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Idiopathic Cryoglobulinemia


Presentation

  • Leading experts from Latin America, Africa, Near and Middle East, Indian Subcontinent, Far East, Oceania and Australia present their expert insights into specific conditions, as well as progress and challenges in the development of the specialty.[books.google.de]
  • Structured chapter template presents information in a consistent format. ICD-9 codes in all disease chapters assist in diagnostic coding. Fast Fact Guide to Patient Assessment on the inside covers puts vital facts at your fingertips.[books.google.com]
  • The data confirmed that the majority of patients presented urinary abnormality, while Nephrotic Syndrome was present in about 30% of cases and Nephrotic Syndrome in 10% (8 - 10).[uninet.edu]
Pediatric Disorder
  • All new reorganization —a real time saver for the busy trainee or clinician More consistent presentation for greater accessibility New entries on pediatric disorders to help treat your young patients The newest anti-rheumatic drugs, including celecoxib[books.google.com]
Abdominal Pain
  • When the gastrointestinal tract is involved, sudden, severe abdominal pain (acute abdomen) may be the initial symptom.[rarediseases.org]
  • Patients usually have constitutional symptoms such as arthralgias or abdominal pain.[aafp.org]
  • Extrarenal signs of the disease (artralgya, abdominal pain and purpura) were extremely more frequent in group C (80% of pts), again underlying a more active disease in this group of cases with more severe renal damage.[uninet.edu]
  • Abdominal pain (20%) is best explained by vasculitis of the gastrointestinal tract. Membranoproliferative glomerulonephritis (MPGN) is found in many patients, although the prevalence is difficult to estimate.[ard.bmj.com]
  • pain, hepatosplenomegaly, abnormal liver enzymes B.[clinicaladvisor.com]
Acrocyanosis
  • Features of Type I cryoglobulinaemia Features of Type II and III cryoglobulinaemia Acrocyanosis (blueness of the hands and feet from the cold Blue toe syndrome Retinal haemorrhage (bleeding) Severe Raynaud phenomenon Arterial thrombosis Gangrene Arthralgia[dermnetnz.org]
  • Interruption of blood flow to other tissues in type I disease can cause cutaneous manifestations of purpura, blue discoloration of the arms or legs (acrocyanosis), necrosis, ulcers, and livedo reticularis ; spontaneous nose bleeds, joint pain, membranoproliferative[en.wikipedia.org]
  • Use cautiously with Raynaud's disease, chilblains, acrocyanosis, erythrocyanosis, or impaired temperature sensitivity, such as neuropathy. Use cautiously if pregnant or breastfeeding.[natural-pantry.com]
Purpura
  • Henoch-Schönlein purpura. D. Idiopathic thrombocytopenic purpura. E. Schamberg's disease. Discussion The answer is B: essential mixed cryoglobulinemia.[aafp.org]
  • D69.2 ICD-10-CM Diagnosis Code D69.2 Other nonthrombocytopenic purpura 2016 2017 2018 2019 Billable/Specific Code Applicable To Purpura NOS Purpura simplex Senile purpura cryoglobulinemic D89.1 Pyelonephritis - see also Nephritis, tubulo-interstitial[icd10data.com]
  • Cryoglobulinemia (essential) (idiopathic) (mixed) (primary) (purpura) (secondary) (vasculitis) (D89.1) Cryoglobulinemia (essential) (idiopathic) (mixed) (primary) (purpura) (secondary) (vasculitis) (D89.1) Equivalent ICD-9 Code GENERAL EQUIVALENCE MAPPINGS[icd.codes]
  • […] cryoglobulinemia [ kri″o-glob″u-lin-e me-ah ] the presence of cryoglobulin in the blood, associated with a variety of clinical manifestations including Raynaud's phenomenon, vascular purpura, cold urticaria, necrosis of extremities, bleeding disorders[medical-dictionary.thefreedictionary.com]
  • ., antiphospholipid syndrome, thrombotic thrombocytopenic purpura, hemolytic uremic syndrome, atrial myxoma) Cryoglobulinemic hyperviscosity syndrome (type I) Proliferative: polyclonal/monoclonal gammopathy (Waldenstrom hyperglobulinemic purpura, Waldenstrom[clinicaladvisor.com]
Palpable Purpura
  • Cryoglobulin deposition causes a vasculitis that leads to petechiae and palpable purpura.[aafp.org]
  • Cutaneous vasculitis with palpable purpura, especially in the lower extremities, should suggest cryoglobulinemia. Biopsy of the rash commonly demonstrates leukocytoclastic vasculitis.[rheumaknowledgy.com]
  • A physical examination revealed the presence of palpable purpura in the lower extremities; the rest of the exam was normal.[revistanefrologia.com]
  • Skin problem Type I (%) Types II and III (%) Ischaemic necrosis (skin and tissue damage due to lack of blood flow) 40 0-20 Palpable purpura 15 80 Livedoid vasculopathy 1 14 (in type III) Cold-induced urticaria 15 10 (in type III) Scarring of the tip of[dermnetnz.org]
  • The classical presentation is “Meltzer’s triad” of palpable purpura, arthralgias and weakness (present in 25-30% and up to 80% at the disease onset).[clinicaladvisor.com]
Urticaria
  • Patients with concomitant hepatitis C infection may also need antiviral therapy. 6 Cold urticaria lesions are erythematous wheals with central pallor.[aafp.org]
  • […] cryoglobulinemia [ kri″o-glob″u-lin-e me-ah ] the presence of cryoglobulin in the blood, associated with a variety of clinical manifestations including Raynaud's phenomenon, vascular purpura, cold urticaria, necrosis of extremities, bleeding disorders[medical-dictionary.thefreedictionary.com]
  • Skin problem Type I (%) Types II and III (%) Ischaemic necrosis (skin and tissue damage due to lack of blood flow) 40 0-20 Palpable purpura 15 80 Livedoid vasculopathy 1 14 (in type III) Cold-induced urticaria 15 10 (in type III) Scarring of the tip of[dermnetnz.org]
  • Other skin manifestations include urticaria, Raynaud’s phenomenon with sometimes acral necrosis.[ard.bmj.com]
  • Although small petechial lesions are typically seen on the skin, other manifestations have been described, including Raynaud’s phenomenon, livedo reticularis, urticaria, and edema.[intechopen.com]
Hematuria
  • After receiving the above-mentioned treatment, her creatinine went back to normal with resolution of proteinuria and hematuria, normalization of serum complements, and significant improvement in her clinical picture.[ncbi.nlm.nih.gov]
  • Treatment with IV methylprednisolone, followed by oral prednisone and Rituximab was initiated with resolution of hematuria and normalization of creatinine.[omicsonline.org]
  • Repeat urine sediment, analyzed by phase-contrast microscopy, showed severe microscopic hematuria ( 50 erythrocytes/microscopic field), many dysmorphic erythrocytes and casts (ialine, granular and red cell casts).[wjgnet.com]
  • The renal symptoms typically consisted of mild proteinuria, microscopic hematuria, and hypertension [ 24 – 27 ]. More than 50% of patients had mild renal failure [ 24 – 27 ].[annalsofintensivecare.springeropen.com]
Peripheral Neuropathy
  • Nonsteroidal anti-inflammatory drugs (NSAIDs) Interferon Immunosuppressive drugs (Cytoxan ) Plasmapheresis Corticosteroids For peripheral neuropathy: Discontinue drugs that cause peripheral neuropathy Institute non-drug treatments to reduce pain, such[peripheralneuropathycenter.uchicago.edu]
  • Affected individuals may experience damage to the nerves outside the central nervous system (peripheral neuropathy).[rarediseases.org]
  • neuropathy Fever may also occur.[dermnetnz.org]
  • One patient showed an improvement of peripheral neuropathy after induction with the combined therapy.[link.springer.com]

Workup

  • Urine analysis also is an essential component of the workup to assess for active sediment, hematuria and proteinuria.[healio.com]
Human Papillomavirus
  • ., Human papillomavirus types 6 and 1 1 DNA sequences in genital and laryngeal papillomas and in some cervical cancers, ‏ الصفحة 477 - Lane, HC, Depper, JM, Greene, WC, Whalen, G., Waldmann, TA, and Fauci, AS, Qualitative analysis of immune function in[books.google.com]
  • Other such viruses include human papillomavirus (HPV), associated with cervical, ano-genital, skin and head and neck cancers [ 97 ]; human T-cell leukemia virus type 1 (HTLV-1), which causes adult T-cell leukemia [ 98 ]; and human herpes virus type 8[intechopen.com]

Treatment

  • We conclude that rituximab could be an effective treatment for idiopathic cryoglobulnemia.[ncbi.nlm.nih.gov]
  • However, treatment of severe vasculitis is traditionally based on a combination of corticosteroids and immunosuppressants or plasmapheresis, and more recently rituximab .[journals.lww.com]
  • This quick-consult covers the full scope of rheumatologic diagnosis and treatment... A wide range of diagnostic tests —including ANA, DNA, RF, ANCA and CCP antibody tests—show you what test to order and how to interpret the results.[books.google.com]
  • Treatment Table 1. Table 1. Characteristics of the Patients at Entry into the Study.[nejm.org]

Prognosis

  • Clinical observations, with special reference to factors influencing prognosis, Rev. Infect.[books.google.com]
  • What is the prognosis (outlook) for patients with essential mixed cryoglobulinemia? The prognosis and natural history of the illness is not predictable.[medicinenet.com]
  • PROGNOSIS AND OUTCOME • Roughly half of patients have chronic cryoglobulinemic disease with no involvement of vital organs.[slideshare.net]
  • Additionally, several different studies have shown that lymphomatous infiltration of the kidney is associated with a poor prognosis from a haematological point of view. 2 However, there are other patterns of renal damage associated with lymphomas, such[revistanefrologia.com]
  • […] process determines the prognosis.[ard.bmj.com]

Etiology

  • ., An assay for circulating antibodies to a major etiologic virus of human non-A, non-B hepatitis, Science, 244, 362, 1989. ‏[books.google.com]
  • Certain conditions have both an underlying etiology and multiple body system manifestations due to the underlying etiology.[icd10coded.com]
  • Etiology Genetic Disease Carbonic Anhydrase I (CA-I) Deficiency/Alteration Ehlers-Danlos Syndrome (see Ehlers-Danlos Syndrome, [[Ehlers-Danlos Syndrome]]) Familial Type 1 Distal Renal Tubular Acidosis Autosomal Dominant Autosomal Recessive Hereditary[mdnxs.com]
  • Etiology Cryoglobulinemias are associated with a variety of illnesses (listed below), three most common categories being: infections, autoimmune disorders, and malignancies.[clinicaladvisor.com]
  • The term “essential” defines cryoglobulinemic syndromes without an underlying identifiable etiology.[intechopen.com]

Epidemiology

  • […] with low initial concentrations of M-protein Usually associated with some treatment Only 1% disappeared spontaneously No associated systemic disorders: 65% Monoclonal gammopathy of unknown significance (MGUS) Light chain: kappa more often than lambda Epidemiology[neuromuscular.wustl.edu]
  • It is considered to be a rare disorder, however, yet there are no adequate epidemiological studies regarding its overall prevalence.[ojrd.biomedcentral.com]
  • OBJECTIVES • Discovery of cryoglobulins, definitions and classification • Discussion of etiology and epidemiology • Understanding of pathogenesis of cryoglobulinemic vasculitis and role of HCV • Clinical features and renal involvement • Laboratory diagnosis[slideshare.net]
  • Of note, in HCV-related disease, HCV-related proteins are thought to play a direct role in pathogenesis and are present in damaged skin, blood vessels, and kidneys. [15, 17, 18, 19] Epidemiology Frequency United States Cryoglobulins are reported in otherwise[emedicine.medscape.com]
  • Together, these data support epidemiological observations of an association between HCV chronic infection and B-NHL.[intechopen.com]
Sex distribution
Age distribution

Pathophysiology

  • Brenner/Rector remains the go-to resource for practicing and training nephrologists and internists who wish to master basic science, pathophysiology, and clinical best practices.[books.google.de]
  • N Engl J Med 1988; 318 594-599 A modified classification of metabolic acidosis: a pathophysiological approach. Nephron 1992; 60:129-133[mdnxs.com]
  • 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]
  • More vasculitis Pathophysiology Impairment of vasa nervorum microcirculation Intravascular deposition of cryoglobulin Ischemia Necrotizing vasculitis Longstanding cryoglobulin precipitation Complement fixation Rheumatoid factor activity Vessels involved[neuromuscular.wustl.edu]
  • Though the pathophysiological mechanisms leading to the progressive decline of kidney function in the setting of membranoproliferative glomerulonephritis involve a myriad of factors, cryoglobulin-containing immune complex deposition is an inciting event[omicsonline.org]

Prevention

  • Other condition that relates to cryoglobulinemia may include; Multiple myelomas, Primary macroglobulinemia, Leukemia, Rheumatoid arthritis, Systemic lupus erythematosus, and Mycoplasma pneumonia Prevention The condition can be prevented by minimizing[healthmagreviews.com]
  • 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. See smartphone apps to check your skin.[dermnetnz.org]
  • Tea tree oil : Tea tree oil is purported to have antiseptic properties, and has been used traditionally to prevent and treat infections.[natural-pantry.com]

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