Cryoglobulinemic vasculitis

disease

On this page

Also known as essential cryoglobulinemiaessential mixed cryoglobulinemiafamilial mixed cryoglobulinemiaMCMixed Cryoglobulinemiaprimary cryoglobulinemia

Summary

Cryoglobulinemic vasculitis (MONDO:0007407) is a disease and 9 clinical trials. Top therapeutic interventions include isatuximab and rituximab. A subtype of immune complex mediated vasculitis — broader associated-gene and molecular evidence is on the parent page (see Disease family below).

At a glance

Prevalence: 1-9 / 100 000 (United States) [Orphanet-validated]
Phenotypes (HPO): 29
Clinical trials: 9

Clinical features

Epidemiology

Prevalence records

1 prevalence record(s), Orphanet:

Type	Class	Value	Geography	Validation
Point prevalence	1-9 / 100 000	1	United States	Validated

Signs & symptoms

Clinical features (HPO)

29 HPO clinical features (Orphanet curated; top 29 by frequency):

HPO ID	Term	Frequency
HP:0000965	Cutis marmorata	Very frequent (80-99%)
HP:0000967	Petechiae	Very frequent (80-99%)
HP:0000979	Purpura	Very frequent (80-99%)
HP:0001324	Muscle weakness	Very frequent (80-99%)
HP:0001945	Fever	Very frequent (80-99%)
HP:0002633	Vasculitis	Very frequent (80-99%)
HP:0012224	Circulating immune complexes	Very frequent (80-99%)
HP:0100721	Mediastinal lymphadenopathy	Very frequent (80-99%)
HP:0100778	Cryoglobulinemia	Very frequent (80-99%)
HP:0200042	Skin ulcer	Very frequent (80-99%)
HP:0000083	Renal insufficiency	Frequent (30-79%)
HP:0000093	Proteinuria	Frequent (30-79%)
HP:0000790	Hematuria	Frequent (30-79%)
HP:0001369	Arthritis	Frequent (30-79%)
HP:0001392	Abnormality of the liver	Frequent (30-79%)
HP:0001744	Splenomegaly	Frequent (30-79%)
HP:0002027	Abdominal pain	Frequent (30-79%)
HP:0002240	Hepatomegaly	Frequent (30-79%)
HP:0002829	Arthralgia	Frequent (30-79%)
HP:0003326	Myalgia	Frequent (30-79%)
HP:0005244	Gastrointestinal infarctions	Frequent (30-79%)
HP:0006562	Viral hepatitis	Frequent (30-79%)
HP:0007141	Sensorimotor neuropathy	Frequent (30-79%)
HP:0009830	Peripheral neuropathy	Frequent (30-79%)
HP:0009831	Mononeuropathy	Frequent (30-79%)
HP:0100758	Gangrene	Frequent (30-79%)
HP:0100820	Glomerulopathy	Frequent (30-79%)
HP:0001097	Keratoconjunctivitis sicca	Occasional (5-29%)
HP:0002239	Gastrointestinal hemorrhage	Occasional (5-29%)

Identifiers

Disease identifiers

Field	Value
Canonical name	Cryoglobulinemic vasculitis
Mondo ID	MONDO:0007407
MeSH	C565141
OMIM	123550
Orphanet	91138
ICD-11	55133785
SNOMED CT	190815001
UMLS	C1852456
MedGen	343814
GARD	0006386
MedDRA	10027756
NORD	1452
Is cancer (heuristic)	no

Also known as: essential cryoglobulinemia · essential mixed cryoglobulinemia · familial mixed cryoglobulinemia · MC · Mixed Cryoglobulinemia · mixed cryoglobulinemia · primary cryoglobulinemia

Disease family

This is a subtype of immune complex mediated vasculitis. Genetic, therapeutic, and trial evidence is largely curated at the broader-term level — see the parent page for the associated-gene cohort and molecular evidence.

Classification path: disease › human disease › disease by body system or component › cardiovascular disorder › vascular disorder › vasculitis › immune complex mediated vasculitis › Cryoglobulinemic vasculitis

Subtypes (2): type II mixed cryoglobulinemia, mixed cryoglobulinemia type III

Genetics & variants

GWAS landscape

No GWAS associations recorded — common-variant (GWAS) studies don’t cover this disease (typical for Mendelian / rare diseases). See the curated gene cohort and Mendelian overlap below.

Variant details and genetic-evidence tiers

No tiered GWAS variants or ClinVar records for this disease.

Genes & proteins

No associated-gene cohort resolved for this disease. Atlas builds the molecular and therapeutic sections — associated genes, protein families, druggability, pathways, interactions, and drug associations — by aggregating over a disease’s associated genes (resolved via GWAS / GenCC / ClinVar / CIViC), and none resolved here. This is expected for antibody-mediated, autoimmune, or otherwise non-gene-defined conditions; the curated evidence for this disease is its clinical features, GWAS susceptibility, and clinical trials (above).

Function

No pathway enrichment — requires an associated-gene cohort.

Therapeutics

No druggable-target or therapeutic data for this disease’s cohort.

Clinical trials & evidence

Clinical trials

Clinical trials: 9.

Phase distribution (across all retrieved trials)

Phase	Trials
Not specified	6
PHASE2	3

Top trials by phase / activity

NCT	Phase	Status	Title
NCT07268521	PHASE2	NOT_YET_RECRUITING	A Multicenter Phase 2 Single-arm Proof-of-concept Trial Assessing the Efficacy and Safety of Obinutuzumab in the Treatment of Non-infectious Active Cryoglobulinemia Vasculitis Refractory or Intolerant to Rituximab
NCT05114109	PHASE2	UNKNOWN	Isatuximab in Type I Cryoglobulinemia
NCT05168475	PHASE2	TERMINATED	Biologics in Refractory Vasculitis: A Trial of Biologic Therapy for Refractory Primary Non-ANCA Associated Vasculitis
NCT02593565	Not specified	RECRUITING	Vasculitis Pregnancy Registry
NCT03004326	Not specified	RECRUITING	Clinical Transcriptomics in Systemic Vasculitis (CUTIS)
NCT02476292	Not specified	COMPLETED	Impact of Vasculitis on Employment and Income
NCT03342261	Not specified	COMPLETED	Benefit of DAA Therapy in HCV Monoinfected and HIV-HCV Coinfected Patients With Mixed Cryoglobulinemia
NCT03410290	Not specified	COMPLETED	Journey of Patients With Vasculitis From First Symptom to Diagnosis
NCT04692363	Not specified	UNKNOWN	Plasmapheresis Before Rituximab in Cryoglobulinemia

Drugs tested across these trials (top 30)

Molecule	Max phase	Trials referencing
ISATUXIMAB	4	1
RITUXIMAB	4	1

Drugs: Isatuximab, Rituximab