scholarly journals Atypical Endobronchial Carcinoid with Postobstructive Pneumonia Obscuring the Diagnosis of Granulomatosis with Polyangiitis

2015 ◽  
Vol 2015 ◽  
pp. 1-8
Author(s):  
Robert Ali ◽  
Candice Baldeo ◽  
Jesse Onyenekwe ◽  
Roshan Lala ◽  
Cristian Landa ◽  
...  
Keyword(s):  
Granulomatosis With Polyangiitis ◽  
Systemic Vasculitis ◽  
Clinical Manifestations ◽  
Granulomatous Inflammation ◽  
Lower Limbs ◽  
Antineutrophil Cytoplasmic Antibodies ◽  
Small Vessel Vasculitis ◽  
American College Of Rheumatology ◽  
Abnormal Chest ◽  
Necrotizing Glomerulonephritis

Granulomatosis with polyangiitis (GPA), previously termed Wegener’s Granulomatosis, is an autoimmune small vessel vasculitis which is highly associated with antineutrophil cytoplasmic antibodies (ANCA) and has varied clinical manifestations. Diagnosis hinges on identifying a combination of clinical features of systemic vasculitis, positive ANCA serology, and histological evidence of necrotizing vasculitis, necrotizing glomerulonephritis, or granulomatous inflammation from a relevant organ biopsy. The American College of Rheumatology has also developed a classification criteria focusing specifically on nasal or oral inflammation, abnormal chest radiograph, and abnormal urinary sediment, along with granulomatous inflammation, which helps to distinguish GPA from other forms of systemic vasculitis. In the case presented below, the diagnosis of GPA was delayed as the patient had a concomitant atypical endobronchial carcinoid which predisposed to postobstructive pneumonia. Fortunately, the papular lesions that developed across her lower limbs prompted further investigations. The return of appropriate serology coincided with progression to alveolar hemorrhage, offering a more complete clinical picture, and when she responded to the combination of steroid, cyclophosphamide, and plasma exchange, the diagnosis of GPA was cinched.

scholarly journals Granulomatosis con poliangeitis: manifestaciones clínicas en cabeza y cuello

2020 ◽  
Vol 4 (4) ◽  
Author(s):  
Josué D. Ramírez Esquivel
Keyword(s):  
Head And Neck ◽  
Clinical Manifestations ◽  
Granulomatous Inflammation ◽  
Neck Region ◽  
Antineutrophil Cytoplasmic Antibodies ◽  
Small Vessel Vasculitis ◽  
Histopathological Analysis ◽  
Autoimmune Reaction ◽  
Head And Neck Region ◽  
Systemic Form

La granulomatosis con poliangeitis es una enfermedad poco común, cuya incidencia anual es de 8-10 casos por millón de personas, causada por una reacción autoinmune inusual asociada a la elevación de los anticuerpos anticitoplasma de neutrófilos (ANCA) que ocasionan vasculitis de pequeños vasos e inflamación granulomatosa. Estas reacciones ocasionan síntomas que usualmente comienzan de manera localizada, afectando la cabeza y el cuello, que progresan a una afectación sistémica con pobre pronóstico, sin embargo en ocasiones estos pueden ser pasados por altos y atrasar su diagnóstico. El artículo se  centra en mencionar las manifestaciones clínicas más importantes en la cabeza y el cuello. El diagnóstico es difícil y se basa en las manifestaciones clínicas, asociadas a un análisis histopatológico y positividad de los ANCA. El tratamiento temprano ayuda a mejorar el pronóstico de esta, y se necesita de un equipo multidisciplinario para el diagnóstico y tratamiento de esta enfermedad, para así lograr una remisión y prevenir la mayor cantidad de efectos adversos de los tratamientos.  Granulomatosis with polyangiitis is a rare disease, with an anual incidence of 8-10 cases per million people, it´s caused by an unusual autoimmune reaction associated with antineutrophil cytoplasmic antibodies (ANCA), producing small-vessel vasculitis and granulomatous inflammation. These reactions result in symptoms, usually localized starting in the head and neck region, progressing to a systemic form with a poor prognosis, however, some of these symptoms can be overlooked and delay the diagnosis. This article centers in mentioning the most important clinical manifestations in the head and neck. Diagnosis is difficult, and it’s based on clinical manifestations associated with histopathological analysis and ANCA positivity. Early treatment helps improving the prognosis, and a multi-disciplinary cooperation is needed for the diagnosis and treatment of this disease, to adequately achieve the remission and to prevent most of the adverse effects of the treatments.

scholarly journals ANCA-ASSOCIATED VASCULITIS: HETEROGENEITY OF CLINICAL MANIFESTATIONS, PROGNOSIS, CURRENT OPPORTUNITIES OF PHARMACOTHERAPY

2017 ◽  
Vol 13 (1-2) ◽  
pp. 98-105
Author(s):  
O.B. Yaremenko ◽  
L.B.
Keyword(s):  
Nervous System ◽  
Granulomatosis With Polyangiitis ◽  
Systemic Vasculitis ◽  
Clinical Manifestations ◽  
Skin Lesions ◽  
Antineutrophil Cytoplasmic Antibodies ◽  
Joint Involvement ◽  
Upper Respiratory Tract ◽  
Diagnosis And Prognosis ◽  
Anca Associated Vasculitis

The article highlights the new views on classification and nomenclature of systemic vasculitis, the meaning of detection of antineutrophil cytoplasmic antibodies (ANCA) in the diagnosis and prognosis of the disease. The literature data and own research regarding clinical manifestations of ANCA-associated vasculitis are presented.  Analyzing the first clinical manifestations in 41 patients with granulomatosis with polyangiitis, we identified four variants of the debut: with involvement of ENT organs (n ​=21), with lung lesions without involvement of the upper respiratory tract (n=8), with skin lesions, joint involvement and fever (n=7) and other variants (n=5). Fever (76%), involvement of ENT organs (51%), skin (41%), nervous system (39%) and arthritis/arthralgia (37%) were the most frequent first manifestations of granulomatosis with polyangiitis. In comparison with the presenting features there were more often lesions of the skin (66% vs. 41%), nervous system (51% vs. 39%), kidneys (41% vs. 10%), lungs (63% vs. 30%), eyes (32% vs. 10%) and myalgia (34% vs. 12%) throughout course of disease.  Among the lesions of the ENT organs, sinusitis (n=19), rhinitis (n=8) and otitis (n=6) were predominate, laryngotracheitis, sublottic stenosis, saddle-shaped deformation of the nose, destruction of the walls of the paranasal sinuses and mastoiditis were diagnosed less frequently. The latest clinical recommendations for treating patients with ANCA-associated vasculitis, including the use of immunobiological therapy are presented, as well as describing of the results of our own experience in the using of immunobiological therapy in patients with granulomatosis with polyangiitis.

scholarly journals Cutaneous Ulcers as Initial Presentation of Localized Granulomatosis with Polyangiitis: A Case Report and Review of the Literature

2015 ◽  
Vol 2015 ◽  
pp. 1-7
Author(s):  
Noreen Nasir ◽  
Syed Ahsan Ali ◽  
Hafiz Mohammed Mehmood Riaz
Keyword(s):  
Respiratory Tract ◽  
Granulomatosis With Polyangiitis ◽  
Systemic Vasculitis ◽  
Granulomatous Inflammation ◽  
Organ Damage ◽  
Recurrent Fever ◽  
Small Vessel Vasculitis ◽  
Diagnostic Challenge ◽  
Extensive Evaluation ◽  
Atypical Manifestations

Background. Granulomatosis with polyangiitis (GPA) is an ANCA associated small vessel vasculitis characterized by necrotizing granulomatous inflammation involving the upper and the lower respiratory tract and the kidneys. The disease has a broad clinical spectrum that ranges from limited/localized involvement of a single organ system to a generalized systemic vasculitis that affects several organs with evidence of end organ damage. Atypical forms of the disease have been recognized with or without respiratory tract involvement with a long protracted course before manifesting as generalized disease.Case Presentation. We describe a 57-year-old woman who presented with recurrent fever and cutaneous ulcers on her legs who was diagnosed to have granulomatosis with polyangiitis (GPA) after an extensive evaluation which excluded infectious, other vasculitides, connective tissue disease and malignant etiologies.Conclusion. In the absence of typical manifestations, granulomatosis with polyangiitis (GPA) is indeed a diagnostic challenge to the physician. Atypical manifestations like unexplained recurrent fever and cutaneous ulcers nevertheless call for keeping a low threshold for the diagnosis of GPA as the disease can initially present in localized form before heralding into a generalized disease.

scholarly journals Clinical case of eosinophilic granulomatous polyangiitis debuted by myocardial infarction

2021 ◽  
Vol 40 (2) ◽  
pp. 41-48
Author(s):  
Evgeniy V. Kryukov ◽  
Dmitriy V. Cherkashin ◽  
Sergey L. Grishaev ◽  
Sayora A. Turdialieva ◽  
Elena A. Mozharovskaya ◽  
...  
Keyword(s):  
Myocardial Infarction ◽  
Granulomatosis With Polyangiitis ◽  
Systemic Vasculitis ◽  
Clinical Manifestations ◽  
Antineutrophil Cytoplasmic Antibodies ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Therapy Strategy ◽  
Granulomatous Polyangiitis ◽  
Diagnostic Biopsy ◽  
Eosinophilic Granulomatosis

The article presents clinical observations demonstrating the difficulties of diagnostic search in establishing the diagnosis of systemic vasculitis associated with antineutrophil cytoplasmic antibodies, which include a rare disease eosinophilic granulomatosis with polyangiitis (ChurgStrauss syndrome). Carefully collected anamnesis, participation of specialists of different profiles, retrospective analysis of laboratory and instrumental data allowed to verify the diagnosis, to prescribe adequate therapy. The aim of the publication is to discuss the need for early diagnosis of eosinophilic granulomatosis with polyangiitis, which can improve the effectiveness of therapy and improve the overall prognosis for this disease, taking into account modern approaches based on the main provisions of international recommendations that were prepared in 2015 with the participation of leading experts from Europe, USA and Canada and were called to become the basis for choosing a personalized patient therapy strategy. In some cases, the diagnosis of eosinophilic granulomatous polyangiitis is complicated by the diversity of the clinical picture, the non-simultaneous appearance of the main symptoms and the violation of the stages of the process. In the differential diagnosis of systemic vasculitis, assessment of initial clinical manifestations, testing for the presence of antibodies to the cytoplasm of neutrophils, multispiral computed tomography of the chest organs and diagnostic biopsy of the affected tissues are crucial. In the histological conclusion, a picture of focal ulcerative-necrotic lesions of the nasal mucosa with signs of vasculitis and a pronounced eosinophilic cell component of inflammation was noted. The distinctive features of this case include the onset of the disease with the development of myocardial infarction and early damage to the hearing organ in the form of cochlear neuritis, complicated by sensorineural hypoacusis. The combination of high doses of glucocorticosteroids and cyclophosphamide is still the gold standard for the treatment of severe cases, but the use of biological agents such as rituximab or mepolizumab seems to be a promising therapeutic alternative (4 figs, bibliography: 3 refs).

Systemic Vasculitis Syndromes

2017 ◽  
Author(s):  
Alexandra Villa-Forte ◽  
Brian F Mandell
Keyword(s):  
Kawasaki Disease ◽  
Blood Vessels ◽  
Granulomatosis With Polyangiitis ◽  
Systemic Vasculitis ◽  
Plain Radiograph ◽  
Small Vessel ◽  
Small Vessel Vasculitis ◽  
Saddle Nose ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Saddle Nose Deformity

Vasculitis is defined by histologic evidence of inflammation that involves the blood vessels. The diagnosis of a specific primary vasculitic disorder depends on the pattern of organ involvement, the histopathology, the size of affected blood vessels, and the exclusion of diseases that can cause “secondary” vasculitis. This review presents an approach to the patient suspected of having vasculitis, and goes on to discuss small vessel vasculitis, granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis, microscopic polyangiitis, polyarteritis nodosa, Kawasaki disease, large vessel arteritis, and Behçet disease. Figures show classification of the systemic vasculitis syndromes, the relationships among the causes of small vessel (“hypersensitivity”) vasculitis, palpable purpura of the distal extremities, saddle nose deformity, the nodular infiltrates of the lung in granulomatosis with polyangiitis shown on plain radiograph as well as computed tomography, necrotizing scleritis, livedo reticularis, and angiograms of a patient with Takayasu arteritis. Tables list selected laboratory tests for patients with multisystem disease and possible vasculitis, practical comments on immunosuppressive therapies for vasculitis, features of vasculitis, diagnostic criteria for Kawasaki disease, and giant cell arteritis. This review contains 8 highly rendered figures, 5 tables, and 59 references.

scholarly journals Severe course of quickly progressing glomerulonephritis in a patient with ANTSA-associated vasculitis: review and case report

2020 ◽  
Vol 7 (3) ◽  
pp. 55-62
Author(s):  
Iu. V. Lavrishcheva ◽  
Y. S. Kaledinova ◽  
A. A. Yakovenko ◽  
I. A. Artemev
Keyword(s):  
Granulomatosis With Polyangiitis ◽  
Rapidly Progressive Glomerulonephritis ◽  
Granulomatous Inflammation ◽  
Clinical Work ◽  
Chronic Hemodialysis ◽  
Complete Loss ◽  
Vascular Lesions ◽  
Upper Respiratory Tract ◽  
Delay In Diagnosis ◽  
Necrotizing Glomerulonephritis

Granulomatosis with polyangiitis is characterized by necrotizing granulomatous inflammation, vasculitis with vascular lesions of small and medium caliber and focal necrotizing glomerulonephritis. A frequent and one of the most formidable complications is kidney damage, which in a large number of cases leads to a complete loss of organ function and a switch to renal replacement therapy. Given the rare occurrence of this disease in the clinical work of practitioners, and their low awareness of this pathology, problems often arise with the diagnosis and treatment of patients with HPA. Due to the diversity and non-specific nature of the manifestations of the disease, a delay in diagnosis may occur. The presented case illustrates the manifestations of granulomatosis with polyangiitis in the form of severe damage to the upper respiratory tract and kidneys, the diagnosis of which was difficult due to the rarity of the disease and the multiple organ pathology. This article presents a clinical case of severe progression of rapidly progressive glomerulonephritis in a patient with ANCA-associated vasculitis, a brief review of the literature is given. Despite adequate therapy, the disease progressed mainly due to deterioration of renal function, which subsequently led to a complete loss of kidney function and the transition to treatment with chronic hemodialysis.

scholarly journals Eosinophilic granulomatosis with polyangiitis in a Nigerian woman

2019 ◽  
Vol 12 (6) ◽  
pp. e228901
Author(s):  
Ngozi Lina Ekeigwe ◽  
Olufemi Adelowo ◽  
Ehiaghe Lonia Anaba ◽  
Hakeem Olaosebikan
Keyword(s):  
Granulomatosis With Polyangiitis ◽  
Rare Condition ◽  
Antineutrophil Cytoplasmic Antibodies ◽  
Systemic Autoimmune Disease ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
American College Of Rheumatology ◽  
Nigerian Woman ◽  
History Of ◽  
Perinuclear Anca ◽  
Eosinophilic Granulomatosis

Eosinophilic granulomatosis with polyangiitis (EGPA), previously called Churg-Strauss Syndrome, is a systemic autoimmune disease that is usually associated with asthma and eosinophilia. It is a rare condition associated with antineutrophil cytoplasmic antibodies (ANCA). We report a case of a 52-year-old Nigerian woman who presented with bilateral leg swelling with multiple ulcers, background history of allergic rhinitis and chronic sinusitis since adolescence. There were also associated symptoms of peripheral neuropathy, weight loss, peripheral blood eosinophilia and positive perinuclear ANCA and she was previously managed for rheumatoid arthritis and asthma. She fulfilled the American College of Rheumatology criteria for EGPA and was treated with glucocorticoids and methotrexate which led to significant improvement of her symptoms.

Comparison of patient self-reported data to physician-reported data in eosinophilic granulomatosis with polyangiitis (Preprint)

2021 ◽  
Author(s):  
Irena Doubelt ◽  
Jason M. Springer ◽  
Tanaz A. Kermani ◽  
Antoine G. Sreih ◽  
Cristina Burroughs ◽  
...  
Keyword(s):  
Granulomatosis With Polyangiitis ◽  
Clinical Manifestations ◽  
The United States ◽  
Research Network ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
American College Of Rheumatology ◽  
Organ Systems ◽  
Patient Reported ◽  
Reported Data ◽  
Eosinophilic Granulomatosis

BACKGROUND Patient-based registries can help advance research in rare diseases such as eosinophilic granulomatosis with polyangiitis (EGPA), a complex, multi-organ form of anti-cytoplasm neutrophil antibody (ANCA)-associated vasculitis. OBJECTIVE To compare patient-reported vs. physician-reported manifestations, treatments, and outcomes for patients with EGPA. METHODS Comparative analysis of patients ≥18 years with EGPA in Canada or the United States from two separate cohorts: i) The Vasculitis Patient-Powered Research Network (VPPRN), a self-enrolled, secure portal with patient-entered data updated quarterly (2013-2019), vs. ii) The Vasculitis Clinical Research Consortium (VCRC) observational studies, a physician-entered database (2003-2019) of patients who fulfilled the 1990 American College of Rheumatology classification criteria for EGPA. Studied parameters included demographics, clinical manifestations, ANCA status, treatments, and relapses. RESULTS Data from 195 patients with a validated diagnosis of EGPA in the VPPRN and 354 patients enrolled in the VCRC were analyzed. Compared to the VCRC cohort, the patients in the VPPRN cohort were more commonly female (69.2% vs. 59.0% in the VCRC cohort; P =.02), younger at diagnosis (47.3 vs. 50.0 years; P =.03), reported similar frequencies of asthma (96.2% vs 92.9% in VCRC; P =.13), cardiac manifestations (28.8% vs 21.2%; P =.06), but less frequent lung manifestations other than asthma, and more frequent disease manifestations in all other organ systems. ANCA positivity was 48.9% in the VPPRN patients vs. 38.9% (P=.05) in the VCRC cohort. Relapsing disease after study enrollment was reported in 32.3% patients in the VPPRN compared 35.7% of patients in the VCRC. Most therapies (glucocorticoids, cyclophosphamide, mepolizumab) were used at similar frequencies in both groups, except for rituximab with VPPRN patients reporting more use than VCRC cohort (24.1% vs. 10.5%; P =<.001). CONCLUSIONS Patients with EGPA generally report having more manifestations of disease than physicians report for patients with EGPA. These differences imply the need to reconsider how patient- and physician-reported data are collected for the study of EGPA, and reevaluate disease specific definitions. CLINICALTRIAL ClinicalTrials.gov: (1) VCRC Longitudinal Study (LS) NCT00315380 https://clinicaltrials.gov/ct2/show/NCT00315380 and (2) One-Time DNA (OT) study NCT01241305 https://clinicaltrials.gov/ct2/show/NCT01241305

scholarly journals Significance of Small Renal Artery Lesions in Patients with Antineutrophil Cytoplasmic Antibody-associated Glomerulonephritis

2014 ◽  
Vol 41 (6) ◽  
pp. 1140-1146 ◽  
Author(s):  
Akiko Endo ◽  
Junichi Hoshino ◽  
Tatsuya Suwabe ◽  
Keiichi Sumida ◽  
Koki Mise ◽  
...  
Keyword(s):  
Granulomatosis With Polyangiitis ◽  
Systemic Vasculitis ◽  
Rapidly Progressive Glomerulonephritis ◽  
Antineutrophil Cytoplasmic Antibody ◽  
Pulmonary Involvement ◽  
Renal Arteries ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Necrotizing Glomerulonephritis ◽  
Group A ◽  
Group B

Objective.Antineutrophil cytoplasmic antibody (ANCA)-associated glomerulonephritis is a vasculitis affecting the glomerular capillaries and small renal arteries. Although crescent formation has been reported to be characteristic of this condition, the significance of coexisting vasculitis affecting the small renal arteries has not been investigated.Methods.Fifty patients with ANCA-positive rapidly progressive glomerulonephritis whose renal biopsy specimens contained arterioles and/or interlobular arteries were retrospectively evaluated. Cellular crescents and/or necrotizing glomerulonephritis were noted in all 50 patients. Ten patients had vasculitis of the small renal arteries (group A) and 40 patients were without such vasculitis (group B). The clinical features of these 2 groups were compared.Results.Group A comprised 4 patients who had granulomatosis with polyangiitis (GPA) and 6 with microscopic polyangiitis (MPA), while group B included 1 patient with GPA and 39 with MPA. No patient in either group had eosinophilic granulomatosis with polyangiitis. The C-reactive protein (CRP) level was significantly higher in group A compared with group B (11.58 ± 6.19 vs 2.7 ± 3.55 mg/dl, p < 0.05), and pulmonary involvement was more frequent in group A than group B (80% vs 37.5%, p < 0.05).Conclusion.In patients with ANCA-positive glomerulonephritis, vasculitis of small renal arteries may be associated with systemic vasculitis (including pulmonary involvement) because of elevated CRP, a systemic inflammatory marker related to overproduction of interleukin 6.

scholarly journals Tension Pneumothorax as Initial Manifestation of Granulomatosis with Polyangiitis (GPA)

2021 ◽  
Author(s):  
Rúben Reis ◽  
Anneke Joosten ◽  
Francelino Ferreira ◽  
Magda Silva ◽  
Catarina Parente ◽  
...  
Keyword(s):  
Granulomatosis With Polyangiitis ◽  
Kidney Biopsy ◽  
Systemic Vasculitis ◽  
Systemic Disease ◽  
Tension Pneumothorax ◽  
Upper Airway ◽  
Antineutrophil Cytoplasmic Antibodies ◽  
Lower Airway ◽  
Initial Manifestation ◽  
Pulmonary Cavity

Granulomatosis with polyangiitis (Wegener’s granulomatosis) is a systemic vasculitis that primarily affects small and medium vessels. Its manifestations are usually confined to the upper airway, lower airway and kidney. It can also affect other organs and systems, although this is unusual. We describe the case of a 67-year-old woman who presented with a tension pneumothorax due to rupture of a pulmonary cavity. This pulmonary cavity proved to be secondary to systemic disease which also caused a tumour in her kidney. Biopsy showed non-necrotizing granulomatosis, and even though antineutrophil cytoplasmic antibodies (ANCA) were negative, the diagnosis of granulomatosis with polyangiitis was made.

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