cases of WG with mastoiditis as the first manifestation, progressing to peripheral facial paralysis (PFP). Material and MethodA clinical series study. Patients diagnosed with WG that initially presented with otologic findings are described. ResultsThe three cases presented with unilateral otalgia, otorrhea, and hearing loss associated with ipsilateral PFP. None recovered in spite of the treatment; an investigation of associated diseases was therefore undertaken. Positive ANCA-C titers where detected in all patients, confirming the diagnosis of WG. Clinical improvement was seen after treatment of WG; the PFP regressed and hearing thresholds improved partially. ConclusionComplications of otitis media (mastoiditis and PFP) that do not respond to the usual treatment require an investigation of associated diseases; WG should be included for an early diagnosis to change the prognosis in these patients.

18F-FDG PET/CT Findings Preceded Elevation of Serum Proteinase 3 Antineutrophil Cytoplasmic Antibodies in Wegener Granulomatosis

A 67-year-old woman underwent F-FDG PET/CT after developing a fever of unknown origin. PET/CT revealed intensive FDG uptake at the nasal and lung lesions. On the laboratory data, serum myeloperoxidase antineutrophil cytoplasmic antibodies (ANCA) titer was elevated, although serum directed against proteinase 3 (PR3) ANCA titer was within normal limits. One month after treatment, follow-up PET/CT revealed decreased FDG uptake at the lesions. One year later, serum PR3-ANCA titer elevated, which finally led to a diagnosis of Wegener granulomatosis (WG). WG lesions may be detected earlier by FDG PET/CT than by serum PR3-ANCA titers.

Wegener Granulomatosis-associated Optic Perineuritis

Introdunction: We report two patients with optic perineuritis (OPN) and hypertrophic pachymeningitis in Wegener granulomatosis (WG).Case report: Patient 1: a 74-year-old man developed blurred vision in each eye, sequentially, over a year. In the first episode, visual acuity in the right eye was reduced to no light perception, and in the second episode, the vision in the left eye fell to 20/100. Brain and orbital magnetic resonance imaging (MRI) revealed abnormal enhancement in the meninges and the ipsilateral optic nerve sheath. T2-hyperintense lesions were found along the outer rim of the ipsilateral optic nerve. Seropositive proteinase-3-antineutrophil cytoplasmic antibody (PR3-ANCA), microhematuria and multiple pulmonary nodules suggested the diagnosis of WG. Steroid therapy was initiated 3 months after the first onset, but with no clinical response. At the 2nd episode, rapid administration of steroid ameliorated visual disturbance and MRI lesions markedly. Patient 2: a 72-year-old man developed blurred vision in each eye. Visual acuity measured no light perception in OD and 6/12 in OS. Gadolinium-enhanced MRI disclosed enhancement in the meninges and both optic nerve sheaths. T2-weighted imaging displayed hyperintense lesions along the outer rims of optic nerves. Otolaryngologic examination, seropositive PR3-ANCA and pulmonary nodules supported the diagnosis of WG. Steroid and cyclophosphamide treatment improved visual dysfunction and MRI lesions in the meninges and the optic nerve sheaths.Comment: The morphological similarity and the anatomical continuity between the meningeal and the perioptic tissues suggest that extension of granulomatous inflammation along such tissue planes accounted for visual loss in these two patients with WG.

Intense FDG uptake on PET/CT in the upper and lower respiratory system indicative of Wegener's granulomatosis

Wegener's granulomatosis (WG) is an uncommon systemic vasculitis, which involves the upper and lower respiratory tracts and the kidneys. Because the patients generally present with clinical manifestations that are similar to common diseases, WG may be initially misdiagnosed as infection or malignancy. We report the case of a 55-year-old male presenting with weight loss, cough, hemoptysis, low-grade fever, and pulmonary nodules detected on the thoracic CT scan. Malignancy was initially suspected, so a PET/CT was performed. It demonstrated intense FDG uptake in the upper and lower respiratory system. The diagnosis of WG was based on PET findings, elevated serum levels of inflammatory markers, and the presence of c-ANCA. We consider that the knowledge of FDG-PET/CT findings may help to make an easier and earlier diagnosis of WG.

ANCA-Negative Wegener's Granulomatosis with Multiple Lower Cranial Nerve Palsies

Wegener's granulomatosis (WG) is a systemic vasculitis affecting small and medium-sized vessels with granulomatous formation. Though it is known for respiratory tract and kidney involvement, neurologic manifestation has been also reported. Herein we report a patient who suffered pansinusitis with multiple lower cranial nerve palsies but reached remission by immunosuppressant after the diagnosis of WG. A 54-yr-old female visited with headache, hearing difficulty, and progressive bulbar symptoms. She experienced endoscopic sinus surgeries due to refractory sinusitis. Neurologic examination revealed multiple lower cranial nerve palsies. Vasculitic markers showed no abnormality. Nasal biopsy revealed granulomatous inflammation and vasculitis involving small vessels. Given cyclophosphamide and prednisolone, her symptoms were prominently improved. WG should be considered in the patient with multiple cranial nerve palsies, especially those with paranasal sinus disease. Because WG can be lethal if delayed in treatment, prompt immunosuppressant is warranted after the diagnostic tissue biopsy.

Wegener granulomatosis presenting as refractory otitis media: A case report

Wegener's granulomatosis (WG) is a granulomatous widespread necrotizing vasculitis, sometimes progressing rapidly, characterized by a tendency to involve the upper and lower respiratory tracts, and in most cases, the kidneys. Otologic manifestations may be seen during the course of the disease, however, they are not frequently seen as the first and only presenting feature. The presence of anticytoplasmic antibodies versus neutrophil polymorphonucleate granules (c-ANCA) is highly specific for the diagnosis of WG, being positive in 97% of the cases. The early diagnosis and the timely medical treatment result in high rates of remission of this potentially lethal disease. In this article, we report a case of WG presenting with acute serous otitis media, progressing to suppurative otitis media and granulomas as the first and only symptom, which was refractory to medical therapy during follow-up and which was diagnosed with further examination.

D01 Expanding the spectrum of chorea associated with immune-mediated diseases: two case reports

Background Wegener Granulomatosis (WG) and psoriasis are two rheumatologic diseases in which neurological involvement is quite rare. Case history A 72-years-old male, diagnosis of WG since 1999. Since 2003 he presented simple verbal tics, four limbs involuntary movements. The cognitive profile was in line with an amnestic mild cognitive impairment. Hyperkinetic syndrome improved with low dosages of Tetrabenazine. A 67-years-old woman presented with choreic movements mainly at the left limbs and painful swelling left wrist. Neuropsychological evaluation showed a mild frontal dysfunction. Within 4 months diagnosis of psoriasis was made. Methylprednisolone was given with improvement of chorea, instead tetrabenazine was stopped for parkinsonism. Genetic tests for HD, HDL2, SCA-17 and research for acanthocytis were negative. FDG-PET showed right caudo-putaminal hypermetabolism in the first patient and bilateral basal ganglia hypermetabolism in the second patient. Brain MRI didn9t show any significant alteration in both patients. CSF examination, iron and cupper sieric values were in the normal range. Sieric anti-basal ganglia antibodies (ABGA) showed auto-antigen of 45 kDa. Conclusions ABGA positivity has been described during autoimmune diseases such as Sydenham Chorea, ADEM and PANDAS‏ and post-mortem studies showed perivascular infiltration by lymphocytes in the basal ganglia. Striatal hypermetabolism on FDG-PET with chorea associated with SLE and primary antiphospholipid syndrome has been reported probably (1,2) due to autoantibodies vs basal ganglia neurons. The ABGA positivity does not imply a pathogenetic relationship, however the concomitant striatal hypermetabolism in our cases could extend the spectrum of chorea associated with autoimmune diseases.

Urogenital Manifestations in Wegener Granulomatosis

We describe the main characteristics and treatment of urogenital manifestations in patients with Wegener granulomatosis (WG). We conducted a retrospective review of the charts of 11 patients with WG. All patients were men, and their median age at WG diagnosis was 53 years (range, 21-70 yr). Urogenital involvement was present at onset of WG in 9 cases (81%), it was the first clinical evidence of WG in 2 cases (18%), and was a symptom of WG relapse in 6 cases (54%). Symptomatic urogenital involvement included prostatitis (n = 4) (with suspicion of an abscess in 1 case), orchitis (n = 4), epididymitis (n = 1), a renal pseudotumor (n = 2), ureteral stenosis (n = 1), and penile ulceration (n = 1). Urogenital symptoms rapidly resolved after therapy with glucocorticoids and immunosuppressive agents. Several patients underwent a surgical procedure, either at the time of diagnosis (n = 3) (consisting of an open nephrectomy and radical prostatectomy for suspicion of carcinoma, suprapubic cystostomy for acute urinary retention), or during follow-up (n = 3) (consisting of ureteral double J stents for ureteral stenosis, and prostate transurethral resection because of dysuria). After a mean follow-up of 56 months, urogenital relapse occurred in 4 patients (36%). Urogenital involvement can be the first clinical evidence of WG. Some presentations, such as a renal or prostate mass that mimics cancer or an abscess, should be assessed to avoid unnecessary radical surgery. Urogenital symptoms can be promptly resolved with glucocorticoids and immunosuppressive agents. However, surgical procedures, such as prostatic transurethral resection, may be mandatory in patients with persistent symptoms.

Wegener's Granulomatosis with Pulmonary Fungal Infection: A Case Report and Brief Review

Wegener's granulomatosis (WG) is an autoimmune, necrotizing granulomatous disease of unknown aetiology that affects small and medium blood vessels, and is usually recurrent. Infection is the most frequent cause of death in patients with WG. A case of WG with pulmonary fungal infection in a 50-year-old man is reported. The patient was hospitalized following a 2-month history of haemoptysis and a 1-month history of intermittent fever. Examination and pathology results confirmed a diagnosis of WG with associated pulmonary fungal infection. The patient's condition was complicated by a septic pneumothorax and sinus formation after lung biopsy, and preexisting diabetes and hypertension, which worsened rapidly due to his critical condition. He was treated with glucocorticoids and cyclophosphamide therapy with the goal of controlling these complications, and had no recurrence within the 4-year follow-up period. This case demonstrated the utility of combined glucocorticoid and cyclophosphamide therapy for the treatment of infection in WG.

Wegener’s Granulomatosis Presenting with Bilateral Excavated Nodules

Wegener’s granulomatosis (WG) is a systemic inflammatory disease whose histopathologic features often include necrosis, granuloma formation, and vasculitis of small-to-medium-sized vessels. We report a new case of Wegener’s granulomatosis occurring in a 41-year-old man who was admitted for respiratory symptoms, fever, asthenia, night sweats and weight loss since one month. Chest X-ray and computed tomography showed multiple bilateral pulmonary excavated lesions. A trransparietal lung biopsy was performed. Histological examination showed an area of necrosis, surrounded by a palisade of histiocytes. Some of the histiocytes were multinucleated and formed true granulomas within the wall of the adjacent pulmonary artery. Serology was strongly positive for ANCA. A diagnosis of Wegener granulomatosis was conducted and the patient was started on cyclophosphamide and methylprednisolone as an initial treatment, with a favorable evolution.

Orbital pseudotumor: A single precursor clinical manifestation of Wegener granulomatosis in a ten year old boy

Wegener granulomatosis (WG) is a type of vasculitis characterized by the presence of anti-neutrophil cy-toplasmic antibodies (ANCA) and inflammation of small and medium sized vessels with granulomas formation. Most commonly affected organs include upper and lower respiratory tract, kidneys, eyes, nervous system and skin. Kidneys’ involvement has a central position in the classification, diagnosis, treatment and prognosis of patients with WG, and is characterized by the presence of necrotic glomerulonephritis and clinical manifestations that vary from microscopic hematuria to acute renal insufficiency. We describe a case report of a ten year old boy presenting with microscopic hematuria of glomerular origin and a medical history of orbital pseudotumor two years before his hospitalization due to renal symptoms. Renal biopsy revealed lesions of pauci-immune glome-rulonephritis and findings of granulomatous inflam-mation and necrotizing vasculitis. Serum was positive for p-ANCA antibodies (perinuclear staining pattern ANCA antibodies). These findings led to the diagnosis of WG of generalized form (according to EULAR/ PRINTO/PRES criteria). The patient has been treated with aggressive immunotherapy with the use of ster-oids, cyclophosphamide and mycophenolate mofetil. Disease remission has been established and retained one year after initial diagnosis. Orbital pseudotumor, which is a diagnosis of exclusion, has been the initial disease’s clinical manifestation, even though at that time neither the ocular biopsy nor the immunologic workup had been indicative in terms of WG. Although WG is very rare in children, this disease should always been included in the differential diagnosis in patients with similar clinical manifestations and clinicians should emphasize on the recognition of granulomatous vasculitis in biopsies as well as on repeated tests for ANCA antibodies’ detection in serum. High morbidity and mortality rates [1] of this clinical entity necessitates the early recognition of atypical disease’s forms and the close follow up in cases of uncertain initial diagnosis.

Fluorine-18-fluorodeoxyglucose PET/CT rare finding of a unique multiorgan involvement of Wegener's granulomatosis

Wegener's granulomatosis (WG) is an uncommon autoimmune disorder, which mainly involves the blood vessels, kidneys and respiratory tract. We report an interesting case of WG with unusual multiorgan involvement in a young male who presented with a short history of right-sided otalgia, nasal obstruction and a right parotid mass. His initial CT and MRI scans showed a large parotid mass with features suggestive of malignancy with bilateral cavitating pulmonary nodules suggesting metastatic disease. The imaging-based differential diagnosis was squamous cell carcinoma or adenoid cystic carcinoma. The microscopic findings on ultrasound-guided biopsy of the parotid mass were, surprisingly, those of acute necrotising granulomatous inflammation with some features suggestive of a vasculitic process. A multidisciplinary team discussion and further investigation resulted in the additional findings of haematuria, raised erythrocyte sedimentation rate and positive serum cytoplasmic anti-neutrophil cytoplasmic antibody test, which led to the diagnosis of WG. Subsequently, the patient developed acute urinary retention owing to gross prostatic enlargement related to further disease involvement, which was confirmed with a positive biopsy. Fluorine-18-fluorodeoxyglucose (18F-FDG) positron emission tomography (PET)/CT scan showed disease distribution at the right maxillary sinus/nasal cavity, right parotid, mediastinum, lungs and prostate. To our knowledge, this is the first reported 18F-FDG PET/CT case with multiorgan involvement in a single WG patient. The patient has improved both clinically and on imaging after appropriate treatment with immunosuppressive therapy and steroids. Although 18F-FDG PET/CT imaging did not actually alter the management of this patient, it can help to establish the disease distribution and guide the biopsy.

A model to predict cardiovascular events in patients with newly diagnosed Wegener's granulomatosis and microscopic polyangiitis

To create a prognostic tool to quantify the 5-year cardiovascular (CV) risk in patients with newly diagnosed Wegener's granulomatosis (WG) and microscopic polyangiitis (MPA) without premorbid CV disease. We reviewed CV outcomes during the long-term followup of patients in the first 4 European Vasculitis Study Group (EUVAS) trials of WG and MPA. CV events were defined as CV death, stroke, myocardial infarction, coronary artery bypass graft, or percutaneous coronary intervention. Logistic regression was performed to create a model to predict the absolute risk of a CV event. The model was tested using the Wegener's Granulomatosis Etanercept Trial (WGET) cohort. Seventy-four (13.8%) of 535 patients with 5 years of followup from the EUVAS trials had at least 1 CV event: 33 (11.7%) of 281 WG versus 41 (16.1%) of 254 MPA. The independent determinants of CV outcomes were older age (odds ratio [OR] 1.45, 95% confidence interval [95% CI] 1.11-1.90), diastolic hypertension (OR 1.97, 95% CI 0.98-3.95), and positive proteinase 3 (PR3) antineutrophil cytoplasmic antibody (ANCA) status (OR 0.39, 95% CI 0.20-0.74). The model was validated using the WGET cohort (area under the receiver operating characteristic curve of 0.80). Within 5 years of diagnosis of WG or MPA, 14% of patients will have a CV event. We have constructed and validated a tool to quantify the risk of a CV event based on age, diastolic hypertension, and PR3 ANCA status in patients without prior CV disease. In patients with vasculitis, PR3 ANCA is associated with a reduced CV risk compared to myeloperoxidase ANCA or negative ANCA status.

Central retinal artery occlusion in Wegener's granulomatosis: a diagnostic dilemma

PurposeTo report a case of central retinal artery occlusion (CRAO) in a patient with biopsy-verified Wegener's granulomatosis (WG) with positive C-ANCA.MethodsA 55-year-old woman presented with a 3-day history of acute painless bilateral loss of vision; she also complained of fever and weight loss. Examination showed a CRAO in the left eye and angiographically documented choroidal ischemia in both eyes.ResultsThe possibility of systemic vasculitis was not kept in mind until further studies were carried out; methylprednisolone pulse therapy was then started. Renal biopsy disclosed focal and segmental necrotizing vasculitis of the medium-sized arteries, supporting the diagnosis of WG, and cyclophosphamide pulse therapy was administered with gradual improvement, but there was no visual recovery.ConclusionCRAO as presenting manifestation of WG, in the context of retinal vasculitis, is very uncommon, but we should be aware of WG in the etiology of CRAO. This report shows the difficulty of diagnosing Wegener's granulomatosis; it requires a high index of suspicion, and we should obtain an accurate medical history and repeat serological and histopathological examinations. It emphasizes that inflammation of arteries leads to irreversible retinal infarction, and visual loss may occur