The Use of Integra® Dermal Regeneration Template for the Orbital Exenteration Socket: A Novel Technique
Conclusions: Integra® dermal regeneration template can be used for the immediate reconstruction of the socket following exenteration. It is easy to use, and provides a short healing time without any need for any additional reconstructive procedures. (Source: Ophthalmic Plastic and Reconstructive Surgery)
Source: Ophthalmic Plastic and Reconstructive Surgery - January 1, 2018 Category: Opthalmology Tags: Original Investigations Source Type: research

A case of squamous cell carcinoma of the nasal cavity in a patient with granulomatosis with polyangiitis (Wegener granulomatosis).
We report a rare case of squamous cell carcinoma (SCC) of the nasal cavity arising in a patient with granulomatosis with polyangiitis (GPA). The patient was a 35-year-old man who had been diagnosed 15 years earlier with GPA and treated medically for sinonasal, pulmonary, and renal involvement. He presented to us with left-sided orbital and cheek pain and nasal obstruction. Endoscopy detected a friable, exophytic mass that involved the left lateral nasal wall and septum. Biopsy analysis identified the mass as an SCC. A definitive endoscopic resection was performed, followed by chemoradiation, but the patient exhibited progr...
Source: Ear, Nose and Throat Journal - January 1, 2018 Category: ENT & OMF Authors: Kuan EC, Peng KA, Gonzalez LO, Sercarz JA Tags: Ear Nose Throat J Source Type: research

WEGENER's GRANULOMATOSIS ‐ THE CHALLENGES IN ITS REVELATION AND TRIBULATION ON TREATMENT
(Source: Respirology)
Source: Respirology - November 23, 2017 Category: Respiratory Medicine Tags: APSR Poster Abstracts Source Type: research

The Epidemiology of Antineutrophil Cytoplasmic Autoantibody –Associated Vasculitis in Olmsted County, Minnesota
ConclusionThe annual incidence of AAV in Olmsted County, Minnesota over the 20 years of the study was 3.3 per 100,000, with a prevalence of 42.1 per 100,000, which is substantially higher than the rates reported in other areas worldwide. The incidence of GPA was similar to that of MPA. Patients with MPA and those with EGPA, but not patients with GPA, experienced higher rates of mortality than that in the Minnesota general population. MPO‐ANCAs were a marker of poor survival in this population of patients with AAV. (Source: Arthritis and Rheumatism)
Source: Arthritis and Rheumatism - November 9, 2017 Category: Rheumatology Authors: Alvise Berti, Divi Cornec, Cynthia S. Crowson, Ulrich Specks, Eric L. Matteson Tags: Original Article Source Type: research

Seronegative granulomatosis with polyangiitis presenting with multiple cranial nerve palsies
Granulomatosis with polyangiitis (GPA), previously known as Wegener’s granulomatosis (WG), is a rare systemic disease characterized by necrotizing granulomas and vasculitis that usually presents in the respiratory tract and renal system. Here, we report a case of anti‐neutrophil cytoplasmic antibody (ANCA)‐negative GPA with diagnostic difficulties. Neurologic involvement in GPA is not uncommon, with up to 50% of patients manifesting with neurologic defects; however, it is usually a late event. This patient was a 50‐year‐old man whose first manifestation was multiple neurologic defects without respiratory or renal...
Source: Neuropathology - October 1, 2017 Category: Neurology Authors: Eunhye Lee, Jynkyun Park, Seung Hong Choi, Sung ‐Hye Park Tags: Case Report Source Type: research

[Scleritis and episcleritis].
Authors: Héron E, Bourcier T Abstract Episcleritis is generally a benign disease with a self-limited course, while scleritis is a severe ocular condition due to a risk of impaired vision in one-fifth of patients and its association with systemic diseases in one third of them. Infectious scleritis, representing 8 % of the etiologies, is mainly of herpetic origin (varicella zoster and herpes simplex viruses). A systemic autoimmune disease is observed in roughly 30 % of scleritis patients: inflammatory rheumatisms (15 %), firstly rheumatoid polyarthritis, systemic vasculitides (8 %), mainly granulomatosi...
Source: Journal Francais d Ophtalmologie - September 18, 2017 Category: Opthalmology Tags: J Fr Ophtalmol Source Type: research

ANCA Glomerulonephritis and Vasculitis.
Abstract ANCA vasculitis has an associated autoimmune response that produces ANCAs that induce distinct pathologic lesions. Pauci-immune necrotizing and crescentic GN is a frequent component of ANCA vasculitis. ANCA vasculitis is associated with ANCA specific for myeloperoxidase (MPO-ANCA) or proteinase 3 (PR3-ANCA). A diagnosis of ANCA vasculitis should always specify the serotype as MPO-ANCA positive, PR3-ANCA positive, or ANCA-negative. To fully characterize a patient, the serotype also should be accompanied by the clinicopathologic variant if this can be determined: microscopic polyangiitis, granulomat...
Source: Clinical Journal of the American Society of Nephrology : CJASN - August 25, 2017 Category: Urology & Nephrology Authors: Jennette JC, Nachman PH Tags: Clin J Am Soc Nephrol Source Type: research

The role of biopsy in lacrimal gland inflammation: A clinicopathologic study.
CONCLUSIONS: We recommend that in patients presenting with lacrimal gland inflammation (i.e., erythema, edema, tenderness) in which the specific diagnosis cannot be made clinically and on imaging, biopsy is warranted for accurate diagnosis and appropriate treatment. We found that the majority of these patients (61.7%) had specific histopathology, and 38% had systemic diseases. PMID: 28816552 [PubMed - as supplied by publisher] (Source: Orbit)
Source: Orbit - August 17, 2017 Category: Opthalmology Authors: Luemsamran P, Rootman J, White VA, Nassiri N, Heran MKS Tags: Orbit Source Type: research

Peripheral Ulcerative Keratitis Associated with Autoimmune Disease: Pathogenesis and Treatment.
Authors: Cao Y, Zhang W, Wu J, Zhang H, Zhou H Abstract Peripheral ulcerative keratitis (PUK) is type of crescent-shaped inflammatory damage that occurs in the limbal region of the cornea. PUK is always combined with an epithelial defect and the destruction of the peripheral corneal stroma. PUK may have a connection to systemic conditions, such as long-standing rheumatoid arthritis (RA), systemic lupus erythematosus (SLE), Wegener granulomatosis (WG), relapsing polychondritis, classic polyarteritis nodosa and its variants, microscopic polyangiitis, and Churg-Strauss syndrome. However, the most common conne...
Source: Journal of Ophthalmology - August 10, 2017 Category: Opthalmology Tags: J Ophthalmol Source Type: research

Saddle nose deformity and septal perforation in granulomatosis with polyangiitis
ConclusionsThis study systematically reviews the efficacy and safety of surgical reconstruction of external nasal deformities in GPA patients for the first time. Saddle nose reconstruction in GPA patients with minimal or no local disease is a safe procedure despite an increased rate of revision surgery. Further research is required regarding the impact of antibiotic prophylaxis, immune‐modulating therapy, long‐term outcomes, and functional outcomes measured with subjective and objective parameters.This article is protected by copyright. All rights reserved. (Source: Clinical Otolaryngology)
Source: Clinical Otolaryngology - August 1, 2017 Category: ENT & OMF Authors: Annekatrin Coordes, Sonja Maike Loose, Veit M. Hofmann, Grant S. Hamilton, Frank Riedel, Dirk Jan Menger, Andreas E. Albers Tags: Original Article Source Type: research

Necrotizing sarcoid granulomatosis: a distinctive form of pulmonary granulomatous disease
Conclusion: NGS is a disease often confounded clinically with malignancy or with sarcoidosis even histologically when all criteria are not strictly applied. This review provides NGS' characteristics and discusses its differential diagnosis form sarcoidosis, Wegener granulomatosis and tuberculosis. This article is protected by copyright. All rights reserved. (Source: The Clinical Respiratory Journal)
Source: The Clinical Respiratory Journal - July 29, 2017 Category: Respiratory Medicine Authors: Georgia Karpathiou, Anna Batistatou, Panagiotis Boglou, Dimitrios Stefanou, Marios E. Froudarakis Tags: Review Article Source Type: research

Atypical facial involvement of wegener's granulomatosis
Wegener's granulomatosis is an uncommon vasculitis of small- and medium-sized vessels. A 23-year-old female patient was referred with a diffuse gingival swelling and extensive facial ulcers. She reported a 5-month lasting non-responsive retro-auricular pustule-nodular lesion, initially diagnosed as acne. The injury progressed to numerous sharp-demarcated deep facial ulcerations. Skin biopsy was suggestive of pyoderma gangrenosum. The patient also presented with fever, diffuse pulmonary inflammation with cavitation, and gingival hyperplasia. (Source: Oral Surgery, Oral Medicine, Oral Pathology, Oral Radiology, and Endodontics)
Source: Oral Surgery, Oral Medicine, Oral Pathology, Oral Radiology, and Endodontics - July 28, 2017 Category: ENT & OMF Authors: THAYANARA SILVA MELO, LILIANE DE ANDRADE CARVALHO, DANIEL GOMES, ALESSANDRA ALBUQUERQUE TAVARES CARVALHO, JAIR CARNEIRO LE ÃO, LUIZ ALCINO MONTEIRO GUEIROS Tags: CPP - Clinical Poster Presentation Source Type: research

Wegener ’s granulomatosis diagnosis from oral manifestations: a case report
Wegener's granulomatosis (WG) is a rare granulomatous disease characterized by the classic triad of necrotizing granulomas affecting respiratory tracts, disseminated vasculitis, and glomerulonephritis. It can also affect other sites, such as the oral cavity. Herein we report a case of WG with first manifestation in the oral cavity. A 19-year-old male patient had shown a general hyperplasic gingival growth, display of a granular surface, spontaneous bleeding; multiple granulomatous and necrotic ulcers, symptomatic and spontaneous bleeding located in multiple sites of oral mucosa. (Source: Oral Surgery, Oral Medicine, Oral P...
Source: Oral Surgery, Oral Medicine, Oral Pathology, Oral Radiology, and Endodontics - July 28, 2017 Category: ENT & OMF Authors: MA ÍRA TAVARES DE FARIA, MARÍLIA HEFFER CANTISANO, FÁBIO RAMÔA PIRES, RUTH TRAMONTANI RAMOS, ANDREZA MARIA OLIVEIRA FILGUEIRAS, THIAGO MOREIRA PÊSSOA, GERALDO OLIVEIRA SILVA-JUNIOR Tags: CPP - Clinical Poster Presentation Source Type: research

Serum ferritin as an activity marker for granulamotosis with polyangiitis.
CONCLUSIONS: Measurement of serum ferritin might help in assessing disease activity of GPA. PMID: 28741986 [PubMed - in process] (Source: Renal Failure)
Source: Renal Failure - July 27, 2017 Category: Urology & Nephrology Tags: Ren Fail Source Type: research