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Are There Definite Disease Subsets in Polymyalgia Rheumatica? Suggestions from a Narrative Review.

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Summary
This summary is machine-generated.

This study identified seven subsets of polymyalgia rheumatica (PMR), distinguishing true PMR from mimicking conditions. Findings aid in accurate diagnosis, influencing treatment and health policies for PMR patients.

Keywords:
calcium pyrophosphate deposition diseasechondrocalcinosisgiant cell arteritisimmune checkpoint inhibitor drugsinfectionpolymyalgia rheumaticasubsetultrasoundvaccination

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Area of Science:

  • Rheumatology
  • Internal Medicine
  • Clinical Immunology

Background:

  • Polymyalgia rheumatica (PMR) presents with varied onset and course, making differentiation from polymyalgic syndromes challenging.
  • Identifying distinct PMR subsets is crucial for accurate diagnosis, treatment, prognosis, and health policy development.
  • Distinguishing true PMR from PMR-mimicking conditions requires careful clinical evaluation.

Purpose of the Study:

  • To identify and characterize distinct subsets or clusters of polymyalgia rheumatica (PMR) in the scientific literature.
  • To describe PMR subsets supported by findings from at least two independent studies.
  • To differentiate true PMR subsets from PMR-mimicking conditions.

Main Methods:

  • A non-systematic literature search was conducted on Embase and Medline using keywords related to PMR, subsets, mimicking conditions, and potential triggers.
  • Reference lists of relevant articles were scanned for additional publications.
  • Full-length review of 103 articles was performed, with 84 assessed for eligibility.

Main Results:

  • Seven major subsets of PMR were identified: normal acute-phase reactants, infection trigger, vaccination trigger, subclinical giant cell arteritis (GCA), calcium pyrophosphate deposition disease (CPPD), immune checkpoint inhibitor (ICI) therapy-related, and peculiar clinical clusters.
  • PMR with normal acute-phase reactants, and PMR triggered by infection or vaccination, are considered distinct disease subsets.
  • PMR with subclinical GCA and most PMR/CPPD cases are classified as PMR mimickers.

Conclusions:

  • PMR with normal baseline acute-phase reactants and those triggered by infection or vaccination represent valid disease subsets.
  • PMR associated with subclinical GCA and CPPD are largely considered PMR mimickers.
  • Further research is needed to clarify peculiar clinical subsets and ICI-induced PMR.