Polymyalgia rheumatica pathophysiology
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Rim Halaby, M.D. [2]
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Overview
Polymyalgia rheumatica (PMR) is a chronic inflammatory disease of the articular and periarticular structures of the cervical region, shoulder girdle and hip girdle. The underlying pathophysiology of PMR remains unknown.[1] It has been hypothesized that genetic and environmental factors are implicated, particularly due to the seasonal and geographical differences in the prevalence of this disease.[2][3][4] It has also been hypothesized that PMR is associated with infections such as parainfluenza virus type 1,[5] mycoplasma pneumoniae, chlamydia pneumoniae, and parvovirus B19.[6] In addition, histological examinations of synovial biopsies of affected individuals reveal mild synovitis with predominance of CD4 T cells and macrophages.[7] Although myalgia is a symptom of PMR, there is no inflammation of the muscles.
Pathophysiology
Pathogenesis
- PMR is a chronic inflammatory disease of the articular and periarticular structures of the cervical region, shoulder girdle and hip girdle. The cause of PMR remains unknown; however, there is evidence in the literature of possible involvement of genetic and environmental factors.[1]This hypothesis is supported by the seasonal and geographical variations of the prevalence of PMR. In fact, the prevalence of PMR is the highest among patients from northern European descent and Scandanavian countries, which suggests a genetic and/or environmental role in the pathophysiology of PMR.
- Several genes have been reported to be involved in PMR, such as:[8][9][10][3][11]
- HLA DRB1
- Interleukin 6
- Interleukin 1 receptor antagonist
- Tumor necrosis factor 3b (TNFb3)
- Moreover, the association between PMR and infections is another hypothesis for the development of PMR through viral stimulation of the immune system. This hypothesis is supported by the highest incidence of PMR in the time of some infection epidemics. Some of the infections that have been linked to PMR are:[5][6]
- In addition to the previous hypotheses, it has been postulated that PMR can be explained by an age related dysregulation in the hypothalamus, pituitary gland and gonads. This disturbance leads to adrenal insufficiency and decrement in dehydroepiandrosterone or androstenedione.[12]
- Imaging studies such as ultrasound and MRI reveal inflammation in the articular and periarticular regions.[1] The examination of histopathological specimens demonstrates mild synovitis with a predominance of T helper cells and macrophages.[7]
Associated conditions
- Polymyalgia rheumatica may be associated with the following conditions:[13][14]
- Giant cell arteritis (temporal arteritis)
- Systemic lupus erythematosus
- Rheumatoid arthritis
Microscopic Pathology
- In cases the polymyalgia rheumatica associated with giant cell arteritits, microscopic histopathological analysis will show the following findings of arteritis:[15][16][17]
- Skip lesions and normal intervening segments
- Intimal thickening, with prominent cellular infiltration
- Lymphocytes in the internal or external elastic lamina or adventitia
- Areas of necrosis may be present in the arterial wall
- Granulomas containing multinucleated histiocytic and foreign body giant cells, helper T-cell lymphocytes, plasma cells, and fibroblasts[18]
References
- ↑ 1.0 1.1 1.2 Kermani TA, Warrington KJ (2013). "Polymyalgia rheumatica". Lancet. 381 (9860): 63–72. doi:10.1016/S0140-6736(12)60680-1. PMID 23051717.
- ↑ Smeeth L, Cook C, Hall AJ (2006). "Incidence of diagnosed polymyalgia rheumatica and temporal arteritis in the United Kingdom, 1990-2001". Ann Rheum Dis. 65 (8): 1093–8. doi:10.1136/ard.2005.046912. PMC 1798240. PMID 16414971.
- ↑ 3.0 3.1 Alvarez-Rodriguez L, Carrasco-Marin E, Lopez-Hoyos M, Mata C, Fernandez-Prieto L, Ruiz-Soto M; et al. (2009). "Interleukin-1RN gene polymorphisms in elderly patients with rheumatic inflammatory chronic conditions: Association of IL-1RN*2/2 genotype with polymyalgia rheumatica". Hum Immunol. 70 (1): 49–54. doi:10.1016/j.humimm.2008.10.011. PMID 19026700.
- ↑ Cimmino MA, Caporali R, Montecucco CM, Rovida S, Baratelli E, Broggini M (1990). "A seasonal pattern in the onset of polymyalgia rheumatica". Ann Rheum Dis. 49 (7): 521–3. PMC 1004141. PMID 2383076.
- ↑ 5.0 5.1 Duhaut P, Bosshard S, Calvet A, Pinede L, Demolombe-Rague S, Dumontet C; et al. (1999). "Giant cell arteritis, polymyalgia rheumatica, and viral hypotheses: a multicenter, prospective case-control study. Groupe de Recherche sur l'Artérite à Cellules Géantes". J Rheumatol. 26 (2): 361–9. PMID 9972970.
- ↑ 6.0 6.1 Elling P, Olsson AT, Elling H (1996). "Synchronous variations of the incidence of temporal arteritis and polymyalgia rheumatica in different regions of Denmark; association with epidemics of Mycoplasma pneumoniae infection". J Rheumatol. 23 (1): 112–9. PMID 8838518.
- ↑ 7.0 7.1 Meliconi R, Pulsatelli L, Uguccioni M, Salvarani C, Macchioni P, Melchiorri C; et al. (1996). "Leukocyte infiltration in synovial tissue from the shoulder of patients with polymyalgia rheumatica. Quantitative analysis and influence of corticosteroid treatment". Arthritis Rheum. 39 (7): 1199–207. PMID 8670331.
- ↑ Haworth S, Ridgeway J, Stewart I, Dyer PA, Pepper L, Ollier W (1996). "Polymyalgia rheumatica is associated with both HLA-DRB1*0401 and DRB1*0404". Br J Rheumatol. 35 (7): 632–5. PMID 8670595.
- ↑ Weyand CM, Hunder NN, Hicok KC, Hunder GG, Goronzy JJ (1994). "HLA-DRB1 alleles in polymyalgia rheumatica, giant cell arteritis, and rheumatoid arthritis". Arthritis Rheum. 37 (4): 514–20. PMID 8147928.
- ↑ Boiardi L, Casali B, Farnetti E, Pipitone N, Nicoli D, Cantini F; et al. (2006). "Relationship between interleukin 6 promoter polymorphism at position -174, IL-6 serum levels, and the risk of relapse/recurrence in polymyalgia rheumatica". J Rheumatol. 33 (4): 703–8. PMID 16583473.
- ↑ Mattey DL, Hajeer AH, Dababneh A, Thomson W, González-Gay MA, García-Porrúa C; et al. (2000). "Association of giant cell arteritis and polymyalgia rheumatica with different tumor necrosis factor microsatellite polymorphisms". Arthritis Rheum. 43 (8): 1749–55. doi:10.1002/1529-0131(200008)43:8<1749::AID-ANR11>3.0.CO;2-K. PMID 10943865.
- ↑ Straub RH, Cutolo M (2006). "Further evidence for insufficient hypothalamic-pituitary-glandular axes in polymyalgia rheumatica". J Rheumatol. 33 (7): 1219–23. PMID 16821261.
- ↑ Caylor TL, Perkins A (2013). "Recognition and management of polymyalgia rheumatica and giant cell arteritis". Am Fam Physician. 88 (10): 676–84. PMID 24364483.
- ↑ Salvarani, Carlo; Cantini, Fabrizio; Hunder, Gene G (2008). "Polymyalgia rheumatica and giant-cell arteritis". The Lancet. 372 (9634): 234–245. doi:10.1016/S0140-6736(08)61077-6. ISSN 0140-6736.
- ↑ Pountain G, Hazleman B (1995). "ABC of rheumatology. Polymyalgia rheumatica and giant cell arteritis". BMJ. 310 (6986): 1057–9. PMC 2549437. PMID 7728064.
- ↑ Weyand CM, Fulbright JW, Hunder GG, Evans JM, Goronzy JJ (2000). "Treatment of giant cell arteritis: interleukin-6 as a biologic marker of disease activity". Arthritis Rheum. 43 (5): 1041–8. doi:10.1002/1529-0131(200005)43:5<1041::AID-ANR12>3.0.CO;2-7. PMID 10817557.
- ↑ Wang AL, Raven ML, Surapaneni K, Albert DM (2017). "Studies on the Histopathology of Temporal Arteritis". Ocul Oncol Pathol. 3 (1): 60–65. doi:10.1159/000449466. PMC 5318845. PMID 28275606.
- ↑ Liozon E, Ly KH, Robert PY (2013). "[Ocular complications of giant cell arteritis]". Rev Med Interne. 34 (7): 421–30. doi:10.1016/j.revmed.2013.02.030. PMID 23523078.