Materiały źródłowe

• #1 Granulomatosis with polyangiitis (Wegener’s granulomatosis)

  https://patient.info/doctor/granulomatosis-with-polyangiitis-wegeners-granulomatosis-pro

  Granulomatosis with polyangiitis (GPA) – formerly known as Wegener’s granulomatosis – is a rare form of vasculitis. It is thought to be an autoimmune inflammatory process affecting endothelial cells. It is a multisystem disease which can affect many parts of the body, categorised by the ELK classification: it most commonly presents with lesions in the upper respiratory tract (E indicating ears/nose/throat, almost 100%), lungs (L most patients) and kidneys (K75%). Many other areas of the body may also be affected, with joint inflammation occurring in 25-50% of all cases. The sinuses, eyes and skin may also be affected. […] As a multisystem disease, GPA often presents with nonspecific symptoms and can be difficult to recognise in primary care. The delay from onset to diagnosis ranges from 2-20 months.

• #1 Granulomatosis with Polyangiitis (GPA) – Musculoskeletal and Connective Tissue Disorders – Merck Manual Professional Edition

  https://www.merckmanuals.com/professional/musculoskeletal-and-connective-tissue-disorders/vasculitis/granulomatosis-with-polyangiitis-gpa

  Granulomatosis with polyangiitis should be suspected in patients with chronic, unexplained respiratory symptoms and signs (including otitis media in adults), particularly if manifestations in other organ systems, especially the kidneys, also suggest the disorder. […] Diagnosis usually requires biopsy. […] Routine laboratory tests are done, but ANCA testing and selective biopsy of involved tissues yield the most specific findings. […] Serologic testing to detect antineutrophil cytoplasmic antibodies (ANCA) is followed by enzyme-linked immunosorbent assay (ELISA) to check for specific antibodies. […] A positive ANCA test does not exclude mycobacterial and fungal infections; thus, patients with positive ANCA results and cavitary lung lesions still require bronchoscopy and adequate cultures and other tests for tuberculosis and fungal infections.

• #1 Granulomatosis With Polyangiitis – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK557827/

  The 2022 ACR/European Alliance of Associations for Rheumatology (EULAR) classification criteria for GPA includes the following weighted criteria: Bloody nasal discharge, nasal crusting, or sinonasal congestion (+3); Cartilaginous involvement (+2); Conductive or sensorineural hearing loss (+1); Cytoplasmic ANCA or anti-PR3 ANCA positivity (+5); Pulmonary nodules, mass, or cavitation on chest imaging (+2); Granuloma or giant cells on biopsy (+2); Inflammation or consolidation of the nasal/paranasal sinuses on imaging (+1); Pauci-immune glomerulonephritis (+1); Perinuclear ANCA or anti-MPO ANCA positivity (1); Eosinophil count more than 1109 cells/L (4). […] After excluding mimics of vasculitis, a patient diagnosed with small- or medium-vessel vasculitis could be classified as having GPA if the cumulative score is 5 or more points. When these criteria were tested in the validation dataset, the sensitivity was 93%, and the specificity was 94%.

• #1 Granulomatosis with Polyangiitis (GPA, formerly Wegener Granulomatosis) Workup: Approach Considerations, ANCA Detection, Radiography and CT Scanning

  https://emedicine.medscape.com/article/332622-workup

  Routine laboratory tests are nonspecific in granulomatosis with polyangiitis (GPA). Elevated blood urea nitrogen (BUN) and creatinine levels may signal renal involvement. Mild normochromic normocytic anemia is present in 50% of patients. A peripheral blood smear may show schistocytes and burr cells. Leukocytosis is also common, with a neutrophil predominance. Eosinophilia is not a feature of GPA but rather of eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome). […] The erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) levels are elevated in 90% of patients with active and generalized disease. They may decrease in response to treatment. […] In patients with kidney involvement, urinalysis may show nonnephrotic-range (proteinuria 1g or less per day) to nephrotic-range proteinuria, microscopic hematuria, and the presence of red blood cell (RBC) casts consistent with underlying glomerulonephritis. Repeat kidney function studies may show a rise in creatinine over days or weeks.

• #1 Granulomatosis with polyangiitis – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/granulomatosis-with-polyangiitis/diagnosis-treatment/drc-20351093

  Diagnosis involves the steps that your healthcare team takes to find out if you have granulomatosis with polyangiitis (GPA). Your healthcare professional asks you about your symptoms and health history. You also get a physical exam. You may need other tests as well. […] Blood tests can check for: […] Antineutrophil cytoplasmic antibodies. These immune system proteins attack healthy white blood cells called neutrophils by mistake. The antibodies appear in the blood of most people who have GPA. […] Urine tests can reveal if the urine has red blood cells or too much protein. These might mean that the disease is affecting the kidneys. […] If you have lung symptoms, chest X-rays and CT scans can help find out if granulomatosis with polyangiitis is the cause. […] During a biopsy, a healthcare professional removes a small sample of tissue from the affected area of the body. […] This test can confirm if you have granulomatosis with polyangiitis. […] A specialist likely will be the one to find out if you have granulomatosis with polyangiitis.

• #1 Granulomatosis with Polyangiitis (GPA, formerly Wegener Granulomatosis) Workup: Approach Considerations, ANCA Detection, Radiography and CT Scanning

  https://emedicine.medscape.com/article/332622-workup

  Whether tissue diagnosis is always required for GPA remains controversial. As the therapy for severe GPA is not benign, tissue diagnosis is recommended if a biopsy site is available, provided that the patient understands the risks of the procedure. […] Antineutrophil cytoplasmic antibodies (ANCAs) can be detected with serologic assays. The 2 types of assays in common use are immunofluorescence (IF) and enzyme immunoassay. A 2017 position paper by an international consensus panel stated that high-quality immunoassays are the preferred screening method for the diagnosis of ANCA-associated vasculitis, and that screening does not categorically require indirect IF assays. […] C-ANCA directed against PR3 is most specific for GPA. According to the WGET trial, IF shows positive C-ANCA results in 88% of all patients with GPA (in 87% of patients with severe disease and in 90% of those with limited disease).

• #1 Granulomatosis with Polyangiitis (GPA, formerly Wegener Granulomatosis) Workup: Approach Considerations, ANCA Detection, Radiography and CT Scanning

  https://emedicine.medscape.com/article/332622-workup

  Using both IF and ELISA, ANCA is detectable in nearly 100% of patients with active generalized GPA. […] The diagnosis of GPA is generally confirmed with tissue biopsy from a site of active disease, and kidney and lung biopsies are most specific for GPA. However, sampling error may occur, and histopathologic findings can be nonspecific. Tissue diagnosis may not be required if the clinical gestalt is convincing and a site for biopsy is not apparent or would be too invasive to obtain. For example, the finding of leukocytoclastic vasculitis in the setting of pulmonary nodules and PR3-ANCA may be sufficient for the diagnosis. […] Kidney biopsy may be easier to perform than lung biopsy and has a greater diagnostic yield. The typical renal lesion of GPA is segmental crescentic necrotizing glomerulonephritis with little or no immunoglobulin or complement deposition (pauci-immune). Generally, vasculitis is not observed, but this histology helps in establishing the diagnosis. However, kidney biopsy findings cannot be used to distinguish between GPA and microscopic polyarteritis. […] Although findings on a kidney biopsy are often negative for the presence of granulomas in GPA, kidney biopsy is nonetheless a very useful diagnostic tool, especially in the setting of pulmonary-renal syndrome.

• #1 Granulomatosis With Polyangiitis – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK557827/

  Various diagnostic criteria have been proposed to diagnose GPA and distinguish the disease from other forms of vasculitis. The ACR criteria include the following: Urinary sediment showing red blood cell casts or more than 5 red blood cells per high-power field; Abnormal findings on chest radiography; Oral ulcer or nasal discharge; Granulomatous inflammation on biopsy. The presence of 2 or more of the 4 criteria mentioned above is associated with a 92% specificity and 88% sensitivity. […] Although ANCA serology is essential for diagnosing ANCA-associated vasculitis, the diagnosis cannot rely solely on ANCA testing, as positive results may also be found in other inflammatory conditions. ANCA serology for MPO or PR3 is positive in about 90% of GPA and MPO cases, whereas it is positive in about 40% of EGPA cases. A study found that GPA is 80% positive for anti-PR3, 15% positive for anti-MPO, and 5% ANCA-negative. Anti-PR3 antibodies are about 90% sensitive for diagnosing GPA.

• #1 Granulomatosis with Polyangiitis – Vasculitis Foundation

  https://vasculitisfoundation.org/education/vasculitis-types/granulomatosis-with-polyangiitis/

  The most common blood test for GPA checks for the antibody called ANCA. The ANCA test is positive in most individuals with GPA, so it may help support a suspected diagnosis of GPA. However, a positive test alone does not confirm diagnosis. […] A urinalysis can detect red blood cells or excess protein in the urine, which can indicate whether the kidneys are being affected. […] Chest X-rays can show changes in the lungs. Computed tomography (CT) scans and magnetic resonance imaging (MRI) provide more detailed images of the internal organs and can better reveal abnormalities in the sinuses, chest, brain, blood vessels or abdominal organs. […] This surgical procedure removes a small tissue sample from an affected organ such as the lung, kidney, or skin, which is examined under a microscope for signs of inflammation or tissue damage.

• #1 Granulomatosis With Polyangiitis – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK557827/

  Radiological evaluation of the sinuses, lungs, trachea, and orbits can be performed to assess the sites and extent of involvement. A chest x-ray and computed tomography (CT) scan of the lungs can be performed to identify pulmonary lesions and hemorrhage, which can help differentiate GPA from MPA. A chest x-ray is abnormal in about 89% of cases. Nodular lesions are the most common radiologic finding. […] The treatment of GPA involves the use of immunosuppressive agents in a variety of combinations. Treatment is classified into 2 phases – the induction phase and the maintenance phase. Commonly used agents are cyclophosphamide, glucocorticoids, rituximab, azathioprine, methotrexate, and plasmapheresis if indicated. […] For new-onset organ/life-threatening GPA (and MPA), induce remission with glucocorticoids and rituximab or cyclophosphamide. Rituximab is preferred for relapsing disease.

• #1 Granulomatosis With Polyangiitis | Applied Radiology

  https://appliedradiology.com/articles/granulomatosis-with-polyangiitis

  Granulomatosis with polyangiitis (formerly Wegener granulomatosis). […] The American College of Rheumatology criteria for diagnosing GPA require two of the following: a) abnormal chest imaging; b) granulomatous inflammation shown on biopsy; c) oral ulcers or nasal discharge, and d) urinalysis with 5 RBC/ high-power field or red blood cell casts. […] GPA is more commonly diagnosed in adults than in children; prevalence in the pediatric population ranges from 0.45-6.39 cases/million/year depending on the referenced study. […] The radiographic findings of GPA are variable. Chest X-rays often show multiple nodules of variable sizes that can wax and wane over time. Cavitation can be appreciated in approximately 50% of cases. Initial radiographs may show consolidation or multifocal hazy opacities.

• #1 Granulomatosis with Polyangiitis (GPA) – Johns Hopkins Vasculitis Center

  https://www.hopkinsvasculitis.org/types-vasculitis/granulomatosis-with-polyangiitis/

  Granulomatosis with Polyangiitis is a disease involving granulomatous inflammation, necrosis and vasculitis that most frequently targets the upper respiratory tract, lower respiratory tract, and kidneys. […] The diagnosis of Granulomatosis with Polyangiitis is established most securely by biopsy specimens showing the triad of vasculitis, granulomata, and large areas of necrosis (known as geographic necrosis) admixed with acute and chronic inflammatory cells. […] Whenever possible, it is important to confirm the diagnosis of Granulomatosis with Polyangiitis by biopsying an involved organ and finding the pathologic features of this disease under the microscope. […] Since 1982, when ANCAs (antineutrophil cytoplasmic antibodies) were first described, the role of these antibodies in the diagnosis of Granulomatosis with Polyangiitis has grown. […] When CANCAs are present in the blood of a patient whose symptoms or signs suggest Granulomatosis with Polyangiitis, the likelihood of the diagnosis increases considerably. […] Seropositivity for C-ANCAs alone is not specific enough to establish the diagnosis of Granulomatosis with Polyangiitis.

• #1 Granulomatosis with polyangiitis – Wikipedia

  https://en.wikipedia.org/wiki/Granulomatosis_with_polyangiitis

  These granulomas are the main reason for the name granulomatosis with polyangiitis, although it is not an essential feature. Nevertheless, necrotizing granulomas are a hallmark of this disease. Many biopsies can be nonspecific, though, and 50% provide too little information for the diagnosis of GPA. […] In 1990, the American College of Rheumatology accepted classification criteria for GPA. These criteria were not intended for diagnosis but for inclusion in randomized controlled trials. Two or more positive criteria have a sensitivity of 88.2% and a specificity of 92.0% of describing GPA. […] According to the Chapel Hill Consensus Conference (CHCC) on the nomenclature of systemic vasculitis (1992), establishing the diagnosis of GPA demands a granulomatous inflammation involving the respiratory tract, and a vasculitis of small to medium-sized vessels. […] In 2022, the American College of Rheumatology and the European Alliance of Associations for Rheumatology updated the classification criteria for GPA.

• #1 The Classification & Diagnosis of Granulomatosis with Polyangiitis – Page 6 of 8 – The Rheumatologist

  https://www.the-rheumatologist.org/article/the-classification-diagnosis-of-granulomatosis-with-polyangiitis/6/?singlepage=1

  Granulomatosis with polyangiitis (GPA) is a subset of ANCA-associated vasculitis defined as a necrotizing granulomatous inflammation usually involving the respiratory tract and necrotizing vasculitis involving small and medium vessels, which is commonly associated with glomerulonephritis. […] The diagnosis of GPA is based on the patients clinical presentation and supporting laboratory and histologic findings. However, no well-established diagnostic rules or criteria exist. A positive ANCA is not required to diagnose GPA, and diseases that present similarly to a small vessel vasculitis must be excluded. […] The diagnosis of GPA is typically made using clinical judgment by evaluating a patients symptoms together with laboratory and imaging findings while excluding other possible diagnoses, and by observation of improvement on immunosuppressive therapies.

• #1 Too early diagnosis of granulomatosis with polyangiitis (GPA) in the first month of initial presentation

  https://www.probiologists.com/article/too-early-diagnosis-of-granulomatosis-with-polyangiitis-gpa-in-the-first-month-of-initial-presentation

  But they are mainly for classifying GPA and none have sufficient sensitivity and specificity for early diagnosis. […] It is claimed that they are able to diagnose GPA in the first a few months and the first month of the disease, respectively. […] The Iran Criteria for Early Diagnosis of GPA suggests evaluation of three organs and two laboratory findings (ANCA and biopsy), after ruling out other prominent diagnoses by history and physical examination. […] The appropriate approach toward a correct diagnosis for a patient suspected as suffering from GPA is „Amir-A lam Hospital approach towards the diagnosis of GPA”. […] The sensitivity of the two sets of Criteria was measured using the following formula: Sensitivity=(number of the patients classified as GPA by the Criteria)/(number of the patients diagnosed as GPA by an expert Rheumatologist).

• #1 Granulomatosis with Polyangiitis (GPA) – EyeWiki

  https://eyewiki.org/Granulomatosis_with_Polyangiitis_(GPA)

  One of the most common utilized tests in the diagnosis of GPA is ANCA, and has a high diagnostic utility for ANCA-associated vasculitis: GPA, microscopic polyangiitis (MPO), and eosinophilic granulomatosis. Antibodies reacting with PR3 generate a specific immunofluorescence cytoplasmic (c) pattern (also known as c-ANCA) and antibodies reacting to MPO have a perinuclear (p) staining pattern (also known as p-ANCA). […] Differential diagnosis is broad due to the varied presentations of the disease. It can include other forms of ANCA associated vasculitis such as Churg Strauss syndrome and MPA, infections, malignancies, as well as granulomatous and autoimmune disorders.

• #1 The Diagnosis of Granulomatosis With Polyangiitis When Serology and Biopsies are Negative

  https://pmc.ncbi.nlm.nih.gov/articles/PMC11094516/

  Granulomatosis with polyangiitis (GPA) is a potentially fatal condition which often manifests in the head and neck. Currently, diagnosis relies on antineutrophil cytoplasmic autoantibody (cANCA) serology and mucosal or renal biopsy. However, a significant proportion of patients with GPA limited to the head and neck are seronegative and biopsy negative. This study evaluates the role of clinical diagnosis of GPA in the absence of positive laboratory findings. […] GPA cases are often limited to the upper respiratory tract, making diagnosis difficult, particularly in seronegative patients. These results suggest that, when GPA is suspected, despite negative serology, the diagnosis of GPA should be made on clinical grounds, and empiric therapy encouraged to prevent delay in treatment. […] GPA can be a challenging diagnosis to make, particularly in patients with a limited form, due to the nonspecific nature of its symptoms. Often, the early differential is broad and includes common causes of nasal mucosal crusting such as infectious rhinosinusitis, allergic rhinitis, drug-induced rhinosinusitis, and sarcoidosis as well as vasculitides such as microscopic polyangiitis and eosinophilic granulomatosis with polyangiitis (EGPA).

• #1 Granulomatosis with polyangiitis – Knowledge @ AMBOSS

  https://www.amboss.com/us/knowledge/granulomatosis-with-polyangiitis/

  Granulomatosis with polyangiitis (GPA; previously referred to as Wegener granulomatosis) is a systemic vasculitis that affects both small and medium-sized vessels. […] Diagnosis is confirmed by biopsy findings of necrotizing granulomatous inflammation. […] Suspect GPA in patients with either: Characteristic clinical features (i.e., glomerulonephritis, pulmonary nodules, and ENT inflammation) or Positive c-ANCA. […] Characteristic histopathological findings confirm the diagnosis. […] While positive PR3-ANCAs have a high sensitivity for GPA (98%), their absence does not exclude the diagnosis. […] Consider in all patients to confirm the diagnosis. […] Biopsy may not be required in certain patients, e.g., those with characteristic clinical features and positive PR3-ANCA, because results will not affect management. […] For patients with severe kidney or lung disease, do not delay treatment for biopsy. Several days of treatment do not significantly reduce the biopsy yield of biopsy samples.

• #1 Granulomatosis With Polyangiitis – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK557827/

  For maintenance of remission, rituximab is recommended. Azathioprine or methotrexate can also be considered, but their use should be reserved for patients with eGFR more than 60 mL/min/1.73m2. […] Maintenance therapy typically lasts 12 to 36 months after remission, but in high-risk patients, it may be continued indefinitely.

• #1 Granulomatosis with Polyangiitis | Rheumatology & Clinical Research located in North Houston, Tomball, TX | Rheumatology Clinic of Houston

  https://www.houstonrheumatology.com/content/granulomatosis-with-polyangiitis

  However, ANCA cannot be found in up to 20% of people with well-diagnosed GPA, so a negative ANCA test does not eliminate the possibility of GPA in patients with other characteristic clinical features. […] Active GPA involving a critical organ site requires treatment to prevent ongoing inflammation that can result in organ failure. […] Treatment choices are based on the organs affected, disease severity and individual medical factors. […] The patient and his or her doctor will work together to decide whether the patient should receive cyclophosphamide or rituximab for severe GPA. […] Regular doctor visits together with monitoring of laboratory tests and imaging studies can help to detect relapses early.

• #1 Granulomatosis with polyangiitis (Wegener’s) (GPA) | Vasculitis Clinical Research Consortium

  https://vcrc.rarediseasesnetwork.org/diseases-studied/granulomatosis-polyangiitis

  Granulomatosis with polyangiitis (Wegener’s) (GPA) is a rare form of vasculitis mainly involving small and medium-sized blood vessels. […] The diagnosis of GPA is made by combining clinical features with laboratory tests (including tests for ANCA) and biopsy of affected tissues. […] Can you still be diagnosed with granulomatosis with polyangiitis (GPA, Wegeners) if the ANCA is negative? Yes. Although ANCA is elevated in a majority of patients with granulomatosis with polyangiitis (Wegeners) it is not detectable in all patients and is not necessary to make the diagnosis. […] A general and over-simplified answer is: no. This answer is based on the statistical analysis of large groups of patients followed prospectively in clinical trials. […] There are some patients, however, for whom ANCA levels track the disease activity very well. In these patients, ANCA increases do predict relapses. To know how ANCA levels relate to disease activity in individual patients, their ANCA levels need to be monitored over time.

• #1 Granulomatosis with polyangiitis: MedlinePlus Medical EncyclopediaLock

  https://medlineplus.gov/ency/article/000135.htm

  Granulomatosis with polyangiitis (GPA) is a rare disorder in which blood vessels become inflamed. This leads to damage in major organs of the body. It was formerly known as Wegener’s granulomatosis. […] You may have a blood test that looks for ANCA proteins as well as two additional proteins called proteinase 3 (PR3) and myeloperoxidase (MPO). These tests are abnormal in most people with active GPA, however, this test is sometimes negative, even in people with the condition. […] A biopsy is sometimes needed to confirm the diagnosis and check how severe the disease is. A kidney biopsy is most commonly done. […] Without treatment, people with severe forms of this disease can die within a few months. […] With treatment, the outlook for most patients is good. Most people who receive corticosteroids and other medicines that slow the immune response get much better. […] Most people with GPA are treated with ongoing medicines to prevent relapse for at least 12 to 24 months.

• #1 The Diagnosis of Granulomatosis With Polyangiitis When Serology and Biopsies are Negative

  https://pmc.ncbi.nlm.nih.gov/articles/PMC11094516/

  Our findings suggest that the prevalence of double-negative patients is not trivial (16.08% in our cohort). This finding appears to support the preponderance of data in the literature that indicate a relatively low specificity for mucosal biopsy and cANCA in the diagnosis of GPA. […] These findings should inform the most appropriate next step for the patient with a clinical presentation suggestive of GPA but with negative serology and biopsy results. If, as the data from the present series suggests, roughly 16% of patients with limited GPA will present in this manner, the diagnosis of GPA should not be ruled out based on negative serology and biopsies. […] Our series demonstrates that GPA can present without positive serologic or pathologic findings, but that the diagnosis can be made on clinical grounds. If GPA is highly suspected clinically in the setting of negative laboratory findings, we recommend empiric with immunosuppressive therapy. Furthermore, delaying treatment until cANCA titers become positive is not indicated as seroconversion appears to be a rare event.

• #1 Granulomatosis with Polyangiitis initially misdiagnosed as lung cancer | Pulmonology

  https://journalpulmonology.org/en-granulomatosis-with-polyangiitis-initially-misdiagnosed-articulo-S2173511512000504

  Granulomatosis with Polyangiitis (GPA), which was formerly named Wegener’s Granulomatosis (WG) is a systemic disease characterized by necrotizing granulomatous inflammation and vasculitis that primarily involves upper and lower respiratory tract, as well as kidneys. Diagnosing GPA on the basis of transthoracic fine needle aspiration (TFNA) may be problematic, as it can be misdiagnosed as cancer. We describe a patient with a probable GPA which was originally diagnosed as malignancy, but who responded to lung cancer chemotherapy. […] Diagnosing GPA on the basis of transthoracic fine needle aspiration (TFNA) may be problematic, as it can be misdiagnosed as cancer. We describe a patient with GPA originally diagnosed as malignancy. […] A definite histological diagnosis of GPA requires coexistence of vasculitis, granuloma and necrosis although these features are only found in a minority of specimens. Only 1 or 2 of these features may be found, rendering a suggestive biopsy, in which the diagnosis is then supported by clinical criteria. Histopathologic documentation of granulomatous involvement of the respiratory tract is not explicitly required. Therefore, the diagnosis of GPA depends on a combination of clinical, pathologic, and serologic features.

• #1 Granulomatosis with Polyangiitis initially misdiagnosed as lung cancer | Pulmonology

  https://journalpulmonology.org/en-granulomatosis-with-polyangiitis-initially-misdiagnosed-articulo-S2173511512000504

  Our case reports a probable localized GPA, interpreted as stage IV NSCLC. Since the patient only had pulmonary disease and the lesions were suggestive of metastatic lesions, ANCA were not initially tested. However, it is well known that cANCA sensitivity is as low as 6070% in limited GPA, making its diagnosis more difficult. […] Solitary or multifocal involvement of the lung by GPA without evidence of extrapulmonary disease is sometimes a difficult diagnosis due to its histopathological variability and possible misinterpretation as lung cancer. […] Awasthi et al. report six cases of suspected GPA who underwent TNFA; four of them were wrongly diagnosed, one of which was a misdiagnosed squamous cell carcinoma. […] The positive predictive value of lung FNA ranges from 96% to 100%, a patient with a lung fine-needle aspirate diagnosed as malignant has a high probability of harboring a malignancy. However, as with any other diagnostic tests, lung FNA is subject to diagnostic pitfalls that can lead to occasional false-positive diagnoses; the false-positive rate of lung FNA has been cited as being less than 1%.

• #1 Frequency, Diagnosis, Treatment, and Outcome of Gastrointestinal Disease in Granulomatosis with Polyangiitis and Microscopic Polyangiitis | The Journal of Rheumatology

  https://www.jrheum.org/content/early/2018/01/24/jrheum.170249

  Objective Involvement of the gastrointestinal (GI) tract is a rare complication of granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA). The aim was to describe frequency, diagnosis, treatment, and outcome of GI disease in a large series of patients in a single center. […] Results Fourteen (6.5%) of 216 consecutive patients with GPA/MPA had GI manifestations. Abdominal pain and GI bleeding were the most common symptoms. Radiology was important for detection of GI disease, while endoscopy failed to support the diagnosis in many patients. […] Conclusion GI disease was found in 6.5% among 216 patients with GPA or MPA. Surgery was judged necessary only in cases with GI perforation or severe bleeding. Multidisciplinary engagement is strongly recommended.

• #1 Granulomatosis with Polyangiitis (GPA) formerly known as Wegener’s Granulomatosis | Eosinophilic Granulomatosis with Polyangiitis (EGPA) | Phoenix Arizona Sinus Center

  http://www.arizonasinus.com/condition-granulomatosis-with-polyangiitis-wegeners.htm

  Granulomatosis with Polyangiitis (GPA) is a vasculitis (inflammation of blood vessels) characterized by involvement of small to medium sized vessels and necrotizing granulomas (scattered spherical areas of tissue destruction). […] Most patients with GPA present to an Otolaryngologist (ENT) physician (often times before they are diagnosed with GPA) because of ear, nose or throat symptoms. […] To determine if a patient has GPA the ENT physician will perform a thorough ear, nose and throat exam, nasal endoscopy and order or perform tests looking for specific markers of GPA. […] GPA can be difficult to accurately diagnose due to the fact that the blood test and biopsy markers we look for to make the diagnosis may only be present 50% of the time when the disease is present. […] If the ENT physician can make the diagnosis (or is strongly suspicious of the diagnosis), referral to a Rheumatologist is made.

• #1 Comprehensive Granulomatosis with Polyangiitis Treatment in Atlanta

  https://argmd.net/conditions-we-treat/granulomatosis-with-polyangiitis/

  Granulomatosis with Polyangiitis (GPA), formerly known as Wegeners Granulomatosis, is a rare autoimmune disease characterized by inflammation of the blood vessels (vasculitis), which can restrict blood flow and damage vital organs and tissues. […] Early diagnosis and treatment at Arthritis and Rheumatology of Georgia (ARG) are crucial for managing symptoms and preventing serious complications. […] Diagnosis involves a combination of medical history, physical examination, blood tests, imaging tests, and biopsy. […] At Arthritis and Rheumatology of Georgia, we offer comprehensive care for GPA, including diagnostic tests, personalized treatment plans, and ongoing management to reduce inflammation and improve quality of life.

• #1 Granulomatosis with Polyangiitis (GPA)—A Multidisciplinary Approach of a Case Report

  https://www.mdpi.com/1648-9144/58/12/1837

  The treatment of GPA is through medication and is based on a combination of immunosuppressants divided into various phases, followed by a maintenance treatment once remission has been achieved. […] Granulomatosis with polyangiitis is a rare and fatal disease without treatment. Early diagnosis and treatment are key to controlling its progression. Necrotizing granulomatous inflammation and vasculitis on a biopsy, along with the presence of ANCAs, provide support for the diagnosis of the disease.

• #2 Granulomatosis with polyangiitis (GPA): Symptoms, causes, and more

  https://www.medicalnewstoday.com/articles/187807

  Granulomatosis with polyangiitis (GPA), previously known as Wegeners granulomatosis, is a rare and life-threatening disorder that restricts the blood flow to several organs, including the lungs, kidneys, and upper respiratory tract. […] GPA requires long-term immunosuppression. Some patients die due to the toxicity of the treatment. […] With early treatment and diagnosis, the symptoms of GPA can go into remission. Untreated, it can lead to kidney failure and death. […] Diagnosis can be difficult, because a patient may have no symptoms in the early stages, or symptoms may be nonspecific. It can take from 2 to 20 months to reach a diagnosis, in primary care. […] Blood tests may detect ANCAs, the antibodies that are present in over 90 percent of patients with active GPA. […] ANCAs alone cannot confirm a diagnosis, but they can indicate that the condition may be present.

• #2 Granulomatosis with polyangiitis – Harvard Health

  https://www.health.harvard.edu/a_to_z/granulomatosis-with-polyangiitis-a-to-z

  Granulomatosis with polyangiitis (GPA) is a rare disease marked by inflammation of the blood vessels. […] Your doctor may suspect GPA if you have: unusually persistent symptoms of sinusitis, respiratory tract symptoms, unexplained kidney disease. […] Many early symptoms of GPA are similar to those of milder and more common respiratory problems. As a result, the average person with GPA often has symptoms for months before the correct diagnosis is made. […] After the physical exam, your doctor may send you for tests. These tests will look for inflammation and organ damage. The tests will focus on your upper respiratory tract, lungs and kidneys. […] The only way to confirm this diagnosis is to have a biopsy. Tissue is taken from an affected organ. It is then examined under a microscope to check for granulomas and areas of inflammation.

• #2 Granulomatosis with Polyangiitis – Vasculitis Foundation

  https://vasculitisfoundation.org/education/vasculitis-types/granulomatosis-with-polyangiitis/

  The most common blood test for GPA checks for the antibody called ANCA. The ANCA test is positive in most individuals with GPA, so it may help support a suspected diagnosis of GPA. However, a positive test alone does not confirm diagnosis. […] A urinalysis can detect red blood cells or excess protein in the urine, which can indicate whether the kidneys are being affected. […] Chest X-rays can show changes in the lungs. Computed tomography (CT) scans and magnetic resonance imaging (MRI) provide more detailed images of the internal organs and can better reveal abnormalities in the sinuses, chest, brain, blood vessels or abdominal organs. […] This surgical procedure removes a small tissue sample from an affected organ such as the lung, kidney, or skin, which is examined under a microscope for signs of inflammation or tissue damage.

• #2 Granulomatosis With Polyangiitis – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK557827/

  Various diagnostic criteria have been proposed to diagnose GPA and distinguish the disease from other forms of vasculitis. The ACR criteria include the following: Urinary sediment showing red blood cell casts or more than 5 red blood cells per high-power field; Abnormal findings on chest radiography; Oral ulcer or nasal discharge; Granulomatous inflammation on biopsy. The presence of 2 or more of the 4 criteria mentioned above is associated with a 92% specificity and 88% sensitivity. […] Although ANCA serology is essential for diagnosing ANCA-associated vasculitis, the diagnosis cannot rely solely on ANCA testing, as positive results may also be found in other inflammatory conditions. ANCA serology for MPO or PR3 is positive in about 90% of GPA and MPO cases, whereas it is positive in about 40% of EGPA cases. A study found that GPA is 80% positive for anti-PR3, 15% positive for anti-MPO, and 5% ANCA-negative. Anti-PR3 antibodies are about 90% sensitive for diagnosing GPA.

• #2 Granulomatosis with Polyangiitis (GPA, formerly Wegener Granulomatosis) Workup: Approach Considerations, ANCA Detection, Radiography and CT Scanning

  https://emedicine.medscape.com/article/332622-workup

  Whether tissue diagnosis is always required for GPA remains controversial. As the therapy for severe GPA is not benign, tissue diagnosis is recommended if a biopsy site is available, provided that the patient understands the risks of the procedure. […] Antineutrophil cytoplasmic antibodies (ANCAs) can be detected with serologic assays. The 2 types of assays in common use are immunofluorescence (IF) and enzyme immunoassay. A 2017 position paper by an international consensus panel stated that high-quality immunoassays are the preferred screening method for the diagnosis of ANCA-associated vasculitis, and that screening does not categorically require indirect IF assays. […] C-ANCA directed against PR3 is most specific for GPA. According to the WGET trial, IF shows positive C-ANCA results in 88% of all patients with GPA (in 87% of patients with severe disease and in 90% of those with limited disease).

• #2 Granulomatosis with Polyangiitis (GPA) – Johns Hopkins Vasculitis Center

  https://www.hopkinsvasculitis.org/types-vasculitis/granulomatosis-with-polyangiitis/

  Granulomatosis with Polyangiitis is a disease involving granulomatous inflammation, necrosis and vasculitis that most frequently targets the upper respiratory tract, lower respiratory tract, and kidneys. […] The diagnosis of Granulomatosis with Polyangiitis is established most securely by biopsy specimens showing the triad of vasculitis, granulomata, and large areas of necrosis (known as geographic necrosis) admixed with acute and chronic inflammatory cells. […] Whenever possible, it is important to confirm the diagnosis of Granulomatosis with Polyangiitis by biopsying an involved organ and finding the pathologic features of this disease under the microscope. […] Since 1982, when ANCAs (antineutrophil cytoplasmic antibodies) were first described, the role of these antibodies in the diagnosis of Granulomatosis with Polyangiitis has grown. […] When CANCAs are present in the blood of a patient whose symptoms or signs suggest Granulomatosis with Polyangiitis, the likelihood of the diagnosis increases considerably. […] Seropositivity for C-ANCAs alone is not specific enough to establish the diagnosis of Granulomatosis with Polyangiitis.

• #2 Too early diagnosis of granulomatosis with polyangiitis (GPA) in the first month of initial presentation

  https://www.probiologists.com/article/too-early-diagnosis-of-granulomatosis-with-polyangiitis-gpa-in-the-first-month-of-initial-presentation

  The Clinical Triad of the disease include involvement of the ENT (Ear, Nose, and Throat) organs, Lungs, and Kidneys. […] The initial presentations of GPA most often (84-100%) indicate ENT involvement, possibly lungs involvement, and occasionally kidneys involvement. […] ANCA is positive in 90% of the cases with active systemic GPA whereas, it is positive in about 60% of the cases with limited GPA. […] The main pathological findings in the GPA, namely small vessel vasculitis, granulomatous inflammation, and necrosis, refer to the GPA Pathologic Triad. […] The proper areas for biopsy in patients with suspected GPA include: ENT (especially sinuses), skin, kidneys, and lungs. […] The diagnosis of GPA can be established. […] Most Rheumatologists can only diagnose the advanced cases of systemic GPA involving the ENT, lungs and kidneys with vasculitic features including palpable purpura, hemorrhagic blister, necrotic ulcers and mono-neuritis multiplex along with ANCA positivity and the presence of SVV and granulomatous inflammation in pathology.

• #2 Granulomatosis With Polyangiitis – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK557827/

  Radiological evaluation of the sinuses, lungs, trachea, and orbits can be performed to assess the sites and extent of involvement. A chest x-ray and computed tomography (CT) scan of the lungs can be performed to identify pulmonary lesions and hemorrhage, which can help differentiate GPA from MPA. A chest x-ray is abnormal in about 89% of cases. Nodular lesions are the most common radiologic finding. […] The treatment of GPA involves the use of immunosuppressive agents in a variety of combinations. Treatment is classified into 2 phases – the induction phase and the maintenance phase. Commonly used agents are cyclophosphamide, glucocorticoids, rituximab, azathioprine, methotrexate, and plasmapheresis if indicated. […] For new-onset organ/life-threatening GPA (and MPA), induce remission with glucocorticoids and rituximab or cyclophosphamide. Rituximab is preferred for relapsing disease.

• #2 Granulomatosis With Polyangiitis | Applied Radiology

  https://appliedradiology.com/articles/granulomatosis-with-polyangiitis

  Chest CT is recommended by the American College of Rheumatology. It may show multiple pulmonary nodules (2-4 cm), air space consolidation, ground glass opacities, tracheobronchial wall thickening, and mild bronchiectasis. […] Granulomatosis with polyangiitis, while more commonly identified in adults, should be suspected in children presenting with hemoptysis, hematuria, and chest imaging showing cavitary pulmonary nodules, ground-glass opacity, and consolidation.

• #2 Granulomatosis With Polyangiitis – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK557827/

  The 2022 ACR/European Alliance of Associations for Rheumatology (EULAR) classification criteria for GPA includes the following weighted criteria: Bloody nasal discharge, nasal crusting, or sinonasal congestion (+3); Cartilaginous involvement (+2); Conductive or sensorineural hearing loss (+1); Cytoplasmic ANCA or anti-PR3 ANCA positivity (+5); Pulmonary nodules, mass, or cavitation on chest imaging (+2); Granuloma or giant cells on biopsy (+2); Inflammation or consolidation of the nasal/paranasal sinuses on imaging (+1); Pauci-immune glomerulonephritis (+1); Perinuclear ANCA or anti-MPO ANCA positivity (1); Eosinophil count more than 1109 cells/L (4). […] After excluding mimics of vasculitis, a patient diagnosed with small- or medium-vessel vasculitis could be classified as having GPA if the cumulative score is 5 or more points. When these criteria were tested in the validation dataset, the sensitivity was 93%, and the specificity was 94%.

• #2 Granulomatosis with Polyangiitis (GPA, formerly Wegener Granulomatosis) Workup: Approach Considerations, ANCA Detection, Radiography and CT Scanning

  https://emedicine.medscape.com/article/332622-workup

  Using both IF and ELISA, ANCA is detectable in nearly 100% of patients with active generalized GPA. […] The diagnosis of GPA is generally confirmed with tissue biopsy from a site of active disease, and kidney and lung biopsies are most specific for GPA. However, sampling error may occur, and histopathologic findings can be nonspecific. Tissue diagnosis may not be required if the clinical gestalt is convincing and a site for biopsy is not apparent or would be too invasive to obtain. For example, the finding of leukocytoclastic vasculitis in the setting of pulmonary nodules and PR3-ANCA may be sufficient for the diagnosis. […] Kidney biopsy may be easier to perform than lung biopsy and has a greater diagnostic yield. The typical renal lesion of GPA is segmental crescentic necrotizing glomerulonephritis with little or no immunoglobulin or complement deposition (pauci-immune). Generally, vasculitis is not observed, but this histology helps in establishing the diagnosis. However, kidney biopsy findings cannot be used to distinguish between GPA and microscopic polyarteritis. […] Although findings on a kidney biopsy are often negative for the presence of granulomas in GPA, kidney biopsy is nonetheless a very useful diagnostic tool, especially in the setting of pulmonary-renal syndrome.

• #2 The Classification & Diagnosis of Granulomatosis with Polyangiitis – Page 6 of 8 – The Rheumatologist

  https://www.the-rheumatologist.org/article/the-classification-diagnosis-of-granulomatosis-with-polyangiitis/6/?singlepage=1

  Granulomatosis with polyangiitis (GPA) is a subset of ANCA-associated vasculitis defined as a necrotizing granulomatous inflammation usually involving the respiratory tract and necrotizing vasculitis involving small and medium vessels, which is commonly associated with glomerulonephritis. […] The diagnosis of GPA is based on the patients clinical presentation and supporting laboratory and histologic findings. However, no well-established diagnostic rules or criteria exist. A positive ANCA is not required to diagnose GPA, and diseases that present similarly to a small vessel vasculitis must be excluded. […] The diagnosis of GPA is typically made using clinical judgment by evaluating a patients symptoms together with laboratory and imaging findings while excluding other possible diagnoses, and by observation of improvement on immunosuppressive therapies.

• #2 Granulomatosis with Polyangiitis initially misdiagnosed as lung cancer | Pulmonology

  https://journalpulmonology.org/en-granulomatosis-with-polyangiitis-initially-misdiagnosed-articulo-S2173511512000504

  Granulomatosis with Polyangiitis (GPA), which was formerly named Wegener’s Granulomatosis (WG) is a systemic disease characterized by necrotizing granulomatous inflammation and vasculitis that primarily involves upper and lower respiratory tract, as well as kidneys. Diagnosing GPA on the basis of transthoracic fine needle aspiration (TFNA) may be problematic, as it can be misdiagnosed as cancer. We describe a patient with a probable GPA which was originally diagnosed as malignancy, but who responded to lung cancer chemotherapy. […] Diagnosing GPA on the basis of transthoracic fine needle aspiration (TFNA) may be problematic, as it can be misdiagnosed as cancer. We describe a patient with GPA originally diagnosed as malignancy. […] A definite histological diagnosis of GPA requires coexistence of vasculitis, granuloma and necrosis although these features are only found in a minority of specimens. Only 1 or 2 of these features may be found, rendering a suggestive biopsy, in which the diagnosis is then supported by clinical criteria. Histopathologic documentation of granulomatous involvement of the respiratory tract is not explicitly required. Therefore, the diagnosis of GPA depends on a combination of clinical, pathologic, and serologic features.

• #2 Granulomatosis with Polyangiitis | Rheumatology & Clinical Research located in North Houston, Tomball, TX | Rheumatology Clinic of Houston

  https://www.houstonrheumatology.com/content/granulomatosis-with-polyangiitis

  Granulomatosis with polyangiitis (Wegeners) is a rare blood vessel disease. […] However, with prompt diagnosis, granulomatosis with polyangiitis also called GPA can be treated effectively. […] To prevent complications, prompt diagnosis and treatment are essential. […] The diagnosis of GPA is based on clinical features affecting typical organ sites together with the identification of blood vessel and tissue inflammation in those organs. […] Many people with GPA test positive for proteins in their blood called antineutrophil cytoplasmic antibodies, which is commonly called ANCA. A blood test positive for ANCA supports the need for further examination for GPA; it usually does not replace the need for a tissue biopsy. […] In rare instances where people have certain clinical features, a positive test may provide enough evidence to make a diagnosis of GPA.

• #2 Granulomatosis With Polyangiitis – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK557827/

  For maintenance of remission, rituximab is recommended. Azathioprine or methotrexate can also be considered, but their use should be reserved for patients with eGFR more than 60 mL/min/1.73m2. […] Maintenance therapy typically lasts 12 to 36 months after remission, but in high-risk patients, it may be continued indefinitely.

• #2 Granulomatosis with Polyangiitis initially misdiagnosed as lung cancer | Pulmonology

  https://journalpulmonology.org/en-granulomatosis-with-polyangiitis-initially-misdiagnosed-articulo-S2173511512000504

  In our case, although lung cancer could not be completely excluded as a possible diagnosis, complete remission of a stage IV NSCLC made this diagnosis more unlikely. […] It was only when the disease relapsed that histologic samples could be collected and the diagnosis of GPA could be established. […] GPA can emerge as a paraneoplastic syndrome, most frequently renal cell carcinoma. […] Assuming ad initium the diagnosis of GPA, the cytotoxic agents used in lung cancer chemotherapy curiously led to complete remission of lesions, as well as remission maintenance for 15 months. […] Our case describes a probable GPA in which presentation was clinically indistinguishable from lung cancer and where cytology showed, even after exhaustive review, features of undifferentiated carcinoma. It exemplifies the importance of considering alternative diagnoses, even when a particular one seems obvious.

• #3 Granulomatosis With Polyangiitis – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK557827/

  Various diagnostic criteria have been proposed to diagnose GPA and distinguish the disease from other forms of vasculitis. The ACR criteria include the following: Urinary sediment showing red blood cell casts or more than 5 red blood cells per high-power field; Abnormal findings on chest radiography; Oral ulcer or nasal discharge; Granulomatous inflammation on biopsy. The presence of 2 or more of the 4 criteria mentioned above is associated with a 92% specificity and 88% sensitivity. […] Although ANCA serology is essential for diagnosing ANCA-associated vasculitis, the diagnosis cannot rely solely on ANCA testing, as positive results may also be found in other inflammatory conditions. ANCA serology for MPO or PR3 is positive in about 90% of GPA and MPO cases, whereas it is positive in about 40% of EGPA cases. A study found that GPA is 80% positive for anti-PR3, 15% positive for anti-MPO, and 5% ANCA-negative. Anti-PR3 antibodies are about 90% sensitive for diagnosing GPA.

• #3 Granulomatosis With Polyangiitis – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK557827/

  The 2022 ACR/European Alliance of Associations for Rheumatology (EULAR) classification criteria for GPA includes the following weighted criteria: Bloody nasal discharge, nasal crusting, or sinonasal congestion (+3); Cartilaginous involvement (+2); Conductive or sensorineural hearing loss (+1); Cytoplasmic ANCA or anti-PR3 ANCA positivity (+5); Pulmonary nodules, mass, or cavitation on chest imaging (+2); Granuloma or giant cells on biopsy (+2); Inflammation or consolidation of the nasal/paranasal sinuses on imaging (+1); Pauci-immune glomerulonephritis (+1); Perinuclear ANCA or anti-MPO ANCA positivity (1); Eosinophil count more than 1109 cells/L (4). […] After excluding mimics of vasculitis, a patient diagnosed with small- or medium-vessel vasculitis could be classified as having GPA if the cumulative score is 5 or more points. When these criteria were tested in the validation dataset, the sensitivity was 93%, and the specificity was 94%.

• #3 Granulomatosis with Polyangiitis initially misdiagnosed as lung cancer | Pulmonology

  https://journalpulmonology.org/en-granulomatosis-with-polyangiitis-initially-misdiagnosed-articulo-S2173511512000504

  In our case, although lung cancer could not be completely excluded as a possible diagnosis, complete remission of a stage IV NSCLC made this diagnosis more unlikely. […] It was only when the disease relapsed that histologic samples could be collected and the diagnosis of GPA could be established. […] GPA can emerge as a paraneoplastic syndrome, most frequently renal cell carcinoma. […] Assuming ad initium the diagnosis of GPA, the cytotoxic agents used in lung cancer chemotherapy curiously led to complete remission of lesions, as well as remission maintenance for 15 months. […] Our case describes a probable GPA in which presentation was clinically indistinguishable from lung cancer and where cytology showed, even after exhaustive review, features of undifferentiated carcinoma. It exemplifies the importance of considering alternative diagnoses, even when a particular one seems obvious.

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