Conjunctivitis, episcleritis and anterior uveitis as the first presenting features of granulomatosis with polyangiitis

2021 ◽  
Vol 14 (10) ◽  
pp. e243558
Author(s):  
Lucas Donato Foster ◽  
Michael Nyugen ◽  
Edward Margolin
Keyword(s):  
Anterior Uveitis ◽  
Crescentic Glomerulonephritis ◽  
Granulomatosis With Polyangiitis ◽  
Oral Prednisone ◽  
Antineutrophil Cytoplasmic Antibody ◽  
Final Diagnosis ◽  
Ocular Inflammation ◽  
Reactive Protein ◽  
Ocular Manifestations ◽  
Organ Systems

Granulomatosis with polyangiitis (GPA) is a rare disorder characterised by inflammation of small-sized and medium-sized blood vessels that result in damage to various organ systems, but it most commonly affects the respiratory tract and kidneys. It is one of the few entities that can present with ocular inflammation as well as renal impairment at the same time. We describe a case of a 38-year-old man with conjunctivitis, episcleritis, anterior uveitis as a first manifestation of GPA. His presentation with red eye and anterior uveitis prompted further workup, which revealed acute renal failure (creatinine 442 mmol/L), elevated inflammatory markers (erythrocyte sedimentation rate of 85 mmol/hour and C reactive protein of 72 mg/L), and a c-antineutrophil cytoplasmic antibody titre >8. An urgent renal biopsy was performed demonstrating necrotising crescentic glomerulonephritis, which led to the final diagnosis of GPA. Treatment induction with intravenous methylprednisolone and plasmapheresis followed by an oral prednisone taper and intravenous rituximab infusions leading to resolution of all symptoms and normalisation of kidney function. This report highlights conditions that can present with both ocular inflammation and renal dysfunction with a focus on GPA and its ocular manifestations.

scholarly journals Amnesia in a Patient with Rheumatoid Arthritis: A Case of Granulomatosis with Polyangiitis

2020 ◽  
Vol 2020 ◽  
pp. 1-5
Author(s):  
Pablo Weilg ◽  
Giancarlo Diaz-Zamora ◽  
Larry Young
Keyword(s):  
Rheumatoid Arthritis ◽  
Crescentic Glomerulonephritis ◽  
Granulomatosis With Polyangiitis ◽  
Diffuse Alveolar Damage ◽  
Oral Prednisone ◽  
Small Region ◽  
Pulse Therapy ◽  
Steroid Pulse ◽  
Acute Infarction ◽  
History Of

A 56-year-old male with a history of seropositive rheumatoid arthritis complained of polyarthritis and forgetfulness. The initial CT scan of the head without contrast was negative for acute pathologies. However, as he continued to decline despite being on oral prednisone, an MRI of the head was ordered and revealed a subtle small region of acute infarction in the left mammillary body. He was diagnosed with granulomatosis with polyangiitis in light of his positive C-ANCA antibodies, PR3 antibody, and a kidney biopsy, which showed focal necrotizing pauci-immune crescentic glomerulonephritis. Despite undergoing steroid pulse therapy, the patient developed diffuse alveolar damage which finally responded to rituximab infusion.

scholarly journals Granulomatosis with Polyangiitis Restricted to the Back Muscle: The First Case Report

Medicina ◽  
2020 ◽  
Vol 56 (9) ◽  
pp. 431
Author(s):  
Yu-Mi Lee ◽  
Sang Wan Chung
Keyword(s):  
Granulomatosis With Polyangiitis ◽  
Antineutrophil Cytoplasmic Antibody ◽  
Final Diagnosis ◽  
High Dose ◽  
Back Muscle ◽  
Anca Associated Vasculitis ◽  
First Case ◽  
Psoas Muscles ◽  
High Dose Methylprednisolone ◽  
Single Organ

Granulomatosis with polyangiitis (GPA) is an autoimmune disease which is a type of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis that frequently affects the lungs and kidneys. However, GPA limited to a single organ has also been reported. A 71-year-old man was admitted for back pain and fever. We detected elevated levels of inflammatory markers and myeloperoxidase-ANCA. Magnetic resonance imaging indicated diffuse inflammation of the back and psoas muscles. Histology showed degenerated muscle fibers and granulomatosis vasculitis with mixed lymphoplasma cell infiltration. High-dose methylprednisolone therapy improved his symptoms. A final diagnosis of GPA limited to the muscles was made.

scholarly journals Biomarkers Predict Relapse in Granulomatosis with Polyangiitis

2014 ◽  
Vol 2014 ◽  
pp. 1-4 ◽  
Author(s):  
Patrick C. P. Hogan ◽  
Robert M. O’Connell ◽  
Simone Scollard ◽  
Emmett Browne ◽  
Emer E. Hackett ◽  
...  
Keyword(s):  
Neutrophil Count ◽  
Granulomatosis With Polyangiitis ◽  
Antineutrophil Cytoplasmic Antibody ◽  
Organ Damage ◽  
Proteinase 3 ◽  
Disease Relapse ◽  
C Reactive Protein ◽  
Small Blood Vessel ◽  
Reactive Protein ◽  
Specific Reactivity

Granulomatosis with polyangiitis (GPA) is a small blood vessel vasculitic disorder with a high mortality rate if undiagnosed or treated inadequately. Disease relapse is a key feature of this disease and early identification of relapse episodes is very important in limiting end-organ damage. The advent of indirect immunofluorescence to detect antineutrophil cytoplasmic antibody (ANCA) with specific reactivity against the enzyme proteinase-3 (PR3) has been very useful in the diagnosis of GPA but is less helpful in predicting relapse. Indeed, up to date no satisfactory biomarker has been identified that can reliably predict relapse. This study assessed the probability of the occurrence of a relapse when a change was noted in a range of commonly used laboratory tests. These tests included levels of C-reactive protein (CRP), anti-PR3 antibodies, ANCA titre, and the neutrophil count. A group of 30 GPA patients with a total of 66 relapse episodes was investigated and a novel clinical yield score was devised. When a combined rise in CRP, anti-PR3 antibodies, and neutrophil count was observed in the 6-month period before a relapse event, 59% of patient relapses could be predicted. Monitoring changes in this set of parameters helps identify disease relapse.

scholarly journals Ophthalmic Manifestations of ANCA-Associated Vasculitis

Acta Medica ◽  
2021 ◽  
pp. 1-7
Author(s):  
Merve İnanç Tekin ◽  
Müge Pınar Çakar Özdal
Keyword(s):  
Granulomatosis With Polyangiitis ◽  
Systemic Vasculitis ◽  
Systemic Disease ◽  
Retinal Vasculitis ◽  
Antineutrophil Cytoplasmic Antibody ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Ocular Involvement ◽  
High Morbidity ◽  
Ocular Manifestations ◽  
Ocular Findings

Ocular manifestations in antineutrophil cytoplasmic antibody-associated vasculitis can be associated with the general or limited form of disease and can even occur in the absence of systemic disease. Ocular manifestations of associated vasculitis can be the first symptom of previously not manifested or undiagnosed systemic disease, allowing ophthalmologists to contribute to the diagnosis. Although its ocular findings are variable and nonspecific, the presence of necrotizing changes and peripheral corneal involvement accompanying scleral inflammation are important clues suggesting systemic vasculitis, especially associated vasculitis. The disease may affect all layers of the eye; scleritis and orbital involvement being the most common. Conjunctivitis, episcleritis, peripheral ulcerative keratitis, uveitis and retinal vasculitis are other ocular findings that may be observed during the disease course. Ocular involvement is most commonly seen in granulomatosis with polyangiitis followed by eosinophilic granulomatosis with polyangiitis and microscopic polyangiitis. Due to the high morbidity and mortality of associated vasculitis, it is very important to recognize the ocular manifestations of vasculitis as a sign of the underlying systemic disease and an indicator for the disease activity. Treatment varies depending on the location and severity of the ocular involvement. Although localized medical and surgical treatments can help to manage associated ocular disease, systemic immunosuppressive medications are often required to control the underlying disease. With the increasing availability and use of biological agents, prognosis has improved in patients with severe ocular complications. Rituximab appears to be useful in inducing remission and controlling relapses in patients with ocular involvement of associated vasculitis, particularly in cyclophosphamide resistant cases. A multidisciplinary approach in the diagnosis, treatment and follow-up of patients with associated vasculitis is required in order to achieve successful results.

scholarly journals Corneal melt secondary to eosinophilic granulomatosis with polyangiitis

2019 ◽  
Vol 12 (6) ◽  
pp. e229859
Author(s):  
Evelyn Fennelly ◽  
Emily Greenan ◽  
Conor C Murphy
Keyword(s):  
Granulomatosis With Polyangiitis ◽  
Antineutrophil Cytoplasmic Antibody ◽  
Organ System ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Ocular Manifestations ◽  
First Case ◽  
Clinical Presentations ◽  
Central Nervous Systems ◽  
Eosinophilic Granulomatosis ◽  
Peripheral Blood Eosinophilia

Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare antineutrophil cytoplasmic antibody-associated vasculitis that can affect any organ system. It is most often characterised by chronic airway inflammation along with prominent peripheral blood eosinophilia, although the disease can affect the cardiovascular, gastrointestinal, renal or central nervous systems. Ocular manifestations are uncommon and when they do occur, are varied in their clinical presentations. To the best of our knowledge, this is the first case of corneal melt secondary to EGPA to have been reported.

scholarly journals Early recognition of PIMS-TS: a single centre retrospective review

2021 ◽  
Vol 5 (1) ◽  
pp. e001011
Author(s):  
Roshni Mistry ◽  
Nicola Scanlon ◽  
James Hibberd ◽  
Fionnghuala Fuller
Keyword(s):  
Early Recognition ◽  
Febrile Illness ◽  
Final Diagnosis ◽  
University Hospital ◽  
Single Centre ◽  
Retrospective Case ◽  
Reactive Protein ◽  
Review Reports ◽  
Specific Symptoms

IntroductionResearch into paediatric inflammatory multisystem syndrome temporally associated with SARS-CoV-2 (PIMS-TS) has focused on tertiary level management. This review reports on symptoms and investigations at presentation.MethodsSingle centre retrospective case note analysis of patients fulfilling PIMS-TS diagnostic criteria from March to May 2020 in a London district level university hospital.ResultsSix patients presented in the week prior to their final diagnosis with fever and non-specific symptoms. Raised C-reactive protein (CRP), lymphopenia and hyponatraemia were noted. Kawasaki-like symptoms were under-represented in all patients.InterpretationThe results suggest that a proportion of children with early PIMS-TS present with a non-specific febrile illness and abnormal blood results. Further research is needed to determine the most appropriate identification and follow-up of these children.

scholarly journals ANCA Status or Clinical Phenotype — What Counts More?

2021 ◽  
Vol 23 (6) ◽  
Author(s):  
Martin Windpessl ◽  
Erica L. Bettac ◽  
Philipp Gauckler ◽  
Jae Il Shin ◽  
Duvuru Geetha ◽  
...  
Keyword(s):  
Clinical Trials ◽  
Granulomatosis With Polyangiitis ◽  
Clinical Phenotype ◽  
Antineutrophil Cytoplasmic Antibody ◽  
Cardiovascular Death ◽  
Genetic Associations ◽  
Ongoing Debate ◽  
The Past ◽  
Anca Associated Vasculitis

Abstract Purpose of Review There is ongoing debate concerning the classification of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis. That is, whether classification should be based on the serotype (proteinase 3 (PR3)- or myeloperoxidase (MPO)-ANCA) or on the clinical phenotype (granulomatosis with polyangiitis (GPA) or microscopic polyangiitis (MPA)). To add clarity, this review focused on integration of the most recent literature. Recent Findings Large clinical trials have provided evidence that a serology-based risk assessment for relapses is more predictive than distinction based on the phenotype. Research conducted in the past decade indicated that a serology-based approach more closely resembles the genetic associations, the clinical presentation (i.e., lung involvement), biomarker biology, treatment response, and is also predicting comorbidities (such as cardiovascular death). Summary Our review highlights that a serology-based approach could replace a phenotype-based approach to classify ANCA-associated vasculitides. In future, clinical trials and observational studies will presumably focus on this distinction and, as such, translate into a “personalized medicine.”

scholarly journals POS0830 FACTORS INFLUENCING PATIENT-REPORTED OUTCOMES IN ANCA-ASSOCIATED VASCULITIS

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 668.2-669
Author(s):  
S. Monti ◽  
P. Delvino ◽  
C. Klersy ◽  
G. Coppa ◽  
A. Milanesi ◽  
...  
Keyword(s):  
Disease Activity ◽  
Global Assessment ◽  
Patient Reported Outcomes ◽  
Granulomatosis With Polyangiitis ◽  
Disease Duration ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Anca Associated Vasculitis ◽  
Organ Systems ◽  
Damage Accrual ◽  
Patient Reported

Background:Patient-reported outcomes (PROs) are currently poorly integrated in the clinical evaluation of disease activity in patients with ANCA-associated vasculitis (AAV).Objectives:To assess the distribution of the Patient Global Assessment (PtGA) in patients with AAV in stable remission, and to identify correlates of PtGA; to assess the discordance between PtGA score and PhGA.Methods:Patients with a diagnosis of AAV [eosinophilic granulomatosis with polyangiitis, granulomatosis with polyangiitis, microscopic polyangiitis] in stable, complete remission (defined by a BVAS=0) and with a Physician Global Assessment (PhGA)=0 were included. A questionnaire including several aspects of disease captured by PROs was collected. PtGA on a 0-100 mm visual analogue scale (VAS) was assessed, with higher scores representing higher/worse levels of disease activity. Similarly, VAS for pain, chronic damage according to the patient’s opinion, general health (GH), fatigue, and sleep quality were collected. The worst symptom in the patient’s opinion affecting the overall assessment of disease activity was recorded. The Cragg Hurdle model was used to assess the predictors of PtGA.Results:65 patients were included, female 57%, mean age 61±12 years. Mean disease duration at enrollment was 8±6 years. Mean vasculitis damage index (VDI) was 4.4 ±2.3, with 45% of patients having a VDI ≥ 5. Despite having been classified as being in remission, PtGA was elevated in 37% of patients. We explored several correlates of PtGA. Higher degree of damage accrual (VDI) did not influence the patient’s evaluation of current disease activity. Similarly, we did not identify a correlation between older age, educational level, number of organ-systems involved, number of comorbidities, the number of previous major or minor relapses, higher disease duration, nor the type of AAV diagnosis (figure 1, panel A). Only sex significantly correlated with PtGA scores: 19 (51%) of female patients reported an elevated PtGA compared to only 5 (18%) of male (p=0.009). PtGA resulted to be significantly correlated with other (mostly modifiable) PROs including VAS pain, perception of the level of chronic damage accrual, GH, and fatigue (figure 1, panel B). The agreement between patients’ and physicians’ assessments of disease activity was 63%. Patients reported pain, followed by chronic respiratory symptoms to be the worst-experienced ongoing manifestations affecting their evaluation of disease activity.Conclusion:A significant proportion of patients with AAV considered to be in remission by the physician still declares to have persistent aspects of uncontrolled disease. PtGA is significantly influenced by persistent pain and fatigue, which warrant better assessment in the future.Disclosure of Interests:None declared

Morbidity and mortality associated with subglottic laryngotracheal stenosis in granulomatosis with polyangiitis (Wegener's granulomatosis): a single-centre experience in the United Kingdom

2014 ◽  
Vol 128 (9) ◽  
pp. 831-837 ◽  
Author(s):  
N P Jordan ◽  
H Verma ◽  
A Siddiqui ◽  
G A Morrison ◽  
D P D'Cruz
Keyword(s):  
United Kingdom ◽  
Wegener’S Granulomatosis ◽  
Granulomatosis With Polyangiitis ◽  
Systemic Vasculitis ◽  
Antineutrophil Cytoplasmic Antibody ◽  
Wegener's Granulomatosis ◽  
Laryngotracheal Stenosis ◽  
Surgical Interventions ◽  
The United Kingdom ◽  
Single Centre Experience

AbstractObjectives:We aimed to determine the prevalence of symptomatic subglottic laryngotracheal stenosis in patients with granulomatosis with polyangiitis (Wegener's granulomatosis); we also wanted to characterise the clinical outcomes and surgical interventions required, and the relapse rate in our cohort.Methods:We undertook a retrospective clinical review of all granulomatosis with polyangiitis patients with symptomatic subglottic laryngotracheal stenosis attending St Thomas' Hospital, London, United Kingdom.Results:Symptomatic subglottic laryngotracheal stenosis developed in 16 per cent of granulomatosis with polyangiitis patients attending our clinic. The median age of patients at diagnosis was 44 years (range: 34–81 years); 78 per cent of those presenting with subglottic laryngotracheal stenosis were women and 22 per cent were men. All patients were white; 67 per cent of patients were proteinase 3-antineutrophil cytoplasmic antibody-positive and 67 per cent developed relapsing disease requiring repeated surgical intervention. Subglottic laryngotracheal stenosis relapse was not associated with active systemic vasculitis elsewhere.Conclusion:Subglottic laryngotracheal stenosis is an uncommon but significant complication of granulomatosis with polyangiitis. Management of subglottic laryngotracheal stenosis requires a multi-disciplinary approach, with both rheumatological and otolaryngological expertise involved, given the relapsing nature of the disease.

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