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https://www.readbyqxmd.com/read/28637076/-clinical-course-of-an-early-childhood-onset-granulomatosis-with-polyangiitis
#1
Marco Krasselt, Matthias Pierer, Kerstin Amann, Anette Bachmann, Tom H Lindner
History A 26-year-old male patient presented with an eight-week history of unspecific symptoms such as weight loss and fever. Besides, he also suffered from haemoptysis, cough, and arthralgia. Since the age of twelve years, the patient has been treated for Wegner's granulomatosis. At the age of 20 years he received a kidney transplant which failed only four years later. Investigations The relapse we clinically suspected was confirmed by CT scan showing bilateral pulmonary manifestations. Moreover, we found highly positive antibodies against proteinase 3...
June 2017: Deutsche Medizinische Wochenschrift
https://www.readbyqxmd.com/read/28631702/-the-frequency-of-ophthalmologic-manifestations-of-granulomatosis-with-polyangiitis-wegener-s-and-their-relationship-to-systemic-diseases
#2
D S Ismailova, P I Novikov, Ya O Grusha, Yu V Abramova, N M Bulanov, E A Makarov
AIM: To estimate the frequency of lesions in the organ of vision in granulomatosis with polyangiitis (GPA) (Wegener's) and to determine their relationship to systemic diseases. SUBJECTS AND METHODS: The retrospective study enrolled 218 patients followed up at the E.M. Tareyev Clinic of Nephrology, Internal and Occupational Diseases, with a diagnosis of GPA. The frequency and association of ophthalmic manifestations with systemic involvement were statistically analyzed using PASW Statistics 18...
2017: Terapevticheskiĭ Arkhiv
https://www.readbyqxmd.com/read/28628344/rituximab-induction-and-maintenance-treatment-in-patients-with-scleritis-and-granulomatosis-with-polyangiitis-wegener-s
#3
Caiyun You, Lina Ma, Andres F Lasave, C Stephen Foster
AIMS: To evaluate the efficacy and safety of rituximab (RTX) induction and maintenance treatment for patients with scleritis and granulomatosis with polyangiitis (GPA), Wegener's. METHODS: Nine patients (12 eyes) with scleritis with GPA who did not respond to corticosteroids and more than one immunosuppressive agent who received ongoing maintenance RTX treatment were identified. Demographics and outcome measures were recorded. RESULTS: Median follow-up time of 30 months (range, 15 to 87 months)...
June 19, 2017: Ocular Immunology and Inflammation
https://www.readbyqxmd.com/read/28623488/vasculitis-for-the-internist-focus-on-anca-associated-vasculitis
#4
REVIEW
Benjamin Chaigne, Loïc Guillevin
Vasculitides comprise several diseases affecting vessels of different sizes, mainly arteries, but also capillaries and veins. Antineutrophil cytoplasm antibody-associated vasculitides (AAVs) belong to the systemic necrotizing small-vessel vasculitis group that comprises granulomatosis with polyangiitis, microscopic polyangiitis and eosinophilic granulomatosis with polyangiitis. Internists often see AAV patients, and although their outcomes are usually good, the disease can be responsible for morbidity and mortality...
June 16, 2017: Internal and Emergency Medicine
https://www.readbyqxmd.com/read/28619741/saddle-nose-and-bilateral-cauliflower-ear-deformities-with-pyoderma-gangrenosum-like-ulcers-cavitary-pulmonary-lesions-digital-gangrene-and-pulselessness-in-a-young-female
#5
Sweta Subhadarshani, Vishal Gupta, Anurag Chahal, Kaushal K Verma
We report a young female who presented with saddle-nose and bilateral cauliflower ear deformities along with pyoderma gangrenosum-like ulcers, digital gangrene and pulselessness. Subsequently, she was found to have bilateral conductive hearing loss, a corneal opacity, mild aortic regurgitation and radiological evidence of cavitary changes in lungs and aortoarteritis. Our patient had a constellation of symptoms which posed a diagnostic challenge. Finally, a diagnosis of relapsing polychondritis with several unusual features was made...
June 15, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28616380/rare-case-of-pulmonary-histoplasmosis-complicated-with-bronchocentric-granulomatosis-in-a-non-endemic-area
#6
Evanthia Botsa, Ioanna Thanou, Stamatina Kabanarou, Loukas Thanos
BACKGROUND: Pulmonary histoplasmosis is a fungal infection caused by histoplasma capsulatum, rarely diagnosed in non endemic areas and/or immunocompromised patients. Complication of pulmonary histoplasmosis with bronchocentric granulomatosis is extremely rare. CASE REPORT: A 48-year-old man with prolonged fever and nausea was admitted to our hospital. Clinical examination revealed pathological auscultatory sounds to the left lung. Computed tomography was performed and revealed a large solid mass of the left upper lobe, limited pleural and pericardial effusion and calcified lymphadenopathy of mediastinum...
2017: Respiratory Medicine Case Reports
https://www.readbyqxmd.com/read/28615072/chemokine-receptor-co-expression-reveals-aberrantly-distributed-th-effector-memory-cells-in-gpa-patients
#7
Lucas L Lintermans, Abraham Rutgers, Coen A Stegeman, Peter Heeringa, Wayel H Abdulahad
BACKGROUND: Persistent expansion of circulating CD4(+) effector memory T cells (TEM) in patients with granulomatosis with polyangiitis (GPA) suggests their fundamental role in disease pathogenesis. Recent studies have shown that distinct functional CD4(+) TEM cell subsets can be identified based on expression patterns of chemokine receptors. The current study aimed to determine different CD4(+) TEM cell subsets based on chemokine receptor expression in peripheral blood of GPA patients...
June 14, 2017: Arthritis Research & Therapy
https://www.readbyqxmd.com/read/28611282/the-4-plus-2-rituximab-protocol-makes-maintenance-treatment-unneeded-in-patients-with-refractory-anca-associated-vasculitis-a-10-year-observation-study
#8
Dario Roccatello, Savino Sciascia, Daniela Rossi, Mirella Alpa, Carla Naretto, Massimo Radin, Roberta Fenoglio, Simone Baldovino, Elisa Menegatti
BACKGROUND: ANCA associated vasculitides (AAV) often present with a chronic relapsing course. Relapse leads to increased immunosuppressive exposure and consequent toxicity. While two randomized controlled trials have shown rituximab (RTX) to be the most effective induction treatment in patients with relapsing disease, the optimal treatment duration and RTX dose remain debated. Whether to administer a maintenance dose to every patient, at a fixed time interval or on the basis of B cell count and ANCA titre or only when disease manifestations do occur is still debated as well...
May 23, 2017: Oncotarget
https://www.readbyqxmd.com/read/28609772/lymphoproliferative-disorders-of-the-lung
#9
Raphaël Borie, Marie Wislez, Martine Antoine, Jacques Cadranel
This review aims to describe some of the most frequent lymphoproliferative disorders arising from the lung: pulmonary mucosa-associated lymphoid tissue (MALT) lymphoma, lymphomatoid granulomatosis (LG), multicentric Castleman disease (MCD), primary effusion lymphoma (PEL), and nodular lymphoid hyperplasia (NLH). Primary pulmonary lymphoma is defined as a clonal lymphoproliferative disorder affecting one or both lungs, without extrapulmonary involvement 3 months after diagnosis, and includes pulmonary MALT lymphoma and LG...
June 14, 2017: Respiration; International Review of Thoracic Diseases
https://www.readbyqxmd.com/read/28608042/-granulomatous-reaction-pattern-of-the-skin-interstitial-granulomatous-dermatitis-lymphoma-vasculitis
#10
REVIEW
C Rose, K Holl-Ulrich
A particular diagnostic situation in the classification of a granulomatous dermatitis results when no circumscribed granulomas but instead a diffuse interstitial histiocytic inflammatory infiltrate, a granulomatous vasculitis or a neoplastic lymphocytic infiltrate is found. Interstitial granulomatous dermatitis was originally described in patients with arthritis. Later, it was recognized that there are also associations with other usually immunological diseases. Differentiating between interstitial granulomatous dermatitis and the interstitial form of granuloma annulare, early morphea and variants of borreliosis or scleromyxedema as well as interstitial granulomatous drug reaction can be very difficult...
June 12, 2017: Der Hautarzt; Zeitschrift Für Dermatologie, Venerologie, und Verwandte Gebiete
https://www.readbyqxmd.com/read/28605750/anticentromere-antibody-positive-ackerman-s-syndrome-with-granulomatous-anterior-uveitis
#11
Susana Rilhas Fernandes, Vera Las, Joana Borges, Cândida Silva, Augusto Faustino
Ackerman's Syndrome or Intersticial Granulomatous Dermatitis with Arthritis has been an issue of increasing number of reports in the last decade which had focused its heterogeneous cutaneous and rheumatologic expression besides the initial manifestations reported by Ackerman and his group. Granulomatosis anterior uveitis has not been previously described. Some patients are reported to have positive autoantibodies but association with anticentromere antibodies has not been previously described as well, to our knowledge...
June 12, 2017: Acta Reumatológica Portuguesa
https://www.readbyqxmd.com/read/28601424/a-diagnostic-approach-to-recurrent-orofacial-swelling-a-retrospective-study-of-104-patients
#12
Rachel Y Miest, Alison J Bruce, Nneka I Comfere, Elena Hadjicharalambous, Dawnielle Endly, Christine M Lohse, Roy S Rogers
OBJECTIVE: To identify patients evaluated in an outpatient setting at our institution with a presentation of recurrent orofacial swelling and to review the spectrum of causes to outline a diagnostic approach. PATIENTS AND METHODS: A retrospective study of 104 patients with more than 1 episode of orofacial swelling lasting for more than 5 days identified through a keyword search of the electronic health record from January 2, 2000, through July 5, 2011. RESULTS: Patients were categorized according to final cause of orofacial swelling: idiopathic orofacial granulomatosis, solid facial edema due to rosacea and acne vulgaris, Crohn disease, contact dermatitis, sarcoidosis, exfoliative cheilitis, lichen planus, actinic cheilitis, cheilitis glandularis, lymphedema, miscellaneous, and multifactorial...
June 7, 2017: Mayo Clinic Proceedings
https://www.readbyqxmd.com/read/28599871/a-case-report-of-granulomatosis-with-polyangiitis-with-granulomas-in-bone-marrow
#13
Isabel de la Cámara Fernández, Fernando Lozano Morillo, Luis Morillas López, Mario Martínez López
No abstract text is available yet for this article.
June 6, 2017: Reumatología Clinica
https://www.readbyqxmd.com/read/28598870/molecularly-targeted-agents-in-oculoplastic-surgery
#14
Richard C Allen
PURPOSE OF REVIEW: Significant advances have been made in oncology and rheumatology with the introduction of molecularly targeted agents (MTAs). MTAs consist of monoclonal antibodies and small molecule inhibitors. The purpose of this manuscript is to review the recent applications of MTAs to orbital, lacrimal, and eyelid disease. RECENT FINDINGS: The use of monoclonal antibodies has been described in the treatment of orbital vascular lesions, lymphoma, and squamous cell carcinoma...
June 8, 2017: Current Opinion in Ophthalmology
https://www.readbyqxmd.com/read/28598778/uncommon-presentation-of-granulomatosis-with-polyangiitis-in-association-with-subclinical-coeliac-disease
#15
Paraskevi V Voulgari, Fotini Apostolou, Evangelia Dounousi, George Liapis, Moises Elisaf, Alexandros A Drosos
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June 9, 2017: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/28592713/pulmonary-limited-granulomatosis-with-polyangiitis-coexisting-with-mixed-connective-tissue-disease
#16
Masaru Ando, Akihiko Goto, Mari Yamasue, Yuko Usagawa, Hiroaki Oka, Takehiko Shigenaga, Jun-Ichi Kadota
Granulomatosis with polyangiitis (GPA) is a systemic disease characterized by necrotizing, granulomatous vasculitis of the upper and lower respiratory tracts and glomerulonephritis, and is classified as a classical or limited form. The classical form of GPA demonstrates the involvement of the upper respiratory tract, sinuses, lungs and kidneys, whereas the limited form is characterized by the lack of the renal involvement with female predominance. On the other hand, mixed connective tissue disease (MCTD) shows the clinical and laboratorial features of systemic lupus erythematosus, systemic sclerosis and polymyositis, along with high titers of anti-ribonucleoprotein antibodies and is characterized by good response to corticosteroid therapy and favorable prognosis...
2017: Tohoku Journal of Experimental Medicine
https://www.readbyqxmd.com/read/28591497/-anca-associated-vasculitides-at-mexico-city-s-metropolitan-eastern-area
#17
Hernando Cervera-Castillo, Francisco Blanco-Favela, Yolanda Floriberta Silva-López, Guadalupe Eugenia Paredez-Rivera, Verónica Torres-Caballero
BACKGROUND: The anti-neutrophil cytoplasmic antibody-associated (ANCA) vasculitides includes granulomatosis with polyangiitis (Wegener's) (GPW), Chrug-Strauss syndrome (CSS) and microscopic polyangiitis (MPA). Since it has low incidence in our field, there are a few published papers. The aim of this study was to report the clinical characteristics, activity and damage of these vasculitides in Mexico City's metropolitan eastern area. METHODS: We studied 28 cases with ANCA-associated vasculitis in the aforementioned area...
July 2017: Revista Médica del Instituto Mexicano del Seguro Social
https://www.readbyqxmd.com/read/28591026/imaging-diagnosis-of-orbital-wegener-granulomatosis-a-rare-case-report
#18
Bin Yang, Zhijian Yin, Shuai Chen, Feng Yuan, Wei Zhao, Yaying Yang
INTRODUCTION: Wegener granulomatosis (WG) is a rare idiopathic autoimmune disease causing necrotizing granulomatous vasculitis. Whether as the first symptom or as part of systemic changes, ocular manifestations in WG patients are not specific. Any part of the eyes can be affected, with the anterior segment and orbit most commonly involved. So, early diagnosis and treatment are essential for controlling the progression of the disease and improving the quality of life for patients. CLINICAL FINDINGS/PATIENT CONCERNS: Here we present a rare case of orbital WG of a 22-year-old woman was admitted to the hospital because of intense pain associated with decreased visual acuity in her right eye since 1 day...
June 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28588807/eosinophilic-angiitis-presenting-with-a-true-fusiform-temporal-artery-aneurysm
#19
Megan MacDiarmid, Dmitri Nepogodiev, Alok Tiwari, Martin Duddy, Martyn Carey, Paresh Jobanputra
Temporal artery aneurysm is a rare cause of temporal artery swelling in the absence of preceding trauma. Vasculitis other than giant cell arteritis, such as eosinophilic granulomatosis with polyangiitis, should be considered in such cases and a careful assessment of other medium-sized arteries undertaken.
June 2017: Clinical Case Reports
https://www.readbyqxmd.com/read/28588684/successful-treatment-of-granulomatosis-with-polyangiitis-with-hydropneumothorax-using-corticosteroids-and-immunosuppressant
#20
Xu-Hua Shi, Yong-Feng Zhang, Yue-Wu Lu
Pneumothorax and pleural effusion is a rare and serious complication of granulomatosis with polyangiitis (GPA). The present study reported a case with a history of sinusitis for 20 years, dry cough for three years and exacerbated purulent nasal discharge and recurrent skin ulcers for two years. The patient experienced sudden difficulty in breathing two months prior to presentation. Lung computed tomography (CT) showed multiple bilateral lung nodules and cavitary nodules as well as right hydropneumothorax. Paranasal sinus CT showed soft tissue infiltration...
June 2017: Experimental and Therapeutic Medicine
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