[…] 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]

Renal disease, when present, is usually mild to moderate, and obstructive lung disease, which may be severe, occurs in some patients. [mitchmedical.us]

Case presentation A 30-year-old Korean man presented with hypocomplementemic urticarial vasculitis syndrome. [jmedicalcasereports.biomedcentral.com]

[…] uncommon in children. [2] A full-term 9-month-old male infant, born normally to non-consanguineous parents, presented with a three-day history of fever and skin rash. [ijdvl.com]

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]

• Infectious Mononucleosis

  Immunoglobulin disorders such as immunoglobulin A and immunoglobulin M monoclonal gammopathies Leukaemia and internal cancers Viral diseases such as hepatitis B, hepatitis C and infectious mononucleosis (glandular fever ) Drug-induced conditions from [dermnetnz.org]

• Recurrent Abdominal Pain

  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]

  The minor criteria are: [7] [3] Inflammation in the small veins of the dermis (diagnosed by biopsy ) Joint pain or arthritis Mild kidney inflammation ( glomerulonephritis ) Eye inflammation (in the uvea or episclera ) Recurrent abdominal pain The presence [rarediseases.info.nih.gov]

  abdominal pain, and glomerulonephritis. [omicsonline.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]

• Hepatomegaly

  0001654 Ascites Accumulation of fluid in the abdomen 0001541 Ataxia 0001251 Cerebral palsy 0100021 Cranial nerve paralysis 0006824 Diarrhea Watery stool 0002014 Emphysema 0002097 Hemiplegia/hemiparesis Paralysis or weakness of one side of body 0004374 Hepatomegaly [rarediseases.info.nih.gov]

• Arthralgia

  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]

  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]

  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]

  Overview A rare condition characterized by the presence of recurring urticarial vasculitis, arthralgia or arthritis and hypocomplementemia. [checkorphan.org]

  The typical patient is a young female with chronic rash, angioedema, and arthralgias. [mitchmedical.us]

• 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]

  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]

• 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]

  […] 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]

  A clinical form of hypersensitivity vasculitis characterised by urticarial wheals and angioedema, which tend to persist longer than common chronic urticaria. [medical-dictionary.thefreedictionary.com]

  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

  Figure 2 Swelling of the proximal interphalangeal joints with swan neck and flexion deformities of both hands. Figure 3 Findings of kidney biopsy. (A) The mesangium was expanded due to increased matrix without hypercellularity. [jmedicalcasereports.biomedcentral.com]

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]

• Creatinine Decreased

  Further course and work-up Few days later her abdomen pain and nausea resolved, her liver enzymes normalized, creatinine decreased to 179 μmol/L, other routine labs did not changed much, but she complained recurrence of skin rush (Figure 1), eye redness [omicsonline.org]

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]

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]

If you have concerns with your treatment plan, speak up. Your doctor may be able to adjust your dosage or offer different treatment options. [vasculitisfoundation.org]

The patient started symptomatic treatment and was referred to a gastroenterologist for management of the hepatitis C, with progressive improvement of the skin condition. [scielo.br]

COPD and cardiac valvular defects may require specific treatment. [secure.ssa.gov]

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]

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]

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]

Urticarial vasculitis carries a good prognosis, with most occurrences resolving in months to years. [emedicine.medscape.com]

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]

Other etiologies include drug reactions and parasitic infections. [10] Etiology The etiology of urticarial vasculitis has not been elucidated. Associated conditions are listed in History. [emedicine.medscape.com]

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]

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]

Granulomatous diseases of unproved etiology. Connective tissue diseases. Elastic tissue diseases. Vasculitis Discipline Sarcoidosis. Granulomatosis of undetermined origin. Connective tissue diseases. [pascal-francis.inist.fr]

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]

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]

Epidemiology Frequency The exact frequency of urticarial vasculitis is not known in the United States or worldwide. [emedicine.medscape.com]

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]

The hypocomplementemic form more often is associated with systemic symptoms and has been linked to connective-tissue disease (ie, systemic lupus erythematosus [SLE], relapsing polychondritis). [3, 4, 5, 6, 7] Pathophysiology The pathophysiology of urticarial [emedicine.medscape.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]

[…] 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]