Last reviewed: 19 Sep 2021
Last updated: 05 Nov 2019

Summary

Definition

History and exam

Key diagnostic factors

  • presence of risk factors
  • arthralgia
  • weakness
  • vasculitis
  • lower-extremity vascular purpura

Other diagnostic factors

  • peripheral neuropathy
  • sicca symptoms
  • hypertension
  • Raynaud's phenomenon
  • lower-extremity skin ulcers
  • diplopia
  • ataxia
  • headache
  • confusion
  • retinal haemorrhage/retinal vein thrombosis
  • acrocyanosis

Risk factors

  • hepatitis C virus (HCV) infection
  • other viral infections
  • autoimmune disorders
  • lymphoproliferative disorders

Diagnostic investigations

1st investigations to order

  • fasting qualitative serum cryoglobulins
  • FBC
  • comprehensive blood chemistry profile
  • erythrocyte sedimentation rate, CRP
  • urinalysis
  • ECG
  • CXR
  • rheumatoid factor
  • complement CH50, C3, C4
  • hepatitis C virus (HCV) antibody
  • hepatitis B virus antibody

Investigations to consider

  • HIV antibody
  • antinuclear antibody (ANA), Sjogren's syndrome antigens, extractable nuclear antigen (ENA) antibody
  • skin biopsy

Treatment algorithm

Contributors

Authors

Gurjit S. Kaeley, MBBS, MRCP

Professor of Medicine

Chief, Division of Rheumatology and Clinical Immunology

Medical Director, Musculoskeletal Ultrasound Program

Program Director, Rheumatology Fellowship

University of Florida College of Medicine - Jacksonville

Jacksonville

FL

Disclosures

GSK declares that he has no competing interests.

Peer reviewers

Dina Titova, MD, PhD

Rheumatologist

Road Clinic

Lakewood

WA

Disclosures

DT has received a fee from an educational grant from the Center of Excellence for organizing CME South Sound Bond Club conference.

Antonio Palumbo, MD

Associate Professor

University of Turin

Chief

Myeloma Unit

Ospedale Molinette

Turin

Italy

Disclosures

AP declares that he has no competing interests.

Xavier Leleu, MD, PhD

Instructor in Hematology

Department of Hematology

Hopital Huriez CHRU

Lille

France

Disclosures

XL has received lecture fees and research funding from Janssen-Cilag, Celgene, Chugai, Amgen, Novartis, Mundipharma, and Roche. XL is an author of a number of references cited in this topic.

Rajan Madhok, MD, FCRP

Consultant Physician and Rheumatologist

Centre for Rheumatic Diseases

Glasgow Royal Infirmary

Glasgow

UK

Disclosures

RM declares that he has no competing interests.

Use of this content is subject to our disclaimer