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Background: Ocular involvement is relatively common in granulomatosis with polyangiitis (GPA); however, choroidal involvement is rare. We present a case of serous retinal detachment resulting from choroidal involvement in GPA. Case presentation: A 55-year-old male presented to our clinic with bilateral eye redness and pain. Ocular examination revealed bilateral conjunctival injection, and dilation of the episcleral and scleral vessels. Slit-lamp examination revealed anterior chamber cells. Optical coherence tomography (OCT) confirmed serous retinal detachment (SRD) in the left eye. The patient had recently been diagnosed with GPA following a lung biopsy and had received immunosuppressive therapy, including systemic steroids, cyclosporine, mycophenolate mofetil, and rituximab. Five weeks after treatment, the clinical symptoms of the patient, including SRD, improved with alleviation of systemic symptoms. However, tapering systemic steroids and immunosuppressants and discontinuing rituximab led to disease reactivation. OCT demonstrated a recurrence of subretinal fluid, which had previously resolved, and slit-lamp examination showed mild bilateral engorged scleral vessels. Conclusions: Choroidal involvement can present as SRD and may indicate disease activity in patients with GPA. [ABSTRACT FROM AUTHOR]

Objective Von Willebrand factor (VWF) antigen plays a role in vascular inflammation and thrombosis, both of which are important in the pathogenesis of ANCA-associated vasculitis (AAV). Previous work found that VWF correlates with disease activity in childhood-onset primary CNS vasculitis. We sought to determine the relationship between VWF and disease activity over time in children with AAV. Methods AAV patients with more than one VWF level measured were included in this retrospective study, and the relationships between active vasculitis, VWF and other disease measures were analysed. Generalized estimating equations analysis was used to account for repeated VWF measurements within a patient. Repeated measures correlation was used to determine associations of paired laboratory observations. Diagnostic performance was evaluated using receiver operating curve analysis. Results A total of 732 total VWF measurements were collected in 33 AAV patients. VWF antigen levels were higher during active disease [median 2.03 IU/ml, interquartile range (IQR) 1.35, 2.55] compared with inactive disease (median 1.18 IU/ml, IQR 0.94, 1.53). VWF antigen was the only variable that was significantly associated with active disease (odds ratio 3.01, P  < 0.001, 95% CI 2.3, 3.93). The effect of VWF did not show a substantial difference between the disease subtypes. There was a moderate positive correlation between VWF antigen and disease activity, with an acceptable sensitivity and specificity rates. Conclusion Increased VWF antigen levels correlate with active vasculitis in this paediatric-onset AAV cohort and may be used as an additional biomarker in childhood AAV. [ABSTRACT FROM AUTHOR]

No abstract available. Article truncated after 150 words. A 79-year-old man presented to our institution for evaluation of intermittent fevers, profound nasal pain with congestion, cough, sore throat, voice changes, fatigue, generalized weakness, and loose stools which had been progressively affecting the patient for the last 6 months. The patient has a past medical history of ulcerative colitis, hypothyroidism, atrial fibrillation, and hypertension. Just preceding the onset of symptoms, the patient had gone on a month-long trip through Africa and Asia. His symptoms were presumed infectious in the outpatient setting and had responded somewhat to an extended course of ciprofloxacin and metronidazole. The patient had an outpatient head and neck CT that demonstrated significant mucosal thickening of the maxillary sinuses (Figure 3A). An outside hospital CT of the abdomen/pelvis was unremarkable aside from sigmoid diverticulosis. The patient’s significant nasal pain and congestion along with the fevers was suggestive of granulomatosis with polyangiitis (GPA). The differential also included hematologic …

Friedrich Wegener was the author of a comprehensive description of a disease that was named Wegener's granulomatosis. In 2010, its name was changed to granulomatosis with polyangiitis (GPA), the reason being that Wegener's links to Nazism were revealed. The research was conducted between May 2021 and August 2023 and was based on the historical method (understood as defining the subject of research, critical analysis of collected historical source materials, and historiographical synthesis) using the methods of direct and indirect inference ascribed to the historical method. Wegener and his wife were members of numerous Nazi organizations. He was rewarded for his stance by the German Reich. Together with his family, he lived in very comfortable conditions for the standards of the time, unattainable for the average person. He described himself as a frontline combatant in the Nazi invasion of Poland. Friedrich Wegener's links with Nazism were more extensive than has hitherto been known. The links involved his professional and private life. They testify to his membership in numerous Nazi organizations, as well as his active involvement in the activities of various institutions and communities of the German Reich and his overt expression of support for Hitler. Wegener's numerous accolades from the German Reich providing him with professional, material and personal benefits were proof of his links with the Nazi movement. Wegener's association with Nazism helped him to advance his career, and to attain a high social status and quality of life, both before the outbreak and during the Second World War. [ABSTRACT FROM AUTHOR]

Background Granulomatosis with Polyangiitis (GPA) is a rare multi-system autoimmune disorder that may present with cardiac manifestations that are often under-recognized. In this report, we discuss a usual case of a patient who presented as a cardiac emergency with recurrent ST elevation and discuss the approach and management. Case summary A 44-year-old man presented with two episodes of chest pain associated with ST-segment elevation on 12-lead ECG. Under investigation over the past several weeks for fatigue, nasal congestion, and red eyes, his first presentation was associated with widespread ST-segment elevation and an echogenic myocardium suggestive of myocarditis that was confirmed on cardiac MRI. A week later, the development of chest pain, antero-lateral ST elevation, and regional wall motion abnormalities suggested an acute coronary syndrome and he proceeded to primary percutaneous intervention that treated a lesion in the distal left anterior descending artery secondary to coronary arteritis. Diagnosed with GPA, he was started on immunosuppression and has had a resolution of his cardiac involvement at follow-up. Discussion This case report describes an unusual case of myocarditis and coronary arteritis presenting acutely in the same patient and emphasizes the importance of considering systemic autoimmune conditions when encountering primarily cardiac presentations. Early recognition and diagnosis of cardiac involvement will improve the long-term outcomes in these patients. [ABSTRACT FROM AUTHOR]

Wegener's Granulomatosis with polyangiitis (GPA) is a systemic disease of unknown etiology characterized by necrotizing granulomatous inflammation, tissue necrosis, and variable degrees of vasculitis in small- and medium-sized blood vessels. The classical clinical triad consists of involvement of the upper airways, lungs, and kidneys. Saddle-nose deformity is a well-recognized complication of GPA but more commonly occurs due to leprosy, trauma, cocaine abuse, and sarcoidosis. Since this entity is not commonly encountered in our country, we present a unique case of a middle-aged woman presenting with saddle-nose deformity and the diagnostic dilemmas faced by us. This case is being reported due to its rarity in Indian literature.

Background: Ocular involvement is relatively common in granulomatosis with polyangiitis (GPA); however, choroidal involvement is rare. We present a case of serous retinal detachment resulting from choroidal involvement in GPA. Case presentation: A 55-year-old male presented to our clinic with bilateral eye redness and pain. Ocular examination revealed bilateral conjunctival injection, and dilation of the episcleral and scleral vessels. Slit-lamp examination revealed anterior chamber cells. Optical coherence tomography (OCT) confirmed serous retinal detachment (SRD) in the left eye. The patient had recently been diagnosed with GPA following a lung biopsy and had received immunosuppressive therapy, including systemic steroids, cyclosporine, mycophenolate mofetil, and rituximab. Five weeks after treatment, the clinical symptoms of the patient, including SRD, improved with alleviation of systemic symptoms. However, tapering systemic steroids and immunosuppressants and discontinuing rituximab led to disease reactivation. OCT demonstrated a recurrence of subretinal fluid, which had previously resolved, and slit-lamp examination showed mild bilateral engorged scleral vessels. Conclusions: Choroidal involvement can present as SRD and may indicate disease activity in patients with GPA.

Background: The management of subglottic stenosis (SGS) in granulomatosis patients with polyangiitis (GPA) has no clear guidelines. This systematic review aimed to identify different surgical techniques and evaluate the outcomes of applied procedures. Methods: An electronic search was performed using 3 major databases, CINAHL, PubMed, and Clinical key, to include relevant studies published from the databases from inception through January 2017. All primary studies reporting treatment of SGS in cases with GPA were included. Articles were excluded if not relevant to the research topic or if they were duplicates, review articles, editorials, short comments, unpublished data, conference abstracts, case reports, animal studies, or non-English studies. Results: Thirteen papers were included in our systematic review with a total of 267 cases for the qualitative review Endoscopic approaches showed favorable outcomes with the need to use multiple procedures to achieve remission. The open transcervical approach showed excellent results mainly after failure of other endoscopic techniques. Tracheostomy was necessary for severe respiratory obstruction symptoms. Medical treatment was essential for stabilizing the active disease and therefore may enhance the success rate postoperatively. Conclusion: Subglottic stenosis in patients with GPA requires a multidisciplinary approach to provide optimal management regarding disease activity, grade of stenosis, and severity of symptoms. [ABSTRACT FROM AUTHOR]

A Gentleman in his 40s presented to us with left-sided hemifacial pain and nasal discharge for the last three days. He has been on immunosuppressive therapy for Wegener's Granulomatosis for over five years. Crusting was seen in the Left middle meatus upon clinical examination. Based on the post-covid status, steroid therapy, KOH mount, and radiologic imaging, a provisional diagnosis of Sinonasal mucormycosis was considered. He was posted for emergency debridement followed by Liposomal Amphotericin B and steroid therapy. Surgical histopathological specimens were suggestive of Mucormycosis. Limited sinonasal disease with no intracranial, orbital, or palatal involvement was seen in a background of Wegener's Granulomatosis, Covid, and immunosuppressive therapy. Steroids are condemned as a predisposing factor to mucormycosis. Still, the combination of Liposomal Amphotericin B and steroids was used, opening new avenues for managing mucormycosis in patients with vasculitis. The patient is well nine months post-surgery with no signs of disease relapse. [ABSTRACT FROM AUTHOR]

Introduction: The nose and paranasal sinuses are known to be affected by a wide variety of inflammatory, neoplastic, and infectious conditions but the symptoms in each are very similar, thus making the clinical diagnosis very dicey. Case description: This is a report of one such rare case of a middle-ages female who presented with recurrent symptoms fitting into several differentials and was difficult to decipher, each presentation leading to a new diagnosis, when we stumbled upon atrophic rhinitis like symptoms, nasal septal perforation, palatal erosion and later to Wegener's granulomatosis or now known as granulomatosis with polyangiitis (GPA) and finally we found it to be a case of NK T-cell lymphoma of extra nodal variety. The challenges faced in the diagnosis and management have been described in detail. Discussion: Wegener's granulomatosis is itself uncommon in India owing to less reporting and less availability of screening tools which has led us to be more ignorant about its presentation and also its differentials. Both GPA and NK T-cell lymphoma resemble each other closely in histology and need a high degree of suspicion and expertise to differentiate, they have very different treatments and prognoses. We must also distinguish them from the more commoner disease of the tropics which is tuberculosis. Conclusion: The extranodal NK T-cell lymphoma also known as a lethal midline granuloma, which was initially thought to be Wegener's granulomatosis due to their resemblance and the challenges faced in arriving at a final diagnosis emphasizes the rarity of such a case and need for all of us in otorhinolaryngology to familiarize ourselves which such cases, to increase clinical suspicion and also knowledge regarding presentation and management to ensure quick lifesaving treatments as this is a lethal disease. [ABSTRACT FROM AUTHOR]

To share a unique case of granulomatosis with polyangiitis (GPA) identified in a child with CNS involvement, specifically PRES (posterior reversible encephalopathy syndrome). Discuss this uncommon manifestation's clinical characteristics, diagnostic process, and treatment. We are currently discussing a 12-year-old female patient who presented with a chronic cough, shortness of breath, and a new-onset fever. Upon further examination, the patient was diagnosed with GPA, confirmed through positive cytoplasmic antineutrophil cytoplasmic antibodies (C-ANCA), a renal biopsy, and multiple lung cavitary lesions. During her hospitalization, the patient also experienced neurological symptoms, including a severe headache, blurred vision, loss of consciousness, and an abnormal neurological exam, which led to brain MR imaging. The imaging revealed evidence of small vessel vasculitis with confluent T2 hyper signal intensity of gray-white matter junctions in both parietooccipital and frontal lobes containing hemorrhagic components, suggesting Posterior reversible encephalopathy syndrome. This case of Wegener's granulomatosis is noteworthy due to its occurrence in a pediatric patient with CNS involvement, specifically (posterior reversible encephalopathy syndrome). This event highlights the importance of recognizing that autoimmune disorders can present infrequently in young patients. Diagnosing Wegener's granulomatosis can be challenging, particularly when the CNS is affected. However, when appropriate treatment is initiated promptly, favorable outcomes can be achieved, as evidenced by the patient's improved condition with the prednisolone, captopril, and Rituximab treatment plan. Further research is necessary to understand better the underlying pathophysiology and optimal management of CNS involvement in GPA, particularly in the pediatric population.

Granulomatous polyangiitis (GPA) is a rare systemic autoimmune vasculitis disease that is highly correlated with anti-neutrophil cytoplasmic antibodies (ANCAs). It was formerly called as “Wegener’s granulomatosis.” The clinical manifestations are diverse, mainly involving the upper respiratory tract, lungs, and kidneys, and this disease can involve the brain parenchyma as an isolated solid mass. Only one case has been reported thus far. To provide further information on this rare case, we report a case of GPA involving the fourth ventricle and review the relevant literature. A 32-year-old Chinese female developed fever, cough, and shortness of breath for 20 days. An 80 mm × 80 mm skin ulcer was seen on the right lower limb. CT showed multiple large patches of increased density in both lungs. The patient’s serological ANCA was positive. Later, the patient developed dizziness and headache. Magnetic resonance imaging of the head showed a mass of approximately 21 mm × 24 mm in the fourth ventricle. The patient had a craniotomy for mass resection, and macroscopically, the mass was gray–red and measured 25 mm × 20 mm × 20 mm, was soft, had local hemorrhage and necrosis, and had no capsule. The main microscopic features included necrotizing granulomatous vasculitis, the patient’s immunohistochemistry was positive for CD68 and negative for glial fibrillary acidic protein, and the acid-fast staining and hexaamine silver staining were negative. Combined with the clinical history, serology, and imaging, the pathological diagnosis was GPA in the fourth ventricle. The patient was switched to rituximab combined with steroid therapy because she did not tolerate cyclophosphamide. After 5 months of follow-up, the patient’s lung lesions and skin ulcers had completely improved, but the brain lesions had further progressed. When a patient has multiple system diseases, abnormal clinical manifestations, and positive serological ANCAs, a diagnosis of GPA should be carefully considered, and biopsies of easy-to-access sites should be performed. If the patient’s histopathological manifestations include vasculitis, granuloma, and necrosis, a diagnosis of GPA is more likely. If a patient subsequently develops an intraventricular mass, the clinicians should consider a diagnosis of GPA, which can rarely involve the cerebral ventricle to avoid an unnecessary biopsy or surgical treatment of intracranial lesions. When a patient is intolerant to the traditional treatment drug cyclophosphamide and needs to be switched to rituximab, the treatment effect of intracerebral lesions is not ideal; therefore, the treatment of lesions involving GPA in the ventricle is worthy of further exploration.

Granulomatosis with polyangiitis (GPA) is a rare autoimmune disease that affects multiple organs and causes inflammation, necrosis, and vasculitis in small blood vessels. Treatment for GPA involves achieving and maintaining remission. In recent studies, cyclophosphamide-based regimens have been linked to comorbidity hazards, including an increased risk of malignancies, especially hematological ones. Acute myeloid leukemia is the main hematologic malignancy that can complicate GPA. In this context, we report the case of a middle-aged woman with GPA who developed acute promyelocytic leukemia (APL) during maintenance with cyclophosphamide. She was treated with all-trans retinoic acid at 50 mg/day and arsenic trioxide at 10 mg/day, along with steroids. This case highlights the unique emergence of APL in a GPA patient during cyclophosphamide therapy. A single case has previously been reported on the development of APL in a patient with GPA while using azathioprine monotherapy for 2 years.

Key Clinical Message: Here, we report a case of granulomatosis with polyangiitis presenting with unilateral parotid gland enlargement and later developed skin lesions on the lower extremities and abdomen. Although rare, salivary gland enlargement may be the presenting sign of Wegener's granulomatosis or other ANCA associated vasculitides. [ABSTRACT FROM AUTHOR]

Wegener's granulomatosis or Granulomatosis with polyangiitis is a rare multisystem autoimmune vasculitis disease which may become life threatening due to the various system involvement. Granular hyperplastic gingivitis, the so called "strawberry gingivitis" is a rare manifestation of this disease, yet pathognomic when present. Early diagnosis and treatment is very important. [ABSTRACT FROM AUTHOR]

This report presents a rare case of scleritis with peripheral ulcerative keratitis secondary to granulomatosis with polyangiitis (GPA). A 65-year-old Caucasian male presented to a regional ophthalmology service with an atypical red eye. His immune work-up demonstrated positive anti-neutrophil cytoplasmic IgG autoantibodies (ANCA) with anti-proteinase 3 antibody (PR3) elevation. Multi-system vasculitis was discovered including lung, liver, bladder, prostate, nasal and paranasal sinuses involvement. His ocular sequelae included significant peripheral corneal thinning requiring cyanoacrylate gluing, juxtalimbal conjunctival resection, and bandage lens placement. He was treated with systemic methylprednisolone and rituximab achieving remission with ongoing prednisone and methotrexate maintenance therapy. This case demonstrates the importance of recognizing ocular manifestations of GPA as a first presentation of systemic vasculitis.

Granulomatosis with polyangiitis (GPA) is a necrotizing vasculitis which primarily involves upper respiratory tract, lungs and kidneys. Lung involvement is bilateral parenchymal nodules which may cavitate. We present a case of a 60-year-old male with cough, hemoptysis, and fever. Clinical and radiological examination revealed bilateral multilobar cavitating consolidation. Diagnostic bronchoscopy was performed, and bronchial wash grew Klebsiella pneumoniae. Despite sensitive antibiotics, the patient worsened. On further investigations, granulomatosis with polyangiitis (GPA) was proved by serology and histopathological examination of lung biopsy. The patient succumbed to respiratory failure before specific treatment initiation. As there was overlapping Klebsiella infection, underlying pulmonary vasculitis was overlooked. Hence in all cases with hemoptysis and a cavitating consolidation, pulmonary vasculitis should be worked up in order to avoid poor outcomes.

Key Clinical Message Here, we report a case of granulomatosis with polyangiitis presenting with unilateral parotid gland enlargement and later developed skin lesions on the lower extremities and abdomen. Although rare, salivary gland enlargement may be the presenting sign of Wegener's granulomatosis or other ANCA associated vasculitides.

Wegener's granulomatosis is a necrotising vasculitis affecting both arterioles and venules. The classical triad involves acute inflammation of upper airway along with inflammation of lower respiratory tract and renal involvement, however other organ system may also be affected. Our patient presented with severe unilateral earache, ear discharge, hearing loss and ipsilateral facial nerve palsy as the manifestations of the disease, which are rarely reported in medical literature1. [ABSTRACT FROM AUTHOR]

We present a case of granulomatosis with polyangiitis (GPA) presenting with salivary gland enlargement and right parotid abscess, left-sided facial palsy, Lacrimal Gland, extraocular muscle involvement and rapidly progressive necrotizing keratitis. Followed by review of available literature on salivary gland involvement in GPA and how salivary gland involvement may be a good prognostic factor with less renal and central nervous system involvement and argue for greater vigilance in diagnosing salivary gland involvement in GPA. An approach to a patient with salivary gland involvement and suspected autoimmune disease wtih Sjögren's Syndrome, IgG4 disease,Antineutrophil cytoplasmic antibodies (ANCA) associated vasculitis and Sarcoidosis is discussed.

Abstract Background Granulomatosis with polyangiitis (GPA) or Wegener’s granulomatosis is an autoimmune disorder with a wide spectrum of manifestations that mostly primarily presents with respiratory symptoms such as cough, dyspnea, and hemoptysis and leads to a high mortality rate if left untreated. It is a relatively uncommon condition, characterized by necrotizing granulomatous vasculitis of small- and medium-sized vessels. Recent studies have shown that hyperactivation of immune cells in patients with the coronavirus disease 2019 (COVID-19) leads to elevated levels of various autoantibodies and inflammatory cytokines including interferon-gamma (IFN-γ) and tumor necrosis factor-α (TNF-α). There are the same factors that involve in the pathogenesis of autoimmune diseases such as GPA. Case presentation While there have been several reported cases of COVID-19 occurring in patients receiving immunosuppressant treatment for GPA, here we report a case of a 72-year-old woman with a history of coronavirus disease 2019 (COVID-19) who suddenly suffered unilateral vision and hearing loss and peripheral facial palsy on the same side. Chest computed tomography (CT) demonstrated a subpleural consolidation in the inferior lobe of the left lung. Based on the radiology report, chest CT evidence was due to a history of COVID-19 pneumonia. CT scans of the paranasal sinus showed pansinusitis and necrosis of the nasal septum. According to the available evidence, mucormycosis was clinically suspected, and the patient underwent endoscopic sinus surgery. Eventually, the histopathological analysis revealed a diagnosis of Wegener’s granulomatosis. Conclusions Since GPA and its complications can be prevented only through strong clinical suspicion and early diagnosis, our presentation of this case aims to increase awareness of autoimmune diseases in COVID-19 patients even after recovery.

Miranti Pangastuti, Trustia Rizqandaru, Oki Suwarsa, Hartati Purbo Dharmadji, Endang Sutedja Department of Dermatology and Venereology, Faculty of Medicine, Universitas Padjadjaran - Dr. Hasan Sadikin Hospital, Bandung, IndonesiaCorrespondence: Miranti Pangastuti, Department of Dermatology and Venereology, Faculty of Medicine, Universitas Padjadjaran - Dr. Hasan Sadikin Hospital, Jl. Pasteur 38, Bandung, West Java, 40161, Indonesia, Tel +6281223114874, Email pangastuti.miranti@gmail.comAbstract: Bullous dermatoses is a heterogeneous group of blistering skin disorders that can either be inherited or acquired. Subepidermal blisters may result in ulceration and scarring following their rupture. Wegener’s granulomatosis (WG) is a granulomatous necrotizing vasculitis affecting small- to medium-sized blood vessels. It is associated with anti-neutrophil cytoplasmic antibodies (ANCA) and can be manifested cutaneously as multiple bullous and ulcers. A case of WG was reported in an 18-year-old man presented with multiple skin bullous and ulcers. The patient was diagnosed with WG based on the findings from nasopharyngoscopy examination that revealed crusts in his nasal cavity; necrotizing granulomatous appearance on chest radiograph; hematuria on urinalysis; and positive ANCA blood test. This patient received a combination of methylprednisolone and methotrexate, resulting in improvement within four weeks of therapy. His multiple skin ulcers were treated with a combination of dialkyl carbamoyl chloride, hydrocolloid, and hydrogel dressings. This patient was in complete remission state after six months of treatment, which later followed by a relapse episode that occurred within one year. WG with multiple skin bullous and ulcers can mimic other diseases. Various examinations such as histopathology, direct immunofluorescence, and ANCA blood test may aid in determining the etiology of skin bullous and ulcers.Keywords: anti-neutrophil cytoplasmic antibodies, bullous, ulcers, Wegener’s granulomatosis

Abstract Background Granulomatosis with polyangiitis (GPA) is a rare systemic vasculitis. Its severity ranges from indolent disease to fulminant that may cause death. With treatment, remission is seen in more than 80% of cases, although relapse is still common. There have been studies showing that there may be factors to predict relapse in GPA. Based on relapses, the decision to start treatment and/or to monitor the patients more closely is made. Therefore, predicting the relapse of GPA can be effective in controlling the disease. Our aim was to investigate possible factors for relapse in GPA. We recruited 254 patients diagnosed with GPA who were under treatment at Alzahra hospital affiliated to Isfahan University of Medical Sciences (from 2013 to 2020) and Amir Alam Hospital affiliated to Tehran University of Medical Sciences (from 2020 to 2022) to plan a retrospective study. Chi-squared or Fisher’s exact tests were performed to compare categorical variables, while the Mann–Whitney U test was used to compare continuous variables. Results Analysis of our patients (aged 20–80,122 females) showed that 147 patients (57.9%) experienced relapse. Mean age in relapse group was 43.8 ± 16.6 and in no-relapse group was 45.6 ± 15.1 (P > 0.05). Among many potential predictors, we observed in multivariate analysis that positive PR3-ANCA (proteinase 3-antineutrophilic cytoplasmic antibodies) (P = 0.007, OR:2.62,CI:1.29–5.31),nose manifestations (P = 0.004, OR 3.00, CI 1.43–6.26), mucosal membranes involvement (P = 0.009, OR 4.21, CI 1.43–12.38), and gastrointestinal tract (GI) complications (P = 0.03, OR 5.64, CI 1.14–27.90) were significant predictors of GPA relapse. Conclusion Among clinical and laboratory features we studied, positive PR3-ANCA, nose manifestations, mucosal membranes involvement, and GI complications were independent predictors of relapse in patients with GPA.

This report presents a rare case of scleritis with peripheral ulcerative keratitis secondary to granulomatosis with polyangiitis (GPA). A 65-year-old Caucasian male presented to a regional ophthalmology service with an atypical red eye. His immune work-up demonstrated positive anti-neutrophil cytoplasmic IgG autoantibodies (ANCA) with anti-proteinase 3 antibody (PR3) elevation. Multi-system vasculitis was discovered including lung, liver, bladder, prostate, nasal and paranasal sinuses involvement. His ocular sequelae included significant peripheral corneal thinning requiring cyanoacrylate gluing, juxtalimbal conjunctival resection, and bandage lens placement. He was treated with systemic methylprednisolone and rituximab achieving remission with ongoing prednisone and methotrexate maintenance therapy. This case demonstrates the importance of recognizing ocular manifestations of GPA as a first presentation of systemic vasculitis. [ABSTRACT FROM AUTHOR]

We present a case of granulomatosis with polyangiitis (GPA) presenting with salivary gland enlargement and right parotid abscess, left-sided facial palsy, Lacrimal Gland, extraocular muscle involvement and rapidly progressive necrotizing keratitis. Followed by review of available literature on salivary gland involvement in GPA and how salivary gland involvement may be a good prognostic factor with less renal and central nervous system involvement and argue for greater vigilance in diagnosing salivary gland involvement in GPA. An approach to a patient with salivary gland involvement and suspected autoimmune disease wtih Sjögren's Syndrome, IgG4 disease,Antineutrophil cytoplasmic antibodies (ANCA) associated vasculitis and Sarcoidosis is discussed. [ABSTRACT FROM AUTHOR]

ObjectiveTo describe a case series of previously undiagnosed granulomatosis with polyangiitis (GPA) patients who presented primarily with otological manifestations.MethodWe report a series of patients visited at a neurotology clinic who were eventually diagnosed with GPA based on their otologic complaints and had no prior knowledge of having this condition.ResultsIn this series, 10 (91%) patients presented with hearing loss (HL), more than half of which were bilateral (60%). Upon audiometric examination, all but 1 patient had mixed, conductive, or sensorineural HL. All patients presented with eustachian tube dysfunction (ETD), otitis media with effusion (OME), or both. Nasal endoscopy showed intranasal pathology in 3 (27%) patients. Otologic symptoms were improved in all patients after treatment with an average of 4 in-office follow-up appointments.ConclusionGPA should be included in the differential diagnosis of adults with unexplained mixed hearing loss, new onset serous effusion, or acute otitis media in the absence of a previous history of ETD. Laboratory tests (ie, anti-neutrophil cytoplasmic autoantibody, erythrocyte sedimentation rate, and C-reactive protein) along with a urinalysis can aid in screening these patients. In cases in which the index of suspicion is high, repeated testing could reduce the risk of false negative findings.

Abstract Background Granulomatosis with polyangiitis (GPA) is small vessel vasculitis with heterogeneous clinical presentation. In the present population-based cohort study, we classified patients with GPA based on clinical features at presentation using an unsupervised clustering approach and compared their mortality, infections and frequency of comorbidities. Methods In this open cohort study, de-identified primary care data of patients with GPA included in the IQVIA Medical Research Data database between 1 January 1995 and 25 September 2019 was analysed retrospectively. Latent class analysis was performed to create symptom clusters of patients based on 16 categories of symptoms representing various organ involvement. All-cause mortality of resultant clusters was compared after adjusting for age, sex, Townsend deprivation quintile and smoking status at index date using extended Cox proportional hazards models. Prescription of antibiotics, considered as an indirect indicator of recurrent bacterial infection, was compared using a recurrent event model, after adjusting for quarterly use of steroid as a time-dependent covariate. Cumulative frequencies of common comorbidities were compared among the clusters at index visit, 1-year and 3-year follow-up. Results Altogether, 649 patients with GPA [median age 60.0 (IQR: 49.6–70.1)] were included. Three clusters were identified: patients with limited disease mainly with involvement of ENT and cough were classified into cluster 1 (n = 426); cluster 2 had generalised non-renal disease (n = 176); while patients in cluster 3 had renal-predominant disease (n = 47). Many patients in cluster 1 developed generalised disease at the end of 1 year. Mortality in clusters 2 and 3 was higher compared with cluster 1. Mortality in cluster 1 itself was 68% higher than the general population without GPA. The duration of antibiotics prescription and frequency of coexisting medical illnesses was also higher in clusters 2 and 3. Conclusions In a primary care setting, patients with GPA can be classified into three distinct clusters with different prognosis, susceptibility to recurrent infections and presence of comorbidities. The tendency of cluster 1 to evolve into a more generalised disease raises questions about current immunosuppressive treatment approaches in these patients.

ABSTRACT Wegener's granulomatosis is an autoimmune disease that affects the walls of small and medium-sized blood vessels due to an immune complex reaction. Meanwhile, at present, the etiology of the disease is unknown with certainty. One of the diagnoses is the detection of cytoplasmic antineutrophilic cytoplasmic antibody (c-ANCA), but a negative ANCA examination is very rare. Therefore, this is a case report of a 33-year-old man that complained of sores on both legs, which were difficult to heal. The patient also experienced joint pain, fever at night, weight loss, hair loss as well as recurrent nosebleeds with an unknown cause. Furthermore, the physical examination found a saddle nose and black spots from the right and left groin to the back of the legs. Multiple irregular ulcers with different sizes were also discovered in the region cruris and dorsum pedis. The laboratory examination results showed Hb of 8.7 g/dl, 130 mm/hour ESR. Based on peripheral blood smear, the patient was suspected to have hypochromic-microcytic anemia, which caused chronic process along with bleeding. The IF pattern was also speckled with a titer of 1:320, and the ANCA test was negative (-). Meanwhile, the results of routine urine examination found blood +4 macroscopically and observed leukocyturia 2-10 LPB and 8-21 LPB erythrocyturia microsopically. The Doppler ultrasound of the left inferior extremity revealed the swelling of the left pedis soft tissue with peripheral arteritis in the cutis lesion area. The Anatomical Pathology examination showed non-specific chronic inflammation in the cruris and pedis region. Subsequently, the patient was administered with wound debridement by a surgeon, packed red cell (PRC) transfusion, metylprednisolone mg, azathioprine, and cefixime. After the treatment, the nosebleed was no longer felt, the joint pain reduced, and the fever improved. Keywords: Wegener's granulomatosis, Autoimmune vasculitis, negative ANCA

Purpose: To describe the clinical features and management of patients with scleritis associated with granulomatosis with polyangiitis (GPA) at a tertiary eye care center in South India. Methods: The clinical profile and management of patients presenting to a tertiary eye care center in South India with scleritis secondary to GPA from 2003 to 2021 were analyzed retrospectively. Scleritis was classified into anterior diffuse, nodular, and necrotizing scleritis with inflammation according to Watson and Hayreh's classification. Demographic characteristics, clinical features, anti-neutrophil cytoplasmic antibody (ANCA) positivity, treatment response, ocular complications, and status at the last follow-up were analyzed. Statistical analysis of data was performed using Microsoft Excel 2019. Results: Nineteen eyes of 17 patients (15 cytoplasmic staining ANCA [c-ANCA], two p-ANCA positive) were included. Fifteen eyes had necrotizing scleritis, two had diffuse anterior scleritis, and two had nodular scleritis. Remission was induced using a combination of steroids and cyclophosphamide or rituximab. Maintenance therapy was instituted using tapering steroids and immunosuppressants like cyclophosphamide, mycophenolate mofetil, methotrexate, or rituximab. Three eyes required a scleral patch graft. Fourteen patients had good anatomical and visual outcomes, and three were lost to follow-up. Conclusion: GPA is a rare disease, while it is the most common ANCA-associated vasculitis with scleritis. As scleritis may be the presenting sign of the disease, ophthalmologists must be aware of the various features suggestive of GPA. GPA-associated scleritis can have a good prognosis when diagnosed promptly and managed aggressively in the acute stage, and remission is maintained with adequate systemic immunosuppression. [ABSTRACT FROM AUTHOR]

Pulmonary tuberculosis (TB) is the most common cause of bilateral pulmonary cavities with constitutional symptoms in India being endemic and more prevalent nature of the disease, irrespective of microscopy or nucleic acid amplification test abnormalities. Pulmonary manifestations of systemic vasculitis have very diverse involvement ranging from nodule, consolidation, and cavitation. In this case report, a 45-year-old female, presented with constitutional symptoms with lung parenchymal consolidations progressed to cavitation, and started empirical anti-TB treatment without mycobacterial microscopic or genome documentation in sputum with clinical or radiological worsening. Bronchoscopy workup is inconclusive and the tropical screen for bacterial, TB, and malignancy was negative and fungal yield Aspergillus colonization. A vasculitis workup was done in the presence of clinical and radiological worsening documented PR3-antineutrophil cytoplasmic antibody positive with very highly raised titers. We have started on steroids and cyclophosphamide with antifungals and clinical response was documented with a near-complete resolution of shadows in 24 weeks.

Granulomatosis with polyangiitis (GPA), formerly known as Wegener's granulomatosis, is a rare autoimmune vasculitis that primarily affects small to medium-sized blood vessels, typically involving the respiratory tract and kidneys. However, central nervous system involvement, particularly in the form of pachymeningitis, is an exceptionally rare presentation. This case report details a 55-year-old female who presented with pachymeningitis as the initial manifestation of GPA. The patient exhibited non-specific symptoms, including ear pain, voice changes such as hoarseness of voice, and dysphagia, suggestive of neurological deficits affecting lower cranial nerves IX, X, and XII. Diagnostic imaging, particularly MRI, revealed characteristic smooth dural thickening and enhancement, suggesting an inflammatory etiology. Laboratory investigations, including positive cytoplasmic-antineutrophil cytoplasmic autoantibody and anti-proteinase 3 antibodies, confirmed the diagnosis of GPA. The patient was treated with high-dose corticosteroids and mycophenolate mofetil, followed by cyclophosphamide due to a relapse, resulting in significant improvement in her condition. This case underscores the importance of considering GPA in the differential diagnosis of pachymeningitis and highlights the diagnostic challenges posed by its nonspecific symptoms. Early recognition and a multidisciplinary approach are crucial for effective management and prevention of severe neurological complications. The report also emphasizes the need for adherence to treatment regimens to avoid relapses and manage the disease effectively., Competing Interests: Human subjects: Consent was obtained or waived by all participants in this study. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work., (Copyright © 2024, Lankapothu et al.)

Objectives: Physician's evaluation of interstitial lung disease (ILD) extension with high-resolution computed tomography (HRCT) has limitations such as lack of objectivity and reproducibility. This study aimed to investigate the utility of computer-based deep-learning analysis using QZIP-ILD® software (DL-QZIP) compared with conventional approaches in connective tissue disease (CTD) -related ILD., Methods: Patients with CTD-ILD visiting our Rheumatology Centre between December 2020 and April 2024 were recruited. Quantitative scores, including the percentage of lung involvement in ground-glass opacity (QGG), total fibrotic lesion (QFIB), and overall ILD extension encompassing both QGG and QFIB (QILD), calculated by DL-QZIP, were compared with semiquantitative visual method, employing intraclass correlation coefficients (ICC). We compared the capability of QILD scores to distinguish patients with forced vital capacity (FVC) % <70 in both methods determined by the area under the curve (AUC) by the receiver-operating characteristic curve analysis and DeLong's test., Results: Eighty patients (median age, 66 years; 14 men) were included. Median QGG, QFIB, and QILD scores were 3.45%, 2.19%, and 5.35% using DL-QZIP, and 3.25%, 4.06%, and 8.48% using visual method, respectively. Correlations between DL-QZIP and visual method were 0.75 for QGG, 0.61 for QFIB, and 0.75 for QILD. The AUC of QILD scores for FVC% <70 was significantly higher with DL-QZIP (0.833) compared with visual method (0.660) (p < 0.01)., Conclusion: QZIP-ILD® demonstrates superior capability in distinguishing patients with a radiological scenario correlated to severe physiological impairment, while showing relatively good correlations in quantifying the extent on HRCT compared with conventional method in CTD-ILD., (© The Author(s) 2024. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For permissions, please email: journals.permissions@oup.com.)

ABSTRACT: Granulomatosis with polyangiitis formerly known as Wegener's granulomatosis was first described by German pathologist Friedrich Wegener in 1936. It is a multi-system necrotizing noncaseating granulomatous vasculitis which affects small to medium-sized vessels. It can involve any organ system, most commonly the lungs and kidneys. American College of Rheumatology requires 2 of 4 criteria for diagnosis: Positive biopsy for granulomatous vasculitis, urinary sediment with red blood cells, abnormal chest radiograph and oral/nasal inflammation. Here we present a case of Granulomatosis with polyangiitis with brief review of literature.

Abstract Background Granulomatosis with polyangiitis (GPA), also known as Wegener’s granulomatosis, is an idiopathic systemic disease typically affecting the lungs, although other organs may also be involved. Case presentation A 28-year-old male was admitted to Baqiyatallah university hospital in Teheran (Iran) after a 3-week history of fever and productive cough. The patient gradually developed fatigue, arthralgia, hematuria, nausea, vomiting, dyspnea, hemoptysis, weight loss, oliguria and then anuria. Chest-X-ray (CXR) and computerized tomography scan revealed cavitating nodular opacities in the right lung lobe. Furthermore, plasma creatinine increased from 2.2 to 4 mg/dl in a few days. Histopathological examination of kidney biopsy revealed peri-glomerular and peri-vascular inflammation, degeneration and necrosis of the tubular epithelial lining, red blood cell casts, distorted glomerular structure, fibrin thrombi, segmental breaks of the glomerular basal membrane, disruption of Bowman's capsular membrane and crescent formation of the affected glomeruli. An abnormal CXR, an abnormal urinary sediment and a typical kidney histology were used as criteria to diagnose glomerulonefritis with poliangiitis (GPA). Bronchoalveolar lavage smear and PCR turned out positive for mycobacterium tuberculosis. After 3 months of treatment for (GPA) and tuberculosis the patient developed headache and seizure. Cerebral Magnetic Resonance Venography revealed cerebral venous thrombosis of the sinus transverse and sigmoid. Conclusions Tuberculosis may coexist with GPA, as it occurred in our patient. Since a crescentic glomerulonephritis can progress to renal failure, clinicians should always be aware of potential multiple conditions when considering differential diagnoses.