Clinical characteristics and long-term outcomes of 35 patients with Wegener’s granulomatosis followed up at two rheumatology centers in Lithuania

Objective. The aim of this study was to investigate the survival of Lithuanian patients with Wegener’s granulomatosis, who were followed up at two tertiary rheumatology centers, and to find the factors possibly influencing the outcomes of this disease. Material and methods. Thirty-five patients were followed up prospectively from the onset of disease (the first patient was enrolled in 1994) at Vilnius University Hospital and the Center of Rheumatology of Kaunas University of Medicine (17 and 18 patients, respectively). All patients in both the centers were followed up on a routine basis, and their records contained necessary information about laboratory and biopsy data; the censoring date (end of follow-up) was stated in June 2006. Results. Among the patients, the most frequent organs involved were ear, nose, throat (ENT) (82.6%), lungs (74.3%), and kidney (renal involvement was defined by proteinuria/abnormal urine sediment) (45.7%). Renal insufficiency was present in 20.6% of all the patients. At the end of the study, 32.4% of patients had simultaneously all three organ systems involved, namely upper respiratory tract, pulmonary, and renal. ANCA positivity was found for 26 (74.3%) of all the patients. Overall mortality rate was 25.7% (9/35). The mean survival was 99.4 months (95% CI, 73.6; 125.3) limited to 149 months for the longest-surviving patient. Conclusions. Female gender and all three specific organ involvements being present at the same time and higher vasculitis damage index were associated with poor outcome. Overall mortality rate was 25.7% (9/35) during the 12-year follow-up, and it is similar to the data from other European countries.

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A Case of Generalized Wegener's Granulomatosis in Childhood: Successful Therapy With Cyclophosphamide

PEDIATRICS ◽

10.1542/peds.61.2.286 ◽

1978 ◽

Vol 61 (2) ◽

pp. 286-290

Author(s):

Radhakrishna Baliga ◽

Chung-Ho Chang ◽

Anil K. Bidani ◽

Eugene V. D. Perrin ◽

Larry E. Fleischmann

Keyword(s):

Wegener’S Granulomatosis ◽

Renal Involvement ◽

Rare Condition ◽

Wegener's Granulomatosis ◽

Cytotoxic Agents ◽

Upper Respiratory Tract ◽

Pediatric Age Group ◽

Upper Respiratory ◽

Tract Infections

An 11-year-old white boy had Wegener's granulomatosis, a rare condition in the pediatric age group. The clinical course, pathological findings, and mode of treatment are outlined. The disease is in remission on a regimen of cyclophosphamide therapy as judged by both clinical and pathological criteria. This syndrome with protean manifestations should be considered in children with symptoms of repeated upper respiratory tract infections along with pulmonary and renal involvement. Early renal biopsy helps to establish the diagnosis of generalized involvement and to guide the course of treatment. Follow-up renal biopsies may serve as an indication for the continuation of treatment. Cytotoxic agents, especially cyclophosphamide, dramatically alter the course of the disease.

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A Diagnosis Missed for Several Years- Wegener’s Granulomatosis

Kathmandu University Medical Journal ◽

10.3126/kumj.v9i3.6309 ◽

2012 ◽

Vol 9 (3) ◽

pp. 218-221 ◽

Cited By ~ 1

Author(s):

B P Paudyal ◽

S Pantha ◽

N Ranjitkar ◽

A Manandhar ◽

A Arjyal

Keyword(s):

Wegener’S Granulomatosis ◽

Systemic Vasculitis ◽

Systemic Disease ◽

Granulomatous Inflammation ◽

Wegener's Granulomatosis ◽

Antineutrophil Cytoplasmic Antibodies ◽

Upper Respiratory Tract ◽

Delay In Diagnosis ◽

Organ Systems ◽

Wide Range

Wegener’s granulomatosis is a form of systemic vasculitis of small to medium sized vessels and affects upper respiratory tract, lungs and kidneys along with various organs. It causes necrotizing granulomatous inflammation of the affected parts and presents with positive antineutrophil cytoplasmic antibodies in more severe forms. Being a systemic disease with the potential to affect any organ-systems with a wide range of clinical presentations, it is associated with a risk of delay in diagnosis with resultant setback in institution of appropriate treatment. Confusion may arise due to an extent of histological similarity between Wegener’s granulomatosis and the more prevalent tuberculosis, both causing granulomatous inflammation of the affected parts. Here, we present two cases of this rare disorder where the diagnosis was missed for several years in the beginning causing a delay in institution of specific therapy which led to the development of complications.DOI: http://dx.doi.org/10.3126/kumj.v9i3.6309 Kathmandu Univ Med J 2011;9(3):218-21

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Clinical manifestations of granulomatosis with polyangiitis (Wegener’s granulomatosis) in the upper respiratory tract seen by otolaryngologists in Japan

Clinical and Experimental Nephrology ◽

10.1007/s10157-012-0768-2 ◽

2013 ◽

Vol 17 (5) ◽

pp. 663-666 ◽

Cited By ~ 12

Author(s):

Yasuaki Harabuchi ◽

Kan Kishibe ◽

Yuki Komabayashi

Keyword(s):

Respiratory Tract ◽

Wegener’S Granulomatosis ◽

Granulomatosis With Polyangiitis ◽

Clinical Manifestations ◽

Wegener's Granulomatosis ◽

Upper Respiratory Tract ◽

Upper Respiratory

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Anticytoplasmic Autoantibodies in the Diagnosis and Follow-Up of Wegener's Granulomatosis

Journal of Asthma ◽

10.3109/02770908909073270 ◽

1989 ◽

Vol 26 (5) ◽

pp. 319-322

Keyword(s):

Wegener’S Granulomatosis ◽

Wegener's Granulomatosis

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Complications of Classical Tracheostomy and Management

Tropical Doctor ◽

10.1177/004947550303300309 ◽

2003 ◽

Vol 33 (3) ◽

pp. 148-150 ◽

Cited By ~ 2

Author(s):

P A Onakoya ◽

O G B Nwaorgu ◽

L A Adebusoye

Keyword(s):

Mortality Rate ◽

Home Care ◽

Retrospective Review ◽

Postoperative Management ◽

College Hospital ◽

Airway Diseases ◽

Complications And Mortality ◽

Overall Mortality

Tracheostomy can prevent many deaths in otherwise fatal airway diseases and problems but has numerous complications that are mostly avoidable if the procedure is carefully performed together with strict postoperative management. This is a retrospective review of tracheostomy complications over a 10 year period (1991–2002) in the Department of Otorhinolaryngology (ORL), University College Hospital, Ibadan. A total of 179 tracheostomies performed on 168 patients with 69 complications (38.6%) were documented. There was a significantly greater number of complications in the 43 emergency cases (54%) than in the 26 elective cases [(46%), P=0.0002]. The overall mortality rate was 2.2%. The most common complications of tracheostomy were infective in origin, representing 43% of all complications. This study highlights the complications and mortality and gives details of management that will prevent or minimize their occurrences. Those who require long-term tracheostomy must be on regular follow-up and taught home-care of the tracheostomy before discharge from the hospital.

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Effects of individually chosen homoeopathic medicines on recurrent URTI in children

British Homeopathic Journal ◽

10.1016/s0007-0785(96)80018-4 ◽

1996 ◽

Vol 85 (01) ◽

pp. 4-14

Author(s):

J. Blommers ◽

D.J. Kuik ◽

L. Feenstra ◽

P.D. Bezemer ◽

E.S.M. De Lange-De Klerk

Keyword(s):

Clinical Trial ◽

University Hospital ◽

Controlled Clinical Trial ◽

Upper Respiratory Tract ◽

Eligibility Criteria ◽

Double Blind ◽

Upper Respiratory ◽

The University ◽

Current Standards

AbstractThe effects of homoeopathic medicines on children suffering from recurrent upper respiratory tract infection (URTI) were studied in a randomized double-blind placebo-controlled clinical trial conducted at the paediatric outpatients department of the university hospital of the Vrije Universiteit in Amsterdam from 1987 to 1992.The study was designed to meet both the requirements of proper homoeopathic practice and the current standards of a clinical trial. The purpose of a randomized placebo-controlled double-blind trial is to identify the effects of the agents under investigation by equalizing the effects of other factors that may influence outcome.The object of the trial, eligibility criteria, follow-up period, treatments and concurrent interventions, data collection and effect measures are discussed in the light of homoeopathic thinking.

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Wegener's Granulomatosis Presenting as Necrosis of the Left Mainstem Bronchus

Asian Cardiovascular and Thoracic Annals ◽

10.1177/021849230201000323 ◽

2002 ◽

Vol 10 (3) ◽

pp. 277-279 ◽

Cited By ~ 4

Author(s):

Paul Schneider ◽

Jörn Gröne ◽

Jürgen Braun ◽

Alejandra Perez-Canto ◽

Heinz J Buhr

Keyword(s):

Wegener’S Granulomatosis ◽

Renal Involvement ◽

Left Lung ◽

Wegener's Granulomatosis ◽

Antineutrophil Cytoplasmic Antibodies ◽

Saddle Nose ◽

Bronchial Arteries ◽

Left Mainstem ◽

Saddle Nose Deformity ◽

Bronchial Inflammation

A patient with pansinusitis, nasal septum necrosis, and saddle nose deformity showed necrosis of the left mainstem, upper, and lower bronchi, with complete loss of left lung perfusion and ventilation. Pneumonectomy was performed. Histological findings showed extensive necrotizing and granulomatous bronchial inflammation with vasculitis of the bronchial arteries and the pulmonary vein. Wegener's granulomatosis was diagnosed, despite a negative cytoplasmic pattern of antineutrophil cytoplasmic antibodies and the lack of renal involvement.

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Wegener's granulomatosis: clinical course in 108 patients with renal involvement

Nephrology Dialysis Transplantation ◽

10.1093/ndt/15.5.611 ◽

2000 ◽

Vol 15 (5) ◽

pp. 611-618 ◽

Cited By ~ 83

Author(s):

Knut Aasarød ◽

Bjarne M. Iversen ◽

Jens Hammerstrøm ◽

Leif Bostad ◽

Lars Vatten ◽

...

Keyword(s):

Wegener’S Granulomatosis ◽

Clinical Course ◽

Renal Involvement ◽

Wegener's Granulomatosis

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A Case of Wegener’s Granulomatosis Presenting with Unilateral Facial Nerve Palsy

Case Reports in Medicine ◽

10.1155/2016/9153625 ◽

2016 ◽

Vol 2016 ◽

pp. 1-3 ◽

Cited By ~ 1

Author(s):

Roy Ujjawal ◽

Pan Koushik ◽

Panwar Ajay ◽

Chakrabarti Subrata

Keyword(s):

Facial Nerve ◽

Wegener’S Granulomatosis ◽

Nerve Palsy ◽

Acute Inflammation ◽

Granulomatosis With Polyangiitis ◽

Medical Literature ◽

Facial Nerve Palsy ◽

Renal Involvement ◽

Wegener's Granulomatosis ◽

Classical Triad

Wegener’s granulomatosis or granulomatosis with polyangiitis is a necrotizing vasculitis affecting both arterioles and venules. The disease is characterized by the classical triad involving acute inflammation of the upper and lower respiratory tracts with renal involvement. However, the disease pathology can affect any organ system. This case presents Wegener’s granulomatosis presenting with facial nerve palsy as the first manifestation of the disease, which is rarely reported in medical literature.

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