Presentation
CASE PRESENTATION: We report the case of a 44 year old man who presented with an ANCA-associated systemic vasculitis and aortic regurgitation of unusual mechanism. [ncbi.nlm.nih.gov]
CASE • A 29yo woman presents with a 4yr h/o skin ulcers on her lower extremities. A previous punch biopsy showed thrombotic lesions in small blood vessels of the dermis. [slideshare.net]
AVR Non specific Present report At initial presentation AR Septal thickening: incomplete closure of Ao valve, thickened leaflets 4 Mo IST AVR Non specific Half the valvular lesions were present at initial presentation and when the diagnosis of vasculitis [bmcmusculoskeletdisord.biomedcentral.com]
Entire Body System
- Relapsing Polychondritis
polychondritis and one with IgA vasculitis. [ncbi.nlm.nih.gov]
polychondritis (RP), Behcet s disease (BD). [clinicaltrials.gov]
Relapsing polychondritis vasculitis among others 6. Vasculitis associated with probable etiology includes a. Hepatitis B– and C–associated vasculitis b. [medlink.com]
- Sepsis
CHF in ICU 23 M 44 Cryo 1 Pneumonia and ARDS after cyc for of severe cryo secondary to SS 23 32 0 – ATB Died of sepsis in ICU 24 F 29 HIV vasculitis 1 ARDS after bronchoalveolar lavage, bowel ischaemia with angiographically and histologically proven [doi.org]
The toxic products formed by bacteria are absorbed, causing systemic manifestation of sepsis and finally death. The affected part is edematous, soft, putrid, rotten, and dark. [en.wikipedia.org]
PubMed Google Scholar Bone RC, Balk RA, Cerra FB, Dellinger RP, Fein AM, Knaus WA, Schein RM, Sibbald WJ: Definitions for sepsis and organ failure and guidelines for the use of innovative therapies in sepsis. [bmcanesthesiol.biomedcentral.com]
These include serious and life-threatening conditions, such as pneumonia and blood infection (sepsis). Oct. 18, 2017 [mayoclinic.org]
VASCULITIS MIMICS •Systemic illness – must exclude alternative diagnoses: •Sepsis •Drug toxicity •Malignancy •Coagulopathy • INFECTIOUS DISEASES • Endocarditis • HIV • DRUGS • Cocaine • Methamphetamine • CHOLESTEROL EMBOLI • ANTIPHOSPHOLIPID ANTIBODY [slideshare.net]
- Hodgkin Lymphoma
Primary Sjögren's syndrome is associated with enhanced risk for B-cell lymphoma [9], in which the presence of purpura or vasculitis are clinical risk indicators for future non-Hodgkin's lymphoma development, as has been identified in a number of studies [arthritis-research.biomedcentral.com]
- Pallor
Pallor and pedal edema were present. Asterixis was noticeable. The cardiovascular system was normal, but chest examination revealed bronchial breathing in the right inter- and infra-scapular regions. [indianjnephrol.org]
Respiratoric
- Rhinitis
They may develop rhinitis, sinusitis (Fig. 6), septal perforation or stenosis of the nasal airway. [elsevier.es]
Patients typically have a previous history of asthma, allergic rhinitis, and/or sinusitis. A characteristic feature is the finding of nonfixed pulmonary infiltrates. [emedicine.medscape.com]
It can cause: asthma allergic rhinitis ( cold-like symptoms caused by allergies ) a high temperature (fever) muscle and joint pain tiredness loss of appetite and weight loss It can also affect the nerves, causing weakness, pins and needles or numbness [nhs.uk]
Cardiovascular
- Vascular Disease
Prompt referral for a complete rheumatologic workup in cases of unexplained hearing loss would allow for a timely diagnosis of a collagen vascular disease and facilitate early initiation of systemic therapy, possibly yielding better audiological results [ncbi.nlm.nih.gov]
Manfredi, Translational Mini‐Review Series on Immunology of Vascular Disease: Mechanisms of vascular inflammation and remodelling in systemic vasculitis, Clinical & Experimental Immunology, 156, 3, (395-404), (2009). Renato A. [doi.org]
Noninfectious vasculitis Vascular disease Any of a number of conditions characterized by inflammation of vessels in multiple sites in the body, which share many clinical and lab features Clinical Fever, fatigue, anorexia, weight loss, polymyalgia rheumatica [medical-dictionary.thefreedictionary.com]
Jaw & Teeth
- Dental Caries
caries, and the inability to speak for extended periods of time. [ssa.gov]
Neurologic
- Insomnia
Alterations of mental status characterized by delusional thinking, confusion, insomnia, lack of attention, and cognitive decline. 7. headache with a lancinating quality, commonly localized to the areas along the affected arteries of the scalp. persistent [slideshare.net]
In addition, medications used in the treatment of HIV infection may also have effects on mental functioning, including cognition (for example, memory), concentration, and mood, and may result in malaise, severe fatigue, joint and muscle pain, and insomnia [ssa.gov]
Urogenital
- Renal Injury
It is not as common in anti-GBM disease as it is in ANCA disease to reverse renal injury to the point of no longer requiring renal replacement therapy. Anti-GBM disease in managed with corticosteroids, cyclophosphamide, and daily plasmapheresis. [renalandurologynews.com]
Workup
Prompt referral for a complete rheumatologic workup in cases of unexplained hearing loss would allow for a timely diagnosis of a collagen vascular disease and facilitate early initiation of systemic therapy, possibly yielding better audiological results [ncbi.nlm.nih.gov]
Diagnostic workup for MPA reveals the presence of serum ANCA and absence of surface hepatitis B antigen or antibody. [slideshare.net]
Electrocardiograms (ECGs) are a series of leads placed on a person's chest that measure electrical activity associated with contraction of heart muscle.[83] The taking of an ECG is an important part in the workup of an AMI,[24] and ECGs are often not [en.wikipedia.org]
Treatment
Long-term treatment and disease-related morbidity are major threats. The last decade has seen a collaborative international effort to determine effective treatment. [ncbi.nlm.nih.gov]
TREATMENT: CORTICOSTEROIDS, THALIDOMIDE, IMMUNOSUPPRESSIVE 69. [slideshare.net]
Prognosis
The combination of vasculitis and cardiomyopathy is associated with a poor prognosis. Early treatment with immunosuppressants in conjunction with appropriate cardiac pharmacotherapy is considered important and has dramatically improved prognosis. [ncbi.nlm.nih.gov]
For example, a patient with purpura, nephritis, and abdominal pain caused by Henoch–Schönlein purpura usually has a good prognosis. .. [doi.org]
Etiology
Over the past three decades, tremendous progress has been made through investigative efforts to understand etiologic, pathogenetic and clinical underpinnings of these disorders. [uncch.pure.elsevier.com]
All patients with a vasculitis of unidentified etiology should be evaluated for HIV infection. [ncbi.nlm.nih.gov]
Homepage Rare diseases Search Search for a rare disease Vasculitis Disease definition Vasculitis represents a clinically heterogenous group of diseases of multifactorial etiology characterized by inflammation of either large-sized vessels (large-vessel [orpha.net]
These protean clinical manifestations, combined with the etiologic nonspecificity of the histologic lesions, complicate the diagnosis of specific forms of vasculitis. [doi.org]
Hoffman in Anti-neutrophil Cytoplasmic Antibody (ANCA), Associated Vasculitis: Granulomatosis with Polyangiitis (GPA, Wegener's) and Microscopic Polyangiitis (MPA) and Primary, Idiopathic Vasculitis: Presumed Autoimmune Etiology. [youtube.com]
Epidemiology
Scott, Epidemiology of systemic vasculitis, Current Rheumatology Reports, 10.1007/s11926-005-0036-5, 7, 4, (270-275), (2005). [doi.org]
[…] provide our annual digest of the recent literature on systemic vasculitis in which we reviewed all the articles published during the last 12 months on large-, medium- and small-vessel vasculitis, and selected the most relevant studies regarding the epidemiology [ncbi.nlm.nih.gov]
: Epidemiology of vasculitis in Europe. Ann Rheum Dis 2001, 60 :1156–1157. PubMed CrossRef Google Scholar 6. [link.springer.com]
Pathophysiology
Subsequently, we review the literature on the combination of myelodysplastic syndromes and systemic vasculitis and discuss the prognostic consequences, considerations for treatment and possible pathophysiological mechanisms. [ncbi.nlm.nih.gov]
Access personal reporting Related Content This Book Etiology, Pathogenesis and Pathophysiology of Aortic Aneurysms and Aneurysm Rupture Edited by Reinhart Grundmann Next chapter Drug-induced aortic aneurysms, ruptures and dissections By Olav Spigset Related [intechopen.com]
Anti-neutrophil cytoplasmic antibodies: current diagnostic and pathophysiological potential. Kidney Int. 1994;46(1):1-15. http://dx.doi.org/10.1038/ki.1994.239. PMid:7933826. Burns JC, Glodé MP. Kawasaki syndrome. [jvascbras.org]
BOSTON— The latest research on the pathophysiology and treatment of small- and large-vessel vasculitides was presented by three international experts at a clinical symposium called “Therapeutic Decisions in Systemic Vasculitis” at the ACR/ARHP Annual [the-rheumatologist.org]
Prevention
Transthoracic and transesophageal echocardiography disclosed septal hypertrophy preventing a complete closure of the aortic valve and thus responsible for a massive aortic regurgitation. [ncbi.nlm.nih.gov]
Eating well can help prevent potential problems that can result from your medications, such as thinning bones, high blood pressure and diabetes. [mayoclinic.org]