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Cryoglobulinemia

Cryoglobulinemia is a condition in which abnormal antibodies or immune complexes called cryoglobulins clump in the cold and trigger immune‑mediated inflammation of small and medium blood vessels. It often causes skin purpura, joint pain, nerve symptoms, and sometimes kidney damage, and treatment targets the cause while controlling inflammation.

Prevalence

unknown

N/A

US Estimated

N/A

Europe Estimated

Age of Onset

adults - older adults

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ICD-10

D89.1

Inheritance Pattern

Autosomal dominant

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Autosomal recessive

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Mitochondrial/Multigenic

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X-linked dominant

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X-linked recessive

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5 Facts you should know

FACT

1

Occurs when an abnormal immune system makes the protein cryoglobulin

FACT

2

At temperatures less than 98.6 degrees Fahrenheit, cryoglobulins become solid or gel-like and can block blood vessels

FACT

3

Symptoms may include skin ulcers, purpura Raynaud's phenomenon), joint pain, and kidney problems

FACT

4

Kidney disease - primarily membranoproliferative glomerulonephritis – occurs in about 1 in 3 patients

FACT

5

Cryoglobulinemia is also associated with immune system cancers, such as multiple myeloma or non-Hodgkins lymphoma

Cryoglobulinemia is also known as...

Cryoglobulinemia is also known as:

  • Cryoglobulinemic vasculitis

  • Cryoglobulinemic syndrome

What’s your Rare IQ?

Cryoglobulinemia is a disorder characterized by the presence of abnormal proteins in the blood that can form gel-like deposits in blood vessels. What is a common symptom associated with cryoglobulinemia?

 

Common signs & symptoms

Palpable purpura

Arthralgia

Weakness (fatigue)

Skin

Livedo reticularis, ulcers, digital ischemia

Renal

Membranoproliferative glomerulonephritis

Proteinuria, hematuria, renal insufficiency

Neurologic

Peripheral neuropathy (sensory > motor)

Systemic associations

Chronic hepatitis C

B-cell lymphoproliferative disorders

Autoimmune disease

Current treatments

Treat underlying cause

 

Direct-acting antivirals for HCV-associated disease

Immunosuppression

Rituximab ± corticosteroids for moderate–severe disease

Cyclophosphamide in life-threatening vasculitis

Severe / organ-threatening disease

Plasmapheresis for rapidly progressive renal or neurologic involvement

Supportive

Blood pressure control, renal protection, skin care