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Systemic Vasculitis


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]
Splenectomy
  • After some days of irrepressible intestinal hemorrhage, total gastrectomy, splenectomy and resection of the duodenum and pancreas were performed. Histological pictures showed vascular lesions pathognomonic of systemic polyarteritis nodosa (PAN).[ncbi.nlm.nih.gov]
Hyperthermia
  • The effects of hyperthermia and TNF-α on EPC function were also studied. RESULTS: Twenty children, median age 12-years (5-16.5; nine males) were studied.[ncbi.nlm.nih.gov]
Palpable Spleen
  • Examination confirmed aortic regurgitation, palpable spleen and left facial droop. Multiple peripheral blood cultures were negative.[ncbi.nlm.nih.gov]
Upper Abdominal Pain
  • RESULTS: We report a 66-year-old male with a history of renal cell carcinoma and bladder cancer who presented with upper abdominal pain, thoracic myelopathy (progressive weakness and numbness of the lower extremities, and gait disturbances), and weight[ncbi.nlm.nih.gov]
Rectal Bleeding
  • Two weeks before admission to a secondary level hospital, she developed fever up to 40 C followed by abdominal pain, rectal bleeding, hematemesis and blisters on both legs, reason for which she was hospitalized.[ncbi.nlm.nih.gov]
Maculopapular Rash
  • At least 3 out of 5 criteria yields sensitivity and specificity of 71 and 84%: age 16 use of possible triggering drug in relation to symptoms palpable purpura maculopapular rash skin biopsy showing neutrophils around vesselIgA vasculitis (IgAV; formerly[en.wikipedia.org]
Larva Migrans
  • To our knowledge, this is the first observation of temporal vasculitis associated with helminthic infection, extending the clinical spectrum of visceral larva migrans.[ncbi.nlm.nih.gov]
Slurred Speech
  • Author information 1 Department of Rheumatology, North Shore Hospital, North Shore City, New Zealand. laurence_teoh@xtra.co.nz Abstract A 28-year-old man with a bicuspid aortic valve presented with facial droop and slurred speech with several months of[ncbi.nlm.nih.gov]

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]
Legionella Pneumophila
  • Three (12%) patients were admitted to the ICU for infections, including pneumonia ( Legionella pneumophila, n  1; Staphylococcus aureus, n  1) and sepsis ( Escherichia coli, n  1), after 1 month of immunosuppressive treatment for newly diagnosed HBV-related[doi.org]
Bartonella Henselae
  • Grindings from aortic valve leaflets were analysed for rpoB gene, which confirmed the presence of Bartonella henselae. Serological assays demonstrated B henselae IgM 20 (normal 2048 (normal 64).[ncbi.nlm.nih.gov]

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]

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

  • 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]

Epidemiology

  • […] 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]
Sex distribution
Age distribution

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]
  • 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]
  • In some vasculitic disorders, such as granulomatosis with polyangiitis or Kawasaki disease , the vessel inflammation (true vasculitis) is only part of the pathophysiology and there is predominant parenchymal inflammation in a characteristic pattern that[merckmanuals.com]

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]

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