scholarly journals A Case of Granulomatosis with Polyangiitis with Various Breast Lesions as the Initial Symptoms: A Case-Based Review

2021 ◽  
Vol 2021 ◽  
pp. 1-6
Author(s):  
Masatoshi Kawataka ◽  
Toshiki Kido ◽  
Reina Tsuda ◽  
Takafumi Onose ◽  
Ryoko Asano ◽  
...  
Keyword(s):  
Granulomatosis With Polyangiitis ◽  
Antineutrophil Cytoplasmic Antibodies ◽  
Clinical Feature ◽  
Breast Lesions ◽  
Granulomatous Vasculitis ◽  
Typical Clinical Feature ◽  
Initial Symptoms ◽  
Breast Nodule ◽  
Cutaneous Granuloma ◽  
Case Based

A 44-year-old woman presenting with pus-like discharge from the nipples visited our hospital for scleritis. Subcutaneous induration and ulceration were found on her breast. She was diagnosed with granulomatosis with polyangiitis (GPA) considering scleritis, sinusitis, cutaneous granuloma formation, and antiproteinase 3-antineutrophil cytoplasmic antibodies and was successfully treated with glucocorticoids. Fifteen months later, she developed pulmonary consolidation and a right breast nodule. Biopsies of the breast nodule showed granulomatous vasculitis, and she was treated with rituximab. While breast involvement in GPA is rare, unilateral breast mass is a typical clinical feature; thus, GPA should be considered in such cases.

scholarly journals Eosinophilic Cholecystitis Associated with Eosinophilic Granulomatosis with Polyangiitis

2020 ◽  
Vol 14 (3) ◽  
pp. 668-674
Author(s):  
Hiroyuki Ito ◽  
Yusuke Mishima ◽  
Tsubomi Cho ◽  
Naoki Ogiwara ◽  
Yoshimasa Shinma ◽  
...  
Keyword(s):  
Cerebral Hemorrhage ◽  
Granulomatosis With Polyangiitis ◽  
Epigastric Pain ◽  
White Blood Cells ◽  
Gallbladder Wall ◽  
Antineutrophil Cytoplasmic Antibodies ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Test Results ◽  
Laboratory Investigations ◽  
Eosinophilic Granulomatosis

We report a case of eosinophilic cholecystitis associated with eosinophilic granulomatosis with polyangiitis (EGPA) complicated by cerebral hemorrhage. A 60-year-old man presented to a local hospital with a diagnosis of acute cholecystitis, with persistent fever and epigastric pain for 2 weeks. His symptoms persisted despite 3-week hospitalization; therefore, he was transferred to our hospital for further evaluation. Laboratory investigations upon admission showed white blood cells 26,300/µL and significant eosinophilia (eosinophils 61%). Abdominal computed tomography revealed no gallbladder enlargement but a circumferentially edematous gallbladder wall. Additional blood test results were negative for antineutrophil cytoplasmic and perinuclear antineutrophil cytoplasmic antibodies; however, immunoglobulin (Ig)G and IgE levels were high at 1,953 mg/dL and 3,040/IU/mL, respectively. He improved following endoscopic transnasal gallbladder drainage for cholecystitis and was diagnosed with EGPA and received corticosteroid and immunosuppressant combination therapy. The eosinophil count decreased immediately after treatment, and abdominal pain and numbness resolved. He returned with left-sided suboccipital hemorrhage likely attributed to EGPA 6 months after discharge. EGPA is characterized by inflammation of small blood vessels and clinically manifests with an allergic presentation of bronchial asthma, as well as renal dysfunction, interstitial pneumonia, enteritis, and cerebral hemorrhage. Few reports have described cholecystitis as a presenting symptom of EGPA. We report a rare case of such a presentation with added considerations.

scholarly journals Efficacy and safety of rituximab in the treatment of eosinophilic granulomatosis with polyangiitis

RMD Open ◽  
2019 ◽  
Vol 5 (1) ◽  
pp. e000905 ◽  
Author(s):  
Vítor Teixeira ◽  
Aladdin J Mohammad ◽  
Rachel B Jones ◽  
Rona Smith ◽  
David Jayne
Keyword(s):  
Partial Response ◽  
Granulomatosis With Polyangiitis ◽  
Antineutrophil Cytoplasmic Antibodies ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Free Survival ◽  
Prednisolone Dose ◽  
Tertiary Centre ◽  
Limited Experience ◽  
Treatment Responses ◽  
Eosinophilic Granulomatosis

IntroductionEosinophilic granulomatosis with polyangiitis (EGPA) is a subset of antineutrophil cytoplasmic antibodies (ANCA) associated vasculitis with distinct pathophysiological mechanisms, clinical features and treatment responses. Rituximab is a licensed therapy for granulomatosis with polyangiitis and microscopic polyangiitis but there is limited experience of rituximab in EGPA.MethodsEGPA patients from a tertiary centre who received rituximab for mostly refractory EGPA or in whom cyclophosphamide was contra indicated were studied. A standardised dataset was collected at time of initial treatment and every 3 months for 24 months. Response was defined as a Birmingham Vasculitis Activity Score (BVAS) of 0 and partial response as ≥50% reduction in BVAS from baseline. Remission was defined as a BVAS of 0 on prednisolone dose ≤5 mg.ResultsSixty-nine patients (44 female) received rituximab between 2003 and 2017. Improvement (response and partial response) was observed in 76.8% of patients at 6 months, 82.8% at 12 months and in 93.2% by 24 months, while relapses occurred in 54% by 24 months, with asthma being the most frequent manifestation. The median BVAS decreased from 6 at baseline to 1 at 6 months, and 0 at 12 and 24 months. Prednisolone dose (mg/day, median) decreased from 12.5 to 7, 7.5 and 5 at 6, 12 and 24 months, respectively. ANCA positive patients had a longer asthma/ear, nose and throat (ENT) relapse-free survival time and a shorter time to remission.DiscussionRituximab demonstrated some efficacy in EGPA and led to a reduction in prednisolone requirement, but asthma and ENT relapse rates were high despite continued treatment. The ANCA positive subset appeared to have a more sustained response on isolated asthma/ENT exacerbations.

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.

scholarly journals Non-infectious aortic and mitral valve vegetations in a patient with eosinophilic granulomatosis with polyangiitis

2019 ◽  
Vol 12 (5) ◽  
pp. e225947 ◽  
Author(s):  
Kamalesh Karthikeyan ◽  
Sudarshan Balla ◽  
Martin A Alpert
Keyword(s):  
Mitral Valve ◽  
Granulomatosis With Polyangiitis ◽  
Antineutrophil Cytoplasmic Antibodies ◽  
Cardiac Complications ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Rare Form ◽  
Coronary Arteritis ◽  
Endocardial Fibrosis ◽  
Arteries And Veins ◽  
Eosinophilic Granulomatosis

Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare form of vasculitis characterised by atopic manifestations, inflammation of small-sized to medium-sized arteries and veins, hypereosinophilia and tissue infiltration with eosinophils. Cardiac complications occur most commonly in the absence of antineutrophil cytoplasmic antibodies. Cardiac complications include coronary arteritis (rare), pericarditis, myocarditis, endocardial fibrosis (Loeffler’s endocarditis) and intracavitary thrombosis of the left or right ventricle. This is the first reported case of large non-infectious valvular vegetations associated with EGPA.

Urethral involvement in granulomatosis with polyangiitis: a case-based review

2019 ◽  
Vol 39 (7) ◽  
pp. 1279-1284
Author(s):  
Hamdy M. A. Ahmed ◽  
Mohanad M. Elfishawi ◽  
Ahmed Hagiga ◽  
Ibrahim M. A. Ahmed ◽  
Ya Li Chen
Keyword(s):  
Granulomatosis With Polyangiitis ◽  
Case Based

scholarly journals Echocardiogram: The GPS to GPA’s Heart (Granulomatosis with Polyangiitis)

2019 ◽  
Vol 2019 ◽  
pp. 1-3
Author(s):  
Manpreet K. Parmar ◽  
Mariam Alikhan ◽  
Vivien M. Hsu ◽  
Amanda Borham
Keyword(s):  
Cardiac Involvement ◽  
Granulomatosis With Polyangiitis ◽  
Case Reports ◽  
Disease Process ◽  
Cardiac Complications ◽  
Primary Role ◽  
Helpful Tool ◽  
Coronary Arteritis ◽  
Granulomatous Vasculitis ◽  
Lower Airways

Granulomatosis with polyangiitis (GPA) is a rare ANCA-associated necrotizing granulomatous vasculitis affecting small- to medium-sized vessels. Common manifestations of this disease process affect the ear, nose, throat, upper and lower airways, and kidneys. Cardiac involvement has been reported in 6–44% of patients, primarily as coronary arteritis and pericarditis. A majority of case reports of pericardial effusions in patients with GPA identify patients having constrictive pericarditis secondary to uremia. We are presenting a case of hemorrhagic pericarditis in a patient with GPA in which the underlying inflammatory vasculitis likely played the primary role in the patient’s presentation. Echocardiographic abnormalities have been found in 80% of patients with GPA. Given the high mortality from cardiac involvement in patients with GPA, screening echocardiograms for this patient population may serve as a helpful tool in gauging disease severity, thereby guiding therapy to prevent serious cardiac complications, such as cardiac tamponade as presented in this case report.

scholarly journals P1756ANTI-ABO ANTIBODY- VERSUS ANTI-HLA ANTIBODY-INDUCED ANTIBODY MEDIATED REJECTION IN ABO-INCOMPATIBLE KIDNEY TRANSPLANT PATIENTS: RELATIVE INCIDENCE AND PHENOTYPE DIFFERENCE

2020 ◽  
Vol 35 (Supplement_3) ◽  
Author(s):  
Jinmin Kong ◽  
Hyukyong Kwon ◽  
Sung Son ◽  
EUN Whang
Keyword(s):  
Kidney Transplant ◽  
Blood Group ◽  
Relative Frequency ◽  
Response To Treatment ◽  
Clinical Feature ◽  
Antibody Mediated Rejection ◽  
Transplant Patients ◽  
Kidney Transplant Patients ◽  
Hla Antibody ◽  
Case Based

Abstract Background and Aims Antibody-mediated rejection(AMR) in ABO incompatible(ABOi) kidney transplant(KT) patients can either be due to donor-specific anti-HLA antibody(DSA) or anti-blood group antibody. The relative frequency and possible phenotype difference of these two types of AMR in ABOi KT patients have not been reported. Method Of 111 ABOi KT patients between 2007 and 2019 in our center, 15(13.5%) patients developed acute AMR diagnosed by indication biopsy. Since there is no histologic distinction between DSA- and anti-ABO-induced AMR, we assumed the causative antibody in each case based on anti-ABO level and DSA, measured in serum collected at the time of AMR. Results Of these 15 cases of acute AMR, 5 were attributable to anti-ABO(ABO-AMR) since anti-ABO titer was higher (≥16) and DSA was undetectable at the time of rejection. Five cases were attributable to DSA(DSA-AMR) since DSA was detectable and anti-ABO was lower during rejection. Another 3 cases with lower anti-ABO titer and undetectable DSA were also assumed to be DSA-induced, since this low level of anti-ABO is unlikely to cause rejection and DSA can be undetectable in DSA-induced AMR by adsorption of Ab on graft, as frequently seen in ABO-compatible patients Two cases with both higher anti-ABO titer and detectable DSA was regarded as undetermined. The onset of acute AMR was within 2 weeks in all cases(median 7.0 days) and comparable between DSA- and ABO-AMR. Initial anti-ABO titer was also not statistically different; median(range) 256(64-4096) in ABO-AMR and 64(16-256) in DSA-AMR. All the 5 patients with ABO-AMR had negative PRA before KT, whereas 5 of 8 patients with DSA-AMR had positive PRA before KT, and two DSA-AMR patients had preformed DSA before KT. There was no difference in peak creatinine and response to treatment. All the AMR were recovered by treatment and no graft was lost to rejection. No patient with ABO-AMR developed chronic AMR whereas one of DSA-AMR patients developed chronic AMR. Conclusion In summary, among 15 cases of acute AMR, 5 were ABO-AMR, 8 were DSA-AMR and 2 were undetermined. There was no difference in clinical feature between DSA- and ABO-AMR. No patient with ABO-AMR developed chronic AMR.

scholarly journals Granulomatosis with Polyangiitis Presenting as Pyrexia of Unknown Origin, Leukocytosis, and Microangiopathic Haemolytic Anemia

2017 ◽  
Vol 2017 ◽  
pp. 1-6
Author(s):  
Sima Terebelo ◽  
Iona Chen
Keyword(s):  
Granulomatosis With Polyangiitis ◽  
Immunosuppressive Treatment ◽  
Kidney Injury ◽  
Unknown Origin ◽  
Antineutrophil Cytoplasmic Antibodies ◽  
High Dose ◽  
Normal Range ◽  
Pyrexia Of Unknown Origin ◽  
History Of ◽  
Haemolytic Anemia

A 66-year-old woman presented to the Emergency Department with a florid sepsis-like picture, a two-week history of fever, relative hypotension with end organ ischemia (unexplained liver enzyme and troponin elevations), and nonspecific constitutional symptoms. She was initially found to have a urinary tract infection but, despite appropriate treatment, her fever persisted and her white blood cell count continued to rise. During her hospitalization the patient manifested leukocytosis to 47,000 WBC/μL, ESR 67 mm/hr (normal range 0–42 mm/hr), CRP 17.5 mg/dL (normal range 0.02–1.20 mg/dL), and microangiopathic haemolytic anemia, with declining haemoglobin and haematocrit. An infectious aetiology was not found despite extensive bacteriologic studies and radiographic imaging. The patient progressed to acute kidney injury with “active” urinary sediment and proteinuria. Kidney biopsy results and serological titres of myeloperoxidase positive perinuclear-antineutrophil cytoplasmic antibodies (MPO+ p-ANCA) led to a diagnosis of granulomatosis with polyangiitis. Immunosuppressive treatment with high dose methylprednisolone and rituximab led to resolution of the leukocytosis and return of the haemoglobin and haematocrit values toward normal without further signs of hemolysis.

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