Presentation
It is critically important to distinguish skin-limited presentations of CSVV from severe life-threatening systemic vasculitides presenting with CSVV as an initial manifestation. [ncbi.nlm.nih.gov]
This system is based on the association with clinical presentations and underlying etiology [7]. [cureus.com]
Here we present a case of incomplete mixed cryoglobulinemia Type III (as per Brouet's classification) in a young boy aged 13 years who presented with an unusual symptom of pruritus. [ijpmonline.org]
Here we present an interesting, first-ever case report of a 54-year-old Hispanic-American woman with essential mixed cryoglobulinemia presenting with significant digital necrosis in association with membranous nephropathy. [tandfonline.com]
We present a challenging case of idiopathic type III cryoglobulinemia. A 33-year-old man with a history of trismus pseudocamptodactyly syndrome (TPS), chronic pain, and polysubstance abuse presented with several days of worsening rash on both feet. [mushare.marian.edu]
Cardiovascular
- Left Ventricular Dysfunction
Recent data pertaining to the antiviral management in th… Publisher Full Text Severe transient left ventricular dysfunction in a patient with Legionella pneumophila pneumonia. [unboundmedicine.com]
Echocardiography indicated severe left ventricular dysfunction with septal akinesia. The diagnosis was a CV flare involving the skin, gut, kidneys, and heart. [annalsofintensivecare.springeropen.com]
Musculoskeletal
- Tinel's Sign
The examination was significant for bilateral positive Phalen and Tinel's sign, hyperesthesia with weakness on finger extension, and flexion of the lateral three digits. [cureus.com]
Face, Head & Neck
- Malar Rash
However, after 10 months of IFN-α therapy, she developed a photosensitive malar rash, oral ulcers, arthralgias, lymphopenia, and anti-SSA autoantibodies. She was diagnosed with SLE induced by IFN-α therapy. [mayoclinic.pure.elsevier.com]
However, after 10 months of IFN-alpha therapy, she developed a photosensitive malar rash, oral ulcers, arthralgias, lymphopenia, and anti-SSA autoantibodies. She was diagnosed with SLE induced by IFN-alpha therapy. [ncbi.nlm.nih.gov]
Neurologic
- Akinesia
As reported in our patients, echocardiography may reveal diastolic and systolic dysfunction with low cardiac output and segmental dyskinesia or akinesia. [annalsofintensivecare.springeropen.com]
Workup
The patient’s serologic workup revealed evidence of an HCV infection and a positive rheumatoid factor. While the intima is inflamed and edematous, the media is relatively intact without inflammation or necrosis. [arkanalabs.com]
His workup was negative for any thromboembolic cause, with normal ECHO and CT peripheral angiogram showing three-vessel runoff to the ankles bilaterally and no significant stenosis or dissection. [isr.ie]
The patient was admitted for workup for suspected leukocytoclastic vasculitis. Liver biopsy revealed chronic hepatitis, Ishak's grading = 1 + 0 + 1 + 2 = 4 with stage 1 fibrosis [Figure 1]. [ijpmonline.org]
As vasculitis was suspected on initial rheumatologic evaluation, the patient was started on prednisone 60 mg daily as workup commenced. [cureus.com]
Clinical Testing and Workup The key test for mixed cryoglobulinemia is a blood test. When a blood sample is taken, its temperature is 37 Celsius. [rarediseases.org]
Microbiology
- Legionella Pneumophila
Recent data pertaining to the antiviral management in th… Publisher Full Text Severe transient left ventricular dysfunction in a patient with Legionella pneumophila pneumonia. [unboundmedicine.com]
Biopsy
- Renal Biopsy Abnormal
RESULTS: Renal biopsy abnormalities were seen in six females. One patient with PAN showed renal vasculitis and membranoproliferative glomerulonephritis (MPGN) and was HBV and ANA positive. The patient had negative HCV and cryoglobulins. [ncbi.nlm.nih.gov]
Treatment
Type-1 cryoglobulinemic vasculitis (CV) and mixed CV differ in their pathophysiology, clinical expression and treatment response. [ncbi.nlm.nih.gov]
Management and treatment The first-line treatment of MC should focus on eradication of HCV by antiviral treatment (peg-interferon plus ribavirin). [orpha.net]
Prognosis
Prognosis The overall prognosis is poorer in patients with renal disease, liver failure, lymphoproliferative disease and malignancies. The documents contained in this web site are presented for information purposes only. [orpha.net]
What is the prognosis (outlook) for patients with essential mixed cryoglobulinemia? The prognosis and natural history of the illness is not predictable. [medicinenet.com]
Etiology
Hepatitis C virus (HCV) infection is the most frequent cause of mixed cryoglobulinemia vasculitis 80%, while essential or idiopathic cryoglobulinemic vasculitis in the absence of identified etiologic factor represents less than 20%. [onlinelibrary.wiley.com]
Diagnosis is difficult due to various etiologies and clinical manifestations. We present a challenging case of idiopathic type III cryoglobulinemia. [mushare.marian.edu]
Abstract Cryoglobulinemia has an unknown etiology in many cases. In the last years, hepatitis C virus has been known to be involved as a causative agent of so called essential mixed cryoglobulinemia. [ncbi.nlm.nih.gov]
When no significant etiological factors are identified, it is called essential mixed cryoglobulinemia. A detailed and thorough laboratory investigation is required to exclude all possible causes of cryoglobulin formation. [tandfonline.com]
Epidemiology
Skin and glomeruli are often involved [ 1 ]. 14.3 Epidemiology The epidemiology of cryoglobulinemic vasculitis has not been well described. [musculoskeletalkey.com]
A causative role of hepatitis C virus (HCV) in over 4/5 patients has been definitely established on the basis of epidemiological, pathological, and laboratory studies. [ncbi.nlm.nih.gov]
Summary Epidemiology It is considered to be a rare disorder, but its true prevalence remains unknown. The disease is more common in Southern Europe than in Northern Europe or Northern America. [orpha.net]
Pathophysiology
Type-1 cryoglobulinemic vasculitis (CV) and mixed CV differ in their pathophysiology, clinical expression and treatment response. [ncbi.nlm.nih.gov]
Stone Springer Science & Business Media, ٠٣/١٠/٢٠٠٩ - 493 من الصفحات Important strides have been made in understanding the pathophysiologic basis of many inflammatory conditions in recent years, but rheumatology remains a discipline in which diagnosis [books.google.com]
[…] resolubilize upon warming Low concentrations may occur in apparently healthy individuals Clinical Presentation Cryoglobulinemic vasculitis Most common with types II and III Predominantly small vessel involvement – capillaries, venules, or arterioles Pathophysiology [arupconsult.com]
Our case adds to the existing small body of literature on intestinal cryoglobulinemic vasculitis and highlights the pathophysiology of this complex, viral-mediated immunologic mechanism and its management. [acgcasereports.gi.org]
NS3 protein was also detected in 6 patients: 5 were positive in T cells, 3 in B cells, and 3 in monocytes. [7] Pathophysiology The mechanisms of cryoprecipitation are poorly understood, but several factors have been investigated. [emedicine.medscape.com]
Prevention
Prevention There is no known prevention for the condition. Staying away from cold temperatures may prevent some symptoms. Testing and treatment for hepatitis C infection will reduce your risk. Images References Patterson ER, Winters JL. [ufhealth.org]
Prevention There is no known prevention for the condition. Staying away from cold temperatures may prevent some symptoms. Testing and treatment for hepatitis C infection will reduce your risk. References Patterson ER, Winters JL. Hemapheresis. [mountsinai.org]
Prevention Getting treatment for hepatitis C before late-stage disease sets in, and before cryoglobulins form in the blood is the best way to prevent cryoglobulinemia. Need help now? Check out our resources page here. [hepatitisc.net]
Identification and treatment of early symptoms such as skin lesions, fatigue or arthralgia, as a result of cryoglobulinaemia, will prevent further organ damage and improve patient outcomes. [dermnetnz.org]
Such treatment is usually effective in preventing further damage but has potentially serious side effects and requires regular monitoring by a doctor familiar with these medications. [vasculitisfoundation.org]