cryoglobulinemia (cryoglobulinemic vasculitis)
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Classification
See individual types:
Etiology
- hepatitis C (mixed cryoglobulinemia) most common
- HIV infection
- lymphoproliferative disorder, lymphoma, leukemia
- plasma cell dyscrasia
- systemic lupus erythematosus
- rheumatoid arthritis
- vasculitis
Pathology
Clinical manifestations
- cutaneous symptoms (100%)[6]
- vascular purpura 55%, palpable purpura
- erythematous macules[3]; not maculopapular rash[3]
- lower extremities
- distal necrosis 14% (types 1 & 2)
- digital ischemia, earlobe infarctions[3]
- urticaria 10% (types 1 & 3)
- livedo reticularis 8-14% (type 3)
- leg ulcers 5% (types 1 & 3)
- skin nodules or skin ulcers
- Raynaud's phenomenon 50%
- acrocyanosis 9% (types 1 & 2 > type 3)
- articular manifestations 35-70% (type 3 > 2 > 1)[6]
- renal symptoms 21-55% (type 2 > 1 > 3) [6]
- neurologic symptoms 17% (type 3 > 1 > 2)
- pulmonary crackles less common[10]
- hemorrhage 7% (types 1 & 2, 1 > 2)
- abdominal pain 2-5% (type 3)
- arterial thrombosis 1-5% (type 1)
- lymphadenopathy
- hepatosplenomegaly[5]
- not associated with acute febrile onset[3]
Laboratory
- blood collection & specimen processing
- blood collected in pre-warmed tubes
- kept at 37 degrees during clotting to keep cryoglobulins from precipitating with clot
- serum separated from clot at 37 degrees
- incubation at 4 degrees C for 72 hours
- cryoglobulins in serum/plasma
- trace amounts of cryoglobulins may be found in normal individuals
- serum protein electrophoresis to identify cryoglobulin as monoclonal or polyclonal
- serum complement levels are generally decreased (type 2 & 3), serum complement C4 more than serum complement C3
- rheumatoid factor strongly positive (type 2 & 3)
- serum transaminases may be elevated
- hepatitis serology
- urinalysis with urine microscopy
- active urinary sediment, erythrocyte casts,
- see ARUP consult[7]
Management
- therapy directed at underlying disease
- idiopathic or essential cryoglobulinemia
- prednisone
- other immunosuppressive agents
- short course of therapy
- hepatitis C-associated cryoglobulinemia
- ledipasvir & sofosbuvir for treatment of hepatitis C
- 90% effective[3]
- peginterferon-alfa (Pegasys, Pegintron) + ribavirin
- older recommenation, 75% effective
- disease may recur if antiviral therapy discontinued & hepatitis C virus not eradicated[3]
- ledipasvir & sofosbuvir for treatment of hepatitis C
- hyperviscosity responds to plasmapheresis
More general terms
More specific terms
- mixed cryoglobulinemia
- type 1 (monoclonal) cryoglobulinemia
- type 2 (mixed) cryoglobulinemia
- type 3 (mixed) cryoglobulinemia
Additional terms
References
- ↑ Manual of Medical Therapeutics, 28th ed, Ewald & McKenzie (eds), Little, Brown & Co, Boston, 1995, pg 527
- ↑ Mayo Internal Medicine Board Review, 1998-99, Prakash UBS (ed) Lippincott-Raven, Philadelphia, 1998, pg 849
- ↑ 3.00 3.01 3.02 3.03 3.04 3.05 3.06 3.07 3.08 3.09 3.10 3.11 3.12 3.13 3.14 3.15 Medical Knowledge Self Assessment Program (MKSAP) 11, 14, 15, 16, 17, 18, 19. American College of Physicians, Philadelphia 1998, 2006, 2009, 2012, 2015, 2018, 2022.
Medical Knowledge Self Assessment Program (MKSAP) 19 Board Basics. An Enhancement to MKSAP19. American College of Physicians, Philadelphia 2022 - ↑ Harrison's Principles of Internal Medicine, 13th ed. Isselbacher et al (eds), McGraw-Hill Inc. NY, 1994, pg 1307
- ↑ 5.0 5.1 Brahn E, Scoville CD Biochemical markers of disease activity. Baillieres Clin Rheumatol 1988 2:153 PMID: https://www.ncbi.nlm.nih.gov/pubmed/2458192
- ↑ 6.0 6.1 6.2 6.3 Khasnis A and Langford CA. Update on vasculitis. J Allergy Clin Immunol 2009 Jun; 123:1226. PMID: https://www.ncbi.nlm.nih.gov/pubmed/19501230
- ↑ 7.0 7.1 ARUP Consult: Cryoglobulinemia The Physician's Guide to Laboratory Test Selection & Interpretation https://www.arupconsult.com/content/cryoglobulinemia
- ↑ Ramos-Casals M, Stone JH, Cid MC, Bosch X. The cryoglobulinaemias. Lancet. 2012 Jan 28;379(9813):348-60 PMID: https://www.ncbi.nlm.nih.gov/pubmed/21868085
- ↑ Cacoub P, Comarmond C, Domont F, Savey L, Saadoun D. Cryoglobulinemia Vasculitis. Am J Med. 2015 Sep;128(9):950-5. Review. PMID: https://www.ncbi.nlm.nih.gov/pubmed/25837517
- ↑ 10.0 10.1 NEJM Knowledge+ Hematology