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Hypocomplementemic Vasculitis

Urticarial Vasculitis


Presentation

  • His disease resembled the hypocomplementemic vasculitis syndrome but was atypical in its early age of presentation, severe hypocomplementemia, and progression to end-stage renal disease.[ncbi.nlm.nih.gov]
  • C1q-p activity also was observed in and purified from SLE serum; this activity was distinguishable from 7S immune complex activity detected by Raji cells which was also present in SLE serum.[ncbi.nlm.nih.gov]
  • Renal disease, when present, is usually mild to moderate, and obstructive lung disease, which may be severe, occurs in some patients.[mitchmedical.us]
  • […] rheovirus-III infection, presents with interstitial keratitis and hearing loss. 3) Schnitzler syndrome – presents with urticaria and monoclonal IgM gammopathy along with lymphadenopathy, arthralgia and fever, 4) AHA-syndrome – poorly understood entity[omicsonline.org]
Recurrent Infection
  • infections, meaning that people with a normal immune system don't continually catch the same cold or same strain of flu repeatedly.Case studies of individuals with HUV have also highlighted other potential complicating factors which it seems the anti-C1q[en.wikipedia.org]
Turkish
  • History Meanings of "hypocomplementemic vasculitis" in Turkish English Dictionary : 1 result(s) Category English Turkish Medical 1 Medical hypocomplementemic vasculitis hipokomplementemik ürtikaryal vaskülit sendromu Meanings of "hypocomplementemic vasculitis[tureng.com]
Dysphagia
  • Received : 4.3.1999; Accepted : 7.4.1999 ABSTRACT A 55 years post menopausal lady presented with puffiness of face, and a pruritic urticarial rash over face and upper trunk of one week duration with accompanying dysphagia.[japi.org]
Regurgitation
  • In addition to urticarial cutaneous lesions, he experienced polyarthralgia and arthritis that resulted in progressive deformity of the joints of both hands, cardiac valvulopathy with mitral, tricuspid, and aortic regurgitation, and intermittent neck swelling[jmedicalcasereports.biomedcentral.com]
Arthralgia
  • Patients with this condition are likely to have systemic involvement, including angioedema, arthralgias, pulmonary disease, abdominal or chest pain, fever, renal disease, and episcleritis.[diseaseinfosearch.org]
  • Overview A rare condition characterized by the presence of recurring urticarial vasculitis, arthralgia or arthritis and hypocomplementemia.[checkorphan.org]
  • .; licensee BioMed Central Ltd. 2014 Received: 6 May 2014 Accepted: 28 August 2014 Published: 22 October 2014 Abstract Introduction Hypocomplementemic urticarial vasculitis syndrome is a rare disorder characterized by chronic urticarial vasculitis, arthralgia[jmedicalcasereports.biomedcentral.com]
  • AHA-syndrome was ruled out based on the absence of arthritis/ arthralgia, Cogan’s syndrome due to the absence of keratitis (despite hearing loss).[omicsonline.org]
  • Urticaria and arthralgias as manifestations of necrotizing angiitis (vasculitis). J Invest Dermatol 1974;63:485-90. [ PUBMED ] 2. Soter NA. Cutaneous necrotizing vasculitis.[ijdvl.com]
Urticaria
  • From Wikidata Jump to navigation Jump to search No description defined Chronic urticaria as a manifestation of venulitis Hypocomplementemic urticarial vasculitis syndrome Hypocomplementemic vasculitis Unusual lupus-like syndrome Mac Duffie syndrome Anti-C1q[wikidata.org]
  • […] common diagnosis in patients with hives –Often related to food or drug allergies, bites, or stings –25% of patients with one episode will progress to chronic urticaria * Chronic urticaria –Idiopathic in 50% of cases –Chronic idiopathic urticaria spontaneously[checkorphan.org]
  • Associations Hepatitis-B virus, Sjögren syndrome, systemic lupus erythematosus, myeloma, IgM gammopathy, pregnancy, cancer, solar urticaria, cold urticaria, and drugs (e.g., diltiazem).[medical-dictionary.thefreedictionary.com]
  • The KI challenges in the two sensitive patients precipitated urticaria, angioedema, polymyalgias, conjunctivitis, and coryza.[annals.org]
  • A rare autoimmune disease characterized by recurrent urticaria (nettle rash), first described in the 1970s. There is no defined paradigm for the syndrome aetiology and severity in progression.[en.wikipedia.org]
Chronic Urticaria
  • From Wikidata Jump to navigation Jump to search No description defined Chronic urticaria as a manifestation of venulitis Hypocomplementemic urticarial vasculitis syndrome Hypocomplementemic vasculitis Unusual lupus-like syndrome Mac Duffie syndrome Anti-C1q[wikidata.org]
  • urticaria * Chronic urticaria –Idiopathic in 50% of cases –Chronic idiopathic urticaria spontaneously resolves within 2 years in 80% of patients –Criterion for chronic urticaria is duration of more than 6 weeks * Occult infection (e.g., sinusitis, oral[checkorphan.org]
  • The four sensitive patients were strikingly similar in that they exhibited hypocomplementemia and dermal vasculitis associated with chronic urticaria or systemic lupus erythematosus, suggesting that other patients with similar clinical features may be[annals.org]
  • Abstract A 58-year-old Japanese woman presented with recurrent abdominal pain, chronic urticaria, and petechiae on her extremities, and hypocomplementemia, findings that were consistent with hypocomplementemic urticarial vasculitis syndrome (HUVS).[jstage.jst.go.jp]
Palpable Purpura
  • There was scleral erythema, urticaria on the torso, palpable purpura on the legs, and swelling and tenderness of the hand joints. A diagnostic procedure was performed. Funding and Disclosures This case was presented at the Rheumatology Grand Rounds.[nejm.org]
  • Physical exam Palpable purpura is pathognomonic for vasculitis. Palpable purpura consists of elevated, nonblanching erythematous lesions.[bobbybukamd.com]
Neck Swelling
  • In addition to urticarial cutaneous lesions, he experienced polyarthralgia and arthritis that resulted in progressive deformity of the joints of both hands, cardiac valvulopathy with mitral, tricuspid, and aortic regurgitation, and intermittent neck swelling[jmedicalcasereports.biomedcentral.com]
Dysesthesia
  • […] cutaneous diseases Arne Kanig and Rudolf Happle Ichthyoses erythrokeratodermas and related disorders Keratodermas Kro Dariers disease and HaileyHailey disease Daniel Hohl Theodora Mauro and leanPhilippe Gorog Mosaicism and linear lesions Pruritus and dysesthesia[books.google.com]

Workup

  • A laboratory workup showed his levels of total protein to be 5.7g/dL, albumin 3.3g/dL, BUN 21mg/dL, creatinine 1.38mg/dL, D-dimer 3.5μg/mL (normal range, 0 to 1μg/mL), fibrinogen 481mg/dL (normal range, 200 to 400mg/dL), lactate dehydrogenase 751IU/L[jmedicalcasereports.biomedcentral.com]

Treatment

  • On the sixth day of treatment, due to the moderate increase of transaminases and widespread development of oesophagial candidiasis, the steroid treatment was terminated and mycostatin suspension was included in the treatment.[academic.oup.com]
  • Treatments - Procedures Plasma exchange or plasmapheresis This treatment is sometimes used in patients with severe vasculitis where antibodies in the blood are thought to be important in causing the disease.[vasculitis.org.uk]
  • Treatment depends upon the severity of the disease[patient.info]
  • COPD and cardiac valvular defects may require specific treatment.[secure.ssa.gov]
  • Treatment is based on the systemic effects of the disease and the extent of cutaneous involvement. Antihistamines or non-steroidal anti- inflammatory drugs ( NSAIDs ) may be used to relieve symptoms.[dermnetnz.org]

Prognosis

  • Although HUV is uncommon in childhood, the prognosis is worse for those affected at younger ages because of more frequent, severe renal involvement. [3] The prognosis can also be influenced by other underlying disorders that HUV is associated with. [5[rarediseases.info.nih.gov]
  • For information on plasma exchange: Plasma exchange or plasmapheresis Prognosis The overall prognosis in Urticarial Vasculitis depends on the severity of the disease and the amount of damage that has been done to organs, especially the lungs.[vasculitis.org.uk]
  • Diagnosis - Hypocomplementemic urticarial vasculitis Home medical testing related to Hypocomplementemic urticarial vasculitis: * Home Allergy Tests o Home Mold Tests o Home Food Intolerance Tests o Home Radon Tests o Home Lung Function Tests Prognosis[checkorphan.org]
  • ICD-9: 279.4 PROGRESSION The prognosis of HUVS depends on the organ system involved. Lung disease results in significant morbidity and mortality, and is made worse by smoking.[secure.ssa.gov]

Etiology

  • .): 31628 Abstract Hypocomplementemic urticarial vasculitis syndrome is an immune complex-mediated disease of unknown etiology. Theclinical course is characterized by urticaria, conjunctivitis, jointpain, and hypocomplementemia.[platform.almanhal.com]
  • . * Comprehensive current coverage of basic models of etiology and pathogenesis * Complete catalog of autoantibodies produced * Includes an outstanding review of imaging techniques for the diagnosis of lupus and its complications for each organ system[books.google.com]
  • Although it is a chronic and recurrent process and long-term prognosis is good, it is important to differentiate urticarial vasculitis from common urticaria as search for possible underlying etiologic factors may have prognostic implications. 1.[ijdvl.com]
  • Small vessel cutaneous vasculitis: etiology, pathogenesis, classification and diagnostic criteria - Part I. An Bras Dermatol. 2007; 82 :387–406. [ Google Scholar ] 6.[ncbi.nlm.nih.gov]
  • Small vessel cutaneous vasculitis: etiology, pathogenesis, classification and diagnostic criteria - Part I. An Bras Dermatol. 2007;82:387-406. [ Links ] 6. Spicknall KE, Zirwas MJ, English JC 3rd.[scielo.br]

Epidemiology

  • Sarcoidosis 514 28 Storage and Deposition Diseases 523 29 The Amyloidoses 533 30 Neoplasms of the Joint 543 31 Heritable Disorders of Connective Tissue 549 32 Bone and Joint Dysplasias 559 33 Osteonecrosis 565 34 Pagets Disease of Bone 573 35 Osteoporosis A Epidemiology[books.google.com]
  • The objective of this study was to describe the clinical characteristics and epidemiology of HUV in two geographically areas of Sweden during a 16-year period.[acrabstracts.org]
Sex distribution
Age distribution

Pathophysiology

  • Neoplasms of the Joint 543 31 Heritable Disorders of Connective Tissue 549 32 Bone and Joint Dysplasias 559 33 Osteonecrosis 565 34 Pagets Disease of Bone 573 35 Osteoporosis A Epidemiology and Clinical Assessment 576 35 Osteoporosis B Pathology and Pathophysiology[books.google.com]
  • When it comes to pathophysiology of the disease this type of vasculitis is relatively similar to other types of vasculitis that are in the nature leukocytoclastic and affect small blood vessels.[ic.steadyhealth.com]
  • The membrane attack complex of complement alters the membrane integrity of cultured endothelial cells: a possible pathophysiology for immune complex vasculitis.. Acta Derm Venereol 76 :, 13– – 16. [Google Scholar] Kimura M.. ( 1980 ; ).[jmmcr.microbiologyresearch.org]
  • ., 3rd Clubbing: an update on diagnosis, differential diagnosis, pathophysiology and clinical relevance. J Am Acad Dermatol. 2005; 52 :1020–1028. [ PubMed ] [ Google Scholar ] 7. Miyachi H, Akizuki M, Yamagata H, Mimori T, Yoshida S, Homma M.[ncbi.nlm.nih.gov]
  • Clubbing: an update on diagnosis, differential diagnosis, pathophysiology and clinical relevance. J Am Acad Dermatol. 2005;52:1020-8. [ Links ] 7. Miyachi H, Akizuki M, Yamagata H, Mimori T, Yoshida S, Homma M.[scielo.br]

Prevention

  • […] or rubbed skin area * Hereditary or acquired deficiency of complement factor C1 –Generally appears as episodic angioedema in the absence of urticaria –Only in the absence of urticaria should hereditary or acquired complement deficiency be considered Prevention[checkorphan.org]
  • It may also involve giving drugs to thin the blood and prevent it clotting in the machine.[vasculitis.org.uk]
  • Shaw A (2011) Models of preventable disease: contrast-induced nephropathy and cardiac surgery associated acute kidney injury. Contrib Nephrol 174:156–162 CrossRef PubMed Google Scholar 47.[link.springer.com]
  • In one study by Ronkainen et al, prednisone did not prevent renal disease from occurring although it led to faster resolution of disease once it occurred.[bobbybukamd.com]

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