China
Africa
Explore chapters and articles related to this topic
Cardiothoracic and Vascular
Published in Vincent Helyar, Aidan Shaw, The Final FRCR, 2017
Also known as granulomatosis with polyangiitis (the preferred term due to Friedrich Wegener's links to the Nazi Party), it is a necrotising granulomatous vasculitis of the upper and lower respiratory tracts and renal glomeruli, affecting the small arteries and veins. Common clinical features are rhinitis, sinusitis, focal glomerulonephritis and otitis media. There is cardiac involvement (coronary vasculitis, pancarditis and valvular lesions) in up to 30%. Associated with c-ANCA antibodies.
Clinical Cases
Published in S. J. Copley, J. P. Kanne, D. M. Hansell, Thoracic Imaging, 2014
S. J. Copley, J. P. Kanne, D. M. Hansell
Granulomatosis with polyangiitis is a necrotising granulomatous vasculitis that involves the upper and lower respiratory tracts and, in the classic form, there is a focal necrotising glomerulonephritis. Other organs are involved to a variable extent by a small-vessel granulomatous vasculitis. Approximately one-fifth of patients do not have renal or other extrapulmonary involvement. This uncommon disease can occur at any age but the mean age at presentation is 40 years with a male to female ratio of 3:2. ANCA are strongly positive in the small vessel vasculitides, and 85% of patients with untreated granulomatosis with polyangiitis have c-ANCA positivity. More specifically, anti-PR3 ANCA levels are elevated. The clinical presentation is variable and often consists of nonspecific constitutional symptoms. However, respiratory tract symptoms and signs predominate.
Diseases of Blood vessels
Published in P. Chopra, R. Ray, A. Saxena, Illustrated Textbook of Cardiovascular Pathology, 2013
is observed (Fig. 13.30).Etiologyof Wegener granulomatosis is unknown and ill understood. It is believed that in a predisposed individual exposure of the airways to unidentified substances leads to systemic autoimmune disease. Immunoglobulin deposits on the glomerular basement membrane and/or circulating immune complexes have been demonstrated in some patients. Cell mediated immunity has also been implicated. Antinuclear cytoplasmic autoantibodies (c-ANC A) have been demonstrated in about 85-90% patients with active Wegener granulomatosis and about 40% of patients in remission. Serial measurements of c-ANCA have been used to monitor disease activity and effect of therapy.
Depiction of unusual coronary involvement in Wegener’s Granulomatosis on CT
Published in Acta Cardiologica, 2021
SH Chandrashekhara, Vineeta Ojha, Sreenivasa Narayana Raju, Sanjeev Kumar
A 15 year-old girl presented with fever, dyspnoea, haemoptysis, bleeding gums and joint pains for a duration of twenty days. Further evaluation revealed raised total leukocyte count (12,000/cu. mm) and elevated fibrin degradation products. ECG showed sinus tachycardia. Chest X-ray revealed multifocal areas of consolidation some of them showing cavitation. Sputum examination was negative for acid fast bacteria. PCR was also negative for tuberculosis. c-ANCA (antineutrophil cytoplasmic antibodies) were positive. CT angiography, done for further evaluation, revealed ectasia of the proximal left anterior descending (LAD) artery. There was mild irregularity of the wall of the ectatic segment. There was no intra-luminal thrombus. The thoracic aorta and bilateral pulmonary arteries were normal. There were multiple large cavitating lesions in both the lungs, some of them had air fluid levels (Figure 1). There was no significant mediastinal lymph node enlargement. No pleural or pericardial effusion was seen. In addition, there was mild splenomegaly with multiple splenic infarcts. On the basis of clinical, laboratory and imaging findings, an initial diagnosis of ANCA associated vasculitis, Wegener’s Granulomatosis was made and immunosuppressive therapy was started after initial pulse steroid regimen. The patient became better symptomatically on follow up at one month.
Atypical Perinuclear Anti-Neutrophil Cytoplasmic Antibodies in Ocular Inflammatory Diseases
Published in Ocular Immunology and Inflammation, 2019
Nazanin Ebrahimiadib, Lina Ma, Bobeck S. Modjtahedi, Samaneh Davoudi, Safa Rahmani, Sarah Syeda, Andrew Stephenson, Charles Stephen Foster
C-ANCA and P-ANCA are associated with small vessel vasculitis such as granulomatosis with polyangiitis, microscopic polyangiitis, and eosinophilic granulomatosis with polyangiitis. High specificity (98%), sensitivity (81%), and negative predictive value (99%) of ANCA for the aforementioned diseases has been previously demonstrated which makes ANCA testing an essential component in evaluating vasculitis.5,6 ANCA titer level can also be monitored as a measure of disease activity. About 16% of patients with ANCA-associated vasculitis have ocular manifestations in the form of scleritis, episcleritis, orbital inflammation, and retinal vasculitis.7,8 Despite sufficient information on the clinical importance of C-ANCA and P-ANCA, there is limited data regarding the value of atypical P-ANCA.
28-year-old male anabolic steroid abuser with Susac syndrome. An interdisciplinary case report
Published in Modern Rheumatology Case Reports, 2019
Natalia Lewczuk, Alexander Zdebik, Joanna Bogusławska, Magdalena Targońska, Anna Turno-Kręcicka
The patient received vinpocetine 10 mg 3×/day to improve retinal blood flow and to avoid further progression of ischaemic changes in the fundus of the eye. He was also referred to an internal examination and performed tests for a complete blood count, erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), cholesterol and triglycerides in the serum, blood tests for factor V-Leiden, protein C and protein S mutation, homocysteine levels, antithrombin III, anti-phospholipid, c-antineutrophil cytoplasmic antibodies (c-ANCA) and p-antineutrophil cytoplasmic antibodies (p-ANCA), an antinuclear antibody-screening (ANA-screening), venereal disease research lab (VDRL) and Western blot. Immunofluorescence staining for c-ANCA (proteinase 3) and p-ANCA (myeloperoxidase) antibodies has been performed. No cytoplasmic and perinuclear staining in ethanol-fixed neutrophils has been detected. An enzyme-linked immunosorbent assay test for c-ANCA and p-ANCA has therefore not been performed. All performed tests and serological screenings for infectious agents mentioned earlier above were negative. Carotid artery Doppler ultrasonography and transthoracic echocardiography did not show any changes. Holter-ECG readings and blood pressure was normal. The patient was examined by a neurologist because of chronic headaches. On examination the patient was conscious, co-operative, well-oriented, without meningeal signs and with a natural muscle tone and strength (Table 1).