Rituximab: Uses in Dermatology.
Abstract
Rituximab is an anti-CD20 monoclonal antibody with considerable potential in dermatology due to an increase in off-label indications. Chronic graft-versus-host disease and pemphigus vulgaris are two of the most promising indications for off-label use of rituximab. It is a generally safe alternative that should be considered when traditional therapy with corticosteroids or immunosuppressants has failed or caused significant intolerance. Currently, rituximab is only FDA-approved for treatment of follicular and diffuse large B-cell non-Hodgkin's lymphoma, rheumatoid arthritis, chronic lymphocytic leu...
Source: Skin Therapy Letter - August 31, 2016 Category: Dermatology Authors: Gleghorn K, Wilson J, Wilkerson M Tags: Skin Therapy Lett Source Type: research
Poster 85 A Unique Diagnosis of Critical Illness Neuromyopathy in a Patient with Wegener's Granulomatosis: A Case Report
Shweta Duggal: I Have No Relevant Financial Relationships To Disclose (Source: PM and R)
Source: PM and R - August 31, 2016 Category: Rehabilitation Authors: Shweta S. Duggal, Chirag Shah Source Type: research
Multifocal Recurrent Lung Opacities in a Renal Failure Patient
Pulmonary vasculitides include a broad variety of disorders having in common inflammation and destruction of the blood vessels within the lung. Granulomatosis with polyangiitis (GPA), formerly known as Wegener granulomatosis, is an antineutrophil cytoplasmic antibody (ANCA)-associated small-vessel vasculitis that affects a number of organ systems, and is the most common of the ANCA-associated vasculitides. GPA affects a broad age range, most commonly presenting in middle-aged adults. The classic triad of GPA, the combination of upper-airway disease (sinusitis, otitis media, ulcerations, tracheobronchial stenoses), lower-re...
Source: Clinical Pulmonary Medicine - August 27, 2016 Category: Respiratory Medicine Tags: Images in Pulmonary Medicine Source Type: research
Retroperitoneal fibrosis with periaortitis: A case report of an unusual form of presentation of granulomatosis with polyangiitis
We present the case of a 74-year-old man with a past history of infrarenal abdominal aortic aneurysm. He consulted for abdominal pain. Cavitating pulmonary nodules and retroperitoneal fibrosis with periaortic alterations were detected on computed tomography. Laboratory investigations revealed that the patient was positive for cytoplasmic antineutrophil cytoplasmic antibodies (c-ANCA) and necrotising granulomas were observed on biopsies of the lung lesions and retroperitoneal tissue. The patient was diagnosed with GPA and treatment was started with glucocorticoids and immunosuppressive agents, which led to a significant cli...
Source: Respiratory Medicine Case Reports - August 25, 2016 Category: Respiratory Medicine Source Type: research
Rash and fever in a man with granulomatosis with polyangiitis (Wegener's)
(Source: Nephrology)
Source: Nephrology - August 21, 2016 Category: Urology & Nephrology Authors: Dean F Willis, Bobby Chacko Tags: Correspondence Source Type: research
Hydroxyurea for Treatment of Nephrotic Syndrome Associated With Polycythemia Vera
We describe the clinical course of a patient with polycythemia vera and nephrotic-range proteinuria, demonstrated to have FSGS on biopsy. The patient had a distant history of granulomatosis with polyangiitis (Wegener's), for which he routinely had his kidney function and proteinuria measured, allowing for early detection of nephrotic syndrome soon after being diagnosed with polycythemia vera. Treatment with hydroxyurea resulted in rapid improvement in proteinuria that correlated with a decrease in hematocrit. This response was replicated 2 additional times when the patient was taken off and then restarted on hydroxyurea th...
Source: American Journal of Kidney Diseases - August 21, 2016 Category: Urology & Nephrology Source Type: research
Orbital involvement as the initial presentation of Wegener granulomatosis in a 9-year-old girl: MR imaging findings
Publication date: Available online 15 July 2016 Source:Diagnostic and Interventional Imaging Author(s): E. Ure, Y. Kayadibi, D.T. Sanli, Z.I. Hasiloglu (Source: Diagnostic and Interventional Imaging)
Source: Diagnostic and Interventional Imaging - August 16, 2016 Category: Radiology Source Type: research
Sellar Wegener Granulomatosis Masquerading as Cabergoline-Resistant Prolactinoma
Pituitary manifestation of Wegener granulomatosis (WG) is extremely rare. When there is pituitary involvement, the granulomatous inflammatory lesions involving the pituitary gland may appear several months to years after the primary diagnosis. (Source: World Neurosurgery)
Source: World Neurosurgery - August 9, 2016 Category: Neurosurgery Authors: Ilyas M. Eli, Amol Raheja, Heather J. Corn, Debra L. Simmons, Cheryl Ann Palmer, William T. Couldwell Tags: Case Report Source Type: research
[Reversible posterior leukoencephalopathy syndrome in a patient presenting granulomatosis with polyangiitis].
We report a case of RPLS in a woman presenting granulomatosis with polyangiitis (Wegener's granulomatosis).
PATIENTS AND METHODS: A 22-year-old female patient was treated with methylprednisolone pulses for granulomatosis with polyangiitis and neurological impairment. A few hours after the second pulse, the patient had seizures, blindness and confusion associated with high blood pressure and acute renal failure. MRI revealed a high-intensity area on T2-Flair weighted images of the occipital-temporal lobes. The patient was treated with antiepileptic and antihypertensive medications, oral steroids and cyclophosphamide...
Source: Annales de Dermatologie et de Cenereologie - August 4, 2016 Category: Dermatology Authors: Chaput L, Rabot N, Limousin N, Cottier JP, Lioger B, Samimi M Tags: Ann Dermatol Venereol Source Type: research
Gingival hyperplasia as first sign of recurrence of granulomatosis with polyangiitis (Wegener ’s granulomatosis): case report and review of the literature
Granulomatosis with polyangiitis (GPA), formerly referred to as Wegener ’s granulomatosis, is a rare systemic disease of unknown etiology which can affect all areas of the body, including the oral cavity. The t... (Source: BMC Oral Health)
Source: BMC Oral Health - August 2, 2016 Category: Dentistry Authors: Marcel Hanisch, Leopold F. Fr öhlich and Johannes Kleinheinz Source Type: research
[Intraocular Inflammation: Autoimmune or Infectious?]
Authors: Auw-Hädrich C, Heinzelmann S, Coupland S
Abstract
Presentation of 3 cases of intraocular inflammation: 1. 47-year old female patient with severe necrotising scleritis and uveitis with underlying granulomatous polyangiitis (formerly known as Wegener granulomatosis, in honour of the German pathologist Friedrich Wegener), known for 10 years. 2. 48-year old male patient with longstanding bilateral uveitis and granulomatous polyangiitis for 2 years. In the histopathological examination of the enucleation specimen, a retrolental tumour turned out to be a granuloma. 3. 57-year old male patient in status...
Source: Klinische Monatsblatter fur Augenheilkunde - July 30, 2016 Category: Opthalmology Tags: Klin Monbl Augenheilkd Source Type: research
Subglottic Stenosis in Granulomatosis With Polyangiitis (Wegener's Granulomatosis): Report of 4 Cases
Conclusion SGS presents high morbidity. Even though subglottic dilatation provides symptomatic relief, recurrences may present. Severe airway-limiting stenosis often requires tracheostomy. (Source: Reumatologia Clinica)
Source: Reumatologia Clinica - July 25, 2016 Category: Rheumatology Source Type: research
Heart Failure in Granulomatosis with Polyangiitis
Background: While small case series have associated granulomatosis with polyangiitis (GPA; Wegener's granulomatosis) with various cardiovascular manifestations such as valvular heart disease, conduction system disease, and pericardial involvement, scarce data exist regarding the prevalence of cardiovascular risk factors and outcomes, including heart failure (HF). Methods: Using a large, multi-institutional database (Explorys Inc, Cleveland, OH), we performed a retrospective cohort analysis on patients with GPA, HF, and cardiovascular risk factors. (Source: Journal of Cardiac Failure)
Source: Journal of Cardiac Failure - July 23, 2016 Category: Cardiology Authors: Chang H. Kim, Justin Morrison, Ben Alencherry, Fahd Nadeem, Sadeer G. Al-Kindi, Guilherme H. Oliveira Source Type: research
Postepy Hig Med Dosw 2016; 70:210-218 "Wegener ’ s granulomatosis and pyoderma gangrenosum – rare causes of facial ulcerations"
This study revealed a similar rate of comorbidity of WG and PG in both genders and an increased incidence of both diseases after the age of 50. Among skin lesions there was a dominance of ulceration, most often deep and painful, covering a large area with the presence of advanced necrosis and destruction of the surrounding tissue. The most common location proved to be the cervical-cephalic area. The most popular treatment included steroids with cyclophosphamide.
Discussion: The rarity of the coexistence of these two diseases results in a lack of effective therapy. In such cases sulfone derivatives are still effective and ...
Source: Postepy higieny i medycyny doswiadczalnej - July 22, 2016 Category: Research Tags: Original article Source Type: research
Scleritis in patients with granulomatosis with polyangiitis (Wegener)
Conclusions
The presence of necrotising changes or corneal involvement in the setting of scleral inflammation is highly suggestive of an underlying systemic vasculitis, of which GPA is the most common. These features should alert the doctor/optometrist and prompt a thorough diagnostic approach and an aggressive treatment given that it could reveal a life-threatening disease. (Source: British Journal of Ophthalmology)
Source: British Journal of Ophthalmology - July 20, 2016 Category: Opthalmology Authors: Cocho, L., Gonzalez-Gonzalez, L. A., Molina-Prat, N., Doctor, P., Sainz-de-la-Maza, M., Foster, C. S. Tags: Angle, Choroid, Intraocular pressure, Sclera and apisclera, Neurology, Epidemiology Original articles - Clinical science Source Type: research
