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 Granulomatous Inflammation in ANCA-Associated Vasculitis

2021 ◽  
Vol 22 (12) ◽  
pp. 6474
Author(s):  
Antje Müller ◽  
Bettina Krause ◽  
Anja Kerstein-Stähle ◽  
Sara Comdühr ◽  
Sebastian Klapa ◽  
...  
Keyword(s):  
Respiratory Tract ◽  
Granulomatosis With Polyangiitis ◽  
Local Development ◽  
Systemic Vasculitis ◽  
Kidney Tissue ◽  
Granulomatous Inflammation ◽  
Giant Cells ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Factors Affecting ◽  
Anca Associated Vasculitis

ANCA-associated vasculitis (AAV) comprises granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA). While systemic vasculitis is a hallmark of all AAV, GPA is characterized by extravascular granulomatous inflammation, preferentially affecting the respiratory tract. The mechanisms underlying the emergence of neutrophilic microabscesses; the appearance of multinucleated giant cells; and subsequent granuloma formation, finally leading to scarred or destroyed tissue in GPA, are still incompletely understood. This review summarizes findings describing the presence and function of molecules and cells contributing to granulomatous inflammation in the respiratory tract and to renal inflammation observed in GPA. In addition, factors affecting or promoting the development of granulomatous inflammation such as microbial infections, the nasal microbiome, and the release of damage-associated molecular patterns (DAMP) are discussed. Further, on the basis of numerous results, we argue that, in situ, various ways of exposure linked with a high number of infiltrating proteinase 3 (PR3)- and myeloperoxidase (MPO)-expressing leukocytes lower the threshold for the presentation of an altered PR3 and possibly also of MPO, provoking the local development of ANCA autoimmune responses, aided by the formation of ectopic lymphoid structures. Although extravascular granulomatous inflammation is unique to GPA, similar molecular and cellular patterns can be found in both the respiratory tract and kidney tissue of GPA and MPA patients; for example, the antimicrobial peptide LL37, CD163+ macrophages, or regulatory T cells. Therefore, we postulate that granulomatous inflammation in GPA or PR3-AAV is intertwined with autoimmune and destructive mechanisms also seen at other sites.

Isolated deafness as a presenting symptom in granulomatosis with polyangiitis

2021 ◽  
Vol 14 (3) ◽  
pp. e241159
Author(s):  
Amy Kousha ◽  
Michael Reed ◽  
Sara Else
Keyword(s):  
Respiratory Tract ◽  
Granulomatosis With Polyangiitis ◽  
Organ Damage ◽  
Sudden Onset ◽  
Small Vessel Vasculitis ◽  
Upper Respiratory Tract ◽  
Diagnostic Uncertainty ◽  
Tract Involvement ◽  
Upper Respiratory ◽  
Bilateral Deafness

Granulomatosis with polyangiitis (GPA) is a rare small vessel vasculitis commonly affecting the lungs, upper respiratory tract and kidneys. It is an idiopathic condition but likely due to an autoimmune process, resulting in granulomatous lesions and glomerulonephritis. Upper respiratory tract involvement is commonly seen in patients with GPA. Our case is that of an elderly lady (Mrs C) presenting with sudden onset bilateral deafness. She was later found to have extensive lower respiratory tract involvement although she was never particularly symptomatic of this. The presentation suggested a single organ disorder and led to some initial diagnostic uncertainty. Imaging and laboratory investigations eventually led to the diagnosis and she was successfully treated with corticosteroids and rituximab with good response and hearing improvement. This case highlights the importance of early diagnosis in a rapidly progressive disease which undetected can lead to catastrophic end organ damage and disability.

scholarly journals Dermatologic manifestations of granulomatosis with polyangiitis: A case report and literature review

2019 ◽  
Vol 6 (4) ◽  
pp. 19
Author(s):  
Ashley Thomas ◽  
Nithya Krishnan ◽  
Nicole Vesely ◽  
Myint Thway ◽  
Rafik Jacob
Keyword(s):  
Granulomatosis With Polyangiitis ◽  
Systemic Vasculitis ◽  
Clinical Signs ◽  
Granulomatous Inflammation ◽  
Signs And Symptoms ◽  
Organ Damage ◽  
Skin Lesions ◽  
African American Female ◽  
Cutaneous Manifestations ◽  
Serologic Testing

Granulomatosis with polyangiitis (GPA) is an ANCA-associated, systemic vasculitis of small and medium-sized blood vessels. GPA causes inflammation and destruction to the vessel wall and eventual tissue and organ damage. It classically affects the tissues and vasculature of the sinuses, lungs, and kidneys. The organ damage results in epistaxis, cough, hemoptysis, shortness of breath and/or even kidney failure. Cutaneous manifestations are rare, but have been reported. We discuss the case of a 27-year-old African American female who presented with skin lesions on her bilateral forearms and hands, several years after diagnosis and treatment for GPA. The lesions were isolated papules, located on bilateral elbows, palms, and the lateral edge of the 3rd digit on her left hand. The lesions were intensely pruritic and non-painful. A skin biopsy of the lesions showed neutrophilic and granulomatous inflammation with nuclear debris, a largely non-specific histopathologic finding. An extensive autoimmune work up revealed elevated c-ANCA/PR3-ANCA levels supporting a diagnosis of cutaneous GPA. GPA is diagnosed by a combination of clinical signs and symptoms, serologic testing, and histology from biopsy of affected organs. Patient’s who meet the criteria should be tested for anti-neutrophil cytoplasmic antibody (ANCA), specifically c-ANCA/PR3-ANCA. A positive ANCA is supportive for GPA diagnosis. However, a negative ANCA does not rule out disease. Patients with cutaneous findings suggestive of GPA and positive c-ANCA/PR3-ANCA serologic testing should be closely followed up, which will lead to overall better prognosis, improved health outcomes and reduced patient and health care expenses.

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.

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.

Clinical features of ANCA-associated vasculitis

2013 ◽  
pp. 1090-1102
Author(s):  
Wolfgang L. Gross ◽  
Julia U. Holle
Keyword(s):  
Respiratory Tract ◽  
Clinical Features ◽  
Lower Respiratory Tract ◽  
Granulomatosis With Polyangiitis ◽  
Systemic Vasculitis ◽  
Systemic Disease ◽  
Clinical Signs ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Granulomatous Lesions ◽  
Organ Manifestations

The primary ANCA-associated vasculitides are granulomatosis with polyangiitis (Wegener’s, GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA, Churg-Strauss syndrome, CSS). They predominantly affect small (and medium-sized) vessels and share a variable association with ANCA (anti-neutrophil cytoplasm antibody) directed against neutrophil proteinase 3 (PR3, mainly in GPA) and myeloperoxidase (MPO, mainly in MPA and CSS). Crescentic necrotizing glomerulonephritis and alveolar haemorrhage due to pulmonary capillaritis represent classical (vasculitic) organ manifestations of the ANCA-associated vasculitides (AAV). MPA occurs as a ’pure’ small (to medium-size) vessel vasculitis, whereas GPA and CSS are characterized by additional distinct clinical and pathological features. In GPA, granulomatous lesions of the upper and/or lower respiratory tract are a hallmark of the disease. Granulomatous lesions may be large in appearance and occur as space-consuming, infiltrating, and destructive inflammatory masses. GPA is believed to follow a stagewise course with an initial localized form, restricted granulomatous lesions of the upper and/or lower respiratory tract without clinical signs of vasculitis, and a consecutive generalization to systemic vasculitis which may be either non-organ-threatening (early systemic) or organ- and life- threatening (generalized GPA). Rarely, patients arrest in the localized stage and do not progress to systemic disease. In EGPA asthma, hypereosinophilia and eosinophilic organ infiltration (e.g. eosinophilic myocarditis) are typical features of the disease apart from vasculitis. Similarly to GPA, EGPA follows a stagewise course: asthma and eosinophilia may precede full-blown disease for several months or years. Recent cohort studies suggest different phenotypes in EGPA (predominantly vasculitic and MPO-ANCA-positive and predominantly with eosinophilic organ infiltration, usually ANCA-negative). This chapter focuses on the clinical features of the primary AAV and their outcome.

scholarly journals Difficulties in the differential diagnosis of granulomatosis with polyangiitis and scarring pemphigoid

2020 ◽  
Vol 10 (5) ◽  
pp. 398-402
Author(s):  
N. A. Magdeeva ◽  
A. A. Kobriseva ◽  
M. A. Reznikova ◽  
I. F. Melehina
Keyword(s):  
Differential Diagnosis ◽  
Renal Damage ◽  
Clinical Case ◽  
Granulomatosis With Polyangiitis ◽  
Systemic Vasculitis ◽  
Organ Damage ◽  
Pathological Process ◽  
Poor Knowledge ◽  
Eye Damage ◽  
Positive Results

The article presents a clinical case of a rare pathology. The patient for several years visited various specialists. In connection with a similar clinical picture, multi-organ damage, differential diagnosis was performed between systemic vasculitis (granulomatosis with polyangiitis) and scarring pemphigoid.The mucous membrane of the mouth and nose is involved in the pathological process with granulomatosis with polyangiitis in the form of ulcerative defects, which subsequently lead to deformation of the nose. The danger with this systemic vasculitis is renal damage with the development of nephritis, renal failure. With scarring pemphigoid, eye damage is typical. In granulomatosis with polyangiitis, damage to the organ of vision is also sometimes possible, but mainly in the form of an orbit pseudotumor. Despite the fact that treatment for both forms of pathology involves the use of glucocorticoids and cytostatics, with a scarring pemphigoid, the main care is provided by dermatologists and ophthalmologists, while the diagnosis and treatment of systemic vasculitis is the task of rheumatologists. One of the criteria for a scarring pemphigoid is loss of vision. However, in this case, it was possible to establish a diagnosis, obtain the first positive results of therapy before the patient shows signs of disability. So, there is a hope for the possibility of preserving vision and a favorable outcome. The rarity of the disease and its poor knowledge, difficulties in diagnosis and the absence of certain standards of therapy, this diagnosis requires more attention from the specialists.

scholarly journals GRANULOMATOSIS WITH POLYANGIITIS: TREAT THE PATIENT NOT SYMPTOMS

2018 ◽  
Keyword(s):  
Granulomatosis With Polyangiitis ◽  
Treatment Strategies ◽  
Granulomatous Inflammation ◽  
Organ Damage ◽  
Upper Respiratory Tract ◽  
Childbearing Age ◽  
Immunological Mechanisms ◽  
Upper Respiratory ◽  
Tract Infections ◽  
In Pregnancy

Immunological mechanisms of appearance and therapeutic treatment strategies were discussed on example of the rare granulomatosis with polyangiitis clinical case in young patient. This vasculitis, formerly known as Wegener’s granulomatosis, is a rare multisystem autoimmune disease with necrotizing granulomatous inflammation and pauci-immune vasculitis in small- and medium-sized blood vessels. Autoimmune diseases affect 5 to 7% of people, are commoner in women of childbearing age, and are frequently encountered in pregnancy. They may remit or improve during pregnancy, butcan flare or present in pregnancy with disastrous consequences. Otorhinolaryngologist is the first physician to contact for the majority of patients with GPA. This diagnosis must always be taken into consideration in patients with recurrent upper respiratory tract infections, otitis, mucosal ulcers and laryngitis. Proper and early diagnosis is crucial for imminent therapy implementation and allows avoiding irreversible organ damage.

scholarly journals Renal involvement in granulomatosis with polyangiitis

2020 ◽  
Vol 8 (6) ◽  
pp. 158-159
Author(s):  
Aya Fraj ◽  
Olfa Berriche ◽  
Sondes Arfa ◽  
Jihen Chelli ◽  
Wafa Bel Abed ◽  
...  
Keyword(s):  
Renal Failure ◽  
Respiratory Tract ◽  
Granulomatosis With Polyangiitis ◽  
Renal Involvement ◽  
Rapidly Progressive Glomerulonephritis ◽  
Small Vessel ◽  
Small Vessel Vasculitis ◽  
Upper Respiratory Tract ◽  
Terminal Renal Failure ◽  
Upper Respiratory

Granulomatosis with polyangiitis (GPA) is defined by the presence of small vessel vasculitis that affects the upper respiratory tract, lungs and kidneys. Renal involvement is frequent. The disease varies from asymptomatic to fulminant rapidly progressive glomerulonephritis forms, leading to terminal renal failure in days.

scholarly journals AB0098 ASSOCIATED FACTORS IN ONE-YEAR MORTALITY IN PATIENTS WITH SMALL VESSEL SYSTEMIC VASCULITIS

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 1078.2-1078
Author(s):  
E. M. Escudero Tepale ◽  
M. A. Saavedra ◽  
D. Miranda ◽  
Z. Castro ◽  
M. J. R. Arriga Torres
Keyword(s):  
Associated Factors ◽  
Granulomatosis With Polyangiitis ◽  
Systemic Vasculitis ◽  
Small Vessel ◽  
Small Vessel Vasculitis ◽  
Remission Induction ◽  
First Year ◽  
Factors Associated ◽  
Number Of Patients ◽  
One Year

Background:The highest mortality rates in patients with small vessel systemic vasculitis occur within the first year after the diagnosis, however associated factors have been scarcely studied in our population.Objectives:To identify mortality associated factors at the time of diagnosis in patients with small vessel systemic vasculitis.Methods:Retrospective cohort (2009-2020) involving 81 patients diagnosed with systemic small vessel vasculitis. Demographic, clinical and biochemical parameters were studied as potential factors associated with one-year mortality.Results:Of the total of patients (n=81), 36 (44.4%) had generalized granulomatosis with polyangiitis, 32 (39.5%) had localized granulomatosis with polyangiitis and 5 (6.2%) had early systemic granulomatosis with polyangiitis, 7 (8.6%) had microscopic polyangiitis and 1 (1.25%) had eosinophilic granulomatosis with polyangiitis. Twenty-two deaths (27%) were observed, 14 of them (63.6%) happened within the first year of diagnosis. The leading cause of death was infection (64%). Patients who died within the first year of diagnosis had a higher frequency of hypoalbuminemia (p=0.05) and also presented hemoglobin lower than 10.8 g/dL (p=0.035) in comparison with those who died after the first year of diagnosis. Remission induction treatment did not differ between both groups.Conclusion:Our study suggests that hypoalbuminemia and anemia are factors associated with a higher mortality within the first year after the diagnosis in patients with systemic small vessel vasculitis which contrast with previously reported data. The study design and the reduced number of patients are two major limitations of the study.References:[1]Flossmann O, Berden A, Groot K, et al. Long-term patient survival in ANCA-associated vasculitis. Ann Rheum Dis. 2011;70:488-94.Disclosure of Interests:None declared

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