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Polymyalgia rheumatica (which takes its name from the word Πολυμυαλγία "polymyalgia" which means "pain in many muscles" in medical-scientific Greek) abbreviated as PMR, is a syndrome with pain or stiffness, usually in the neck, shoulders, upper arms and hips, but which may occur all over the body. The pain can be very sudden, or can occur gradually over a period. It may be caused by an inflammatory condition of blood vessels such as temporal arteritis.

Most PMR sufferers wake up in the morning with pain in their muscles; however, there have been cases in which the patient has developed the pain during the evenings or have pain and stiffness all day long.^[1] Patients who have polymyalgia rheumatica may also have temporal arteritis, an inflammation of blood vessels in the face which can cause blindness if not treated quickly.

The pain and stiffness can be debilitating if not treated, and can lead to severe depression.

PMR is usually treated with courses of oral corticosteroids.^[2] Most people need to continue the corticosteroid treatment for two to three years.^[3] PMR sometimes goes away on its own in a year or two, but medications and self-care measures can improve the rate of recovery.^[4]

PMR was first established as a distinct disease in 1966 by a case report^[5] on eleven patients at Mount Sinai Hospital in New York, NY.^[6]

Signs and symptoms

There are a wide range of symptoms that indicate if a person has polymyalgia rheumatica. The classic symptoms include:

Pain and stiffness (moderate to severe) in the neck, shoulders, upper arms, thighs and hips, which inhibits activity, especially in the morning/after sleeping. Pain can also occur in the groin area and in the buttocks.
Fatigue and lack of appetite (possibly leading to weight loss) are also indicative of polymyalgia rheumatica.
Anemia
An overall feeling of illness
Low-grade (mild) fever^[7]

About 15% of people who are diagnosed with polymyalgia rheumatica also have temporal arteritis, and about 50% of people with temporal arteritis have polymyalgia rheumatica. Some symptoms of temporal arteritis include headaches, scalp tenderness, jaw or facial soreness, distorted vision or aching in the limbs caused by decreased blood flow, and fatigue.^[1]

Causes

The cause of PMR is not well understood. The pain and stiffness result from the activity of inflammatory cells and proteins that are normally a part of the body's disease-fighting immune system, and the inflammatory activity seems to be concentrated in tissues surrounding the affected joints.^[8] During this disorder, the white blood cells in the body attack the lining of the joints, causing inflammation.^[9] Studies have found that inherited factors also play a role in the probability that an individual will develop polymyalgia rheumatica. Several theories have included viral stimulation of the immune system in genetically susceptible individuals.^[10]

Infectious disease may be a contributing factor. This would be expected with sudden onset of symptoms for example. In addition, appearance of new cases often appear in cycles in the general population, implying a viral connection. Studies are inconclusive, however several somewhat common viruses were identified as possible triggers for PMR.^[8] The viruses thought to be involved include the adenovirus, which causes respiratory infections; the human parvovirus B19, an infection that affects children; and the human parainfluenza virus.^[9] Some sufferers attribute the onset of PMR to stress.

Persons having the HLA-DR4 type of human leucocyte antigen appear to have a higher risk of polymyalgia rheumatica.^[11]

Diagnosis

There is no specific test to diagnose polymyalgia rheumatica. There are many other diseases which cause inflammation and pain in muscles, but there are a few tests that can help narrow down the cause of the pain. Limitation in shoulder motion, or swelling of the joints in the wrists or hands are noted by the doctor.^[10] A patient's answers to questions, a general physical exam, and the results of tests can help a doctor determine the cause of pain and stiffness.^[12]

One blood test that is usually performed is the erythrocyte sedimentation rate (ESR) which measures how fast the patient's red blood cells settle in a test tube. The faster the blood cells settle, the higher the ESR value, which means that there is inflammation. Many conditions can cause an elevated ESR, so this test alone is not proof that a person has polymyalgia rheumatica.^[12]^[13]

Another test that checks the level of C-reactive protein (CRP) in the blood may also be conducted. CRP is produced by the liver in response to an injury or infection and people with polymyalgia rheumatica usually have high levels.^[12]^[13] However, like the ESR, this test is also very non-specific.

Polymyalgia rheumatica is sometimes associated with temporal arteritis, a condition requiring more aggressive therapy. To test for this additional disorder, a biopsy sample may be taken of the temporal artery.^[12]

Treatment

Prednisone is the drug of choice for PMR^[14] and treatment duration is frequently greater than one year.^[10] If the patient does not experience dramatic improvement after three days of 10–20 mg oral prednisone per day, the diagnosis should be reconsidered.^[15]

Non steroidal anti-inflammatory drugs (NSAIDs) such as ibuprofen are ineffective in the initial treatment of PMR,^[16] but they may be used in conjunction with the maintenance dose of corticosteroid.^[17]

Along with medical treatment, patients are encouraged to exercise and eat healthily. Exercise will help strengthen the weak muscles, and help to prevent weight gain. A healthy diet will help to keep a strong immune system, and also help build strong muscles and bones.^[18] Patients are encouraged to eat a diet of fruits, vegetables, whole grains, and low-fat meat and dairy products, avoiding foods with high levels of refined sugars and salt (sodium).^[19]

Epidemiology

There are no certain circumstances for which an individual will get polymyalgia rheumatica, but there are a few factors that show a relationship with the disorder.

Usually only affects adults over the age of 50^[16]
The average age of a person who has PMR is about 70 years old^[1]^[20]
Women are twice as likely to get PMR as men^[20]
Caucasians are more likely to get this disease.^[1] It is more likely to affect people of Northern European origin; Scandinavians are especially vulnerable.^[20]
50% of people with temporal arteritis also have polymyalgia rheumatica^[1]

References

^ ^a ^b ^c ^d ^e Gelfand JL (November 18, 2007). "Polymyalgia Rheumatica and Temporal Arteritis". WebMD. Retrieved 2008-06-10.
^ Polymyalgia rheumatica at eMedicine
^ "Polymyalgia Rheumatica treatments and drugs". MayoClinic. Dec 4, 2010. Retrieved 2012-01-19.
^ "Polymyalgia Rheumatica definition". MayoClinic. May 17, 2008. Retrieved 2012-01-19.
^ Davison S, Spiera H, Plotz CM (Feb 1966). "Polymyalgia rheumatica". Arthritis and Rheumatism 9 (1): 18–23. doi:10.1002/art.1780090103. PMID 4952416.
^ Plotz, Charles; Docken, William (May 2013). "Letters: More on the History of Polymyalgia Rheumatica and Giant Cell Arteritis". The Rheumatologist. ACR/ARHP. Retrieved 2014-06-01.
^ "Polymyalgia Rheumatica symptoms". MayoClinic. Dec 4, 2010. Retrieved 2012-01-19.
^ ^a ^b "Polymyalgia Rheumatica causes". MayoClinic. Dec 4, 2010. Retrieved 2012-01-19.
^ ^a ^b "Polymyalgia Rheumatica causes". MayoClinic. May 17, 2008. Retrieved 2012-01-19.
^ ^a ^b ^c Shiel Jr WC (2008-03-13). "Polymyalgia Rheumatica (PMR) & Giant Cell Arteritis (Temporal Arteritis)". MedicineNet. Retrieved 2008-06-10.
^ Page 255 in: Elizabeth D Agabegi; Agabegi, Steven S. (2008). Step-Up to Medicine (Step-Up Series). Hagerstwon, MD: Lippincott Williams & Wilkins. ISBN 0-7817-7153-6.
^ ^a ^b ^c ^d "Polymyalgia Rheumatica tests and diagnosis". MayoClinic. Dec 4, 2010. Retrieved 2012-01-19.
^ ^a ^b "Polymyalgia Rheumatica tests and diagnosis". MayoClinic. May 17, 2008. Retrieved 2012-01-19.
^ Hernández-Rodríguez J, Cid MC, López-Soto A, Espigol-Frigolé G, Bosch X (Nov 2009). "Treatment of polymyalgia rheumatica: a systematic review". Archives of Internal Medicine 169 (20): 1839–50. doi:10.1001/archinternmed.2009.352. PMID 19901135.
^ McPhee SJ, Papadakis MA (2010). Current Medical Diagnosis and Treatment. p. 767. ISBN 0071624449.
^ ^a ^b Docken WP (August 2009). "Polymyalgia rheumatica". American College of Rheumatology. Retrieved 2012-01-20.
^ "Polymyalgia rheumatica". MDGuidelines. Retrieved 2012-01-20.
^ "Polymyalgia Rheumatica lifestyle and home remedies". MayoClinic. May 17, 2008. Retrieved 2012-01-19.
^ "Polymyalgia Rheumatica lifestyle and home remedies". MayoClinic. Dec 4, 2010. Retrieved 2012-01-19.
^ ^a ^b ^c "Polymyalgia Rheumatica risk factors". MayoClinic. Dec 4, 2010. Retrieved 2012-01-19.

External links

Mayo Clinic PMR factsheets
Questions and Answers about Polymyalgia Rheumatica and Giant Cell Arteritis - US National Institute of Arthritis and Musculoskeletal and Skin Diseases

UpToDate Contents

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1. リウマチ性多発筋痛の臨床症状および診断 clinical manifestations and diagnosis of polymyalgia rheumatica
2. リウマチ性多発筋痛の治療 treatment of polymyalgia rheumatica
3. 巨細胞（側頭）動脈炎の治療 treatment of giant cell temporal arteritis
4. 巨細胞（側頭）動脈炎の臨床症状 clinical manifestations of giant cell temporal arteritis
5. 巨細胞（側頭）動脈炎の診断 diagnosis of giant cell temporal arteritis

English Journal

Google-driven search for big data in autoimmune geoepidemiology: Analysis of 394,827 patients with systemic autoimmune diseases.

Ramos-Casals M1, Brito-Zerón P2, Kostov B3, Sisó-Almirall A3, Bosch X4, Buss D2, Trilla A5, Stone JH6, Khamashta MA7, Shoenfeld Y8.
Autoimmunity reviews.Autoimmun Rev.2015 Aug;14(8):670-679. doi: 10.1016/j.autrev.2015.03.008. Epub 2015 Apr 2.
Systemic autoimmune diseases (SADs) are a significant cause of morbidity and mortality worldwide, although their epidemiological profile varies significantly country by country. We explored the potential of the Google search engine to collect and merge large series (>1000 patients) of SADs report
PMID 25842074

RS3PE: Clinical and Research Development.

Li H1, Altman RD, Yao Q.
Current rheumatology reports.Curr Rheumatol Rep.2015 Aug;17(8):525. doi: 10.1007/s11926-015-0525-0.
Remitting seronegative symmetrical synovitis with pitting edema or RS3PE is a rare elderly-onset rheumatic syndrome. Although there are overlapping clinical manifestations between RS3PE, elderly-onset rheumatoid arthritis, and polymyalgia rheumatica, RS3PE has distinct characteristics. RS3PE can be
PMID 26113011

Ocular Inflammation in the Setting of Concomitant Systemic Autoimmune Conditions in an Older Male Population.

Levitt AE1, McManus KT, McClellan AL, Davis JL, Goldhardt R, Galor A.
Cornea.Cornea.2015 Jul;34(7):762-7. doi: 10.1097/ICO.0000000000000437.
PURPOSE: This retrospective cross-sectional study was designed to investigate the frequency and types of inflammatory ocular manifestations of specific systemic autoimmune diseases in a South Florida Veterans Affairs Hospital population.METHODS: Demographic and medical diagnosis information was extr
PMID 26053887

Japanese Journal

A case of polymyalgia rheumatica following influenza B infection

International Journal of General Medicine 8, 345-347, 2015-10-21
NAID 120005674091

リウマチ性多発筋痛症 (特集一般内科医のためのリウマチ膠原病の診断基準(分類基準)・治療ガイドラインの使い方と注意点)

日本内科学会雑誌 104(10), 2157-2162, 2015-10-10
NAID 40020643495

臨床室化膿性肩関節炎との鑑別を要したリウマチ性多発筋痛症の1例

整形外科 = Orthopedic surgery 66(11), 1159-1162, 2015-10
NAID 40020598296

Related Pictures

★リンクテーブル★

リンク元	「100Cases 27」「100Cases 16」
拡張検索	「polymyalgia rheumatica」「polymyalgia arteritica」

「100Cases 27」

Polymyalgia rheumatica
	In polmyalgia rheumatica (PMR), pain is usually located in the shoulders and hips.
Classification and external resources
ICD-10	M35.3
ICD-9	725
DiseasesDB	10331
MedlinePlus	000415
eMedicine	emerg/473
Patient UK	Polymyalgia rheumatica
MeSH	D011111

　　[★]

☆case27　関節痛

■症例

37歳　女性

主訴：関節痛

現病歴：数ヶ月、膝の痛みがだんだん強くなっていると感じていた。痛みは手や足の小関節に多く、朝歩くときに最もこわばる。痛みはジクロフェナクを飲むと和らぐ。そのほかの症状として疲労感を感じ、最近、体重が３ヶ月で４ｋｇ減っている。

喫煙歴：なし。

飲酒歴：機会飲酒

既往歴：なし

家族歴：既婚、子供は２人

服薬歴：ジクロフェナク

職業歴：legal sevretary

身体所見 examination

　顔貌　青白、臨床的な貧血を認める。近位指節間関節・中手指節関節　腫脹・effusionを伴う疼痛。中足趾節関節　圧痛。それ以外は正常。

検査　 investigation

　血液検査

　　Hb低下

　　ESR 上昇

　　尿素軽度上昇

　　クレアチニン軽度上昇

　　正常：白血球、MCV、血小板、ナトリウム、カリウム、グルコース、アルブミン

　尿検査

　　蛋白(-)、尿糖(-)、潜血(-)、

■問題

　本症例では診断とその根拠を考えるのは簡単なので、鑑別疾患をあげてその疾患に特徴的な症候を列挙しましょう。

■関節痛 DIF.283

　V Vascular

　　血友病 hemophilia　血友病性関節症(急性：疼痛、腫脹、熱感(SOR.241)

慢性：可動域の低下、変形、関節周囲筋萎縮(SOR.241))。家族歴

　　壊血病 scurvy　。生活環境。食事歴

　　無菌性骨壊死 aseptic bone necrosis (Osgood-Schlatter diseaseとか)

　I Inflammatory

　　感染性関節炎(細菌性関節炎(化膿性関節炎・淋菌性関節炎・結核性関節炎・嫌気性菌関節炎 )、真菌性関節炎、スピロヘータ関節炎(梅毒性関節炎・ライム関節炎)、マイコプラズマ関節炎、ウイルス性関節炎)

　N Neoplastic disorders

　　骨原性肉腫 osteogenic sarcoma

　　巨細胞腫 giant cell tumors

　D Degenerative disorders

　　degenerative joint disease

　　変形性関節症 osteoarthritis

　I Intoxication

　　痛風 gout (uric acid)

　　偽痛風 pseudogout (calcium pyrophosphate)

　　ループス症候群 lupus syndrome of hydralazine (Apresoline) and procainamide

　　gout syndrome of diuretics

　C Congenital and acquired malformations bring to mind the joint deformities of tabes dorsalis and syringomyelia and congenital dislocation of the hip. Alkaptonuria is also considered here.

　A Autoimmune indicates

　　関節リウマチ RA

　　血清病 serum sickness

　　全身性エリテマトーデス lupus erythematosus

　　リウマチ熱 rheumatic fever

　　ライター症候群 Reiter syndrome

　　潰瘍性大腸炎 ulcerative colitis

　　クローン病=限局性回腸炎 regional ileitis

　　乾癬性関節炎 psoriatic arthritis

　　リウマチ性多発筋痛症 polymyalgia rheumatica

　T Trauma

　E Endcrine

　　先端肥大症 acromegaly

　　閉経 menopause

　　糖尿病 diabetes mellitus

■関節炎の分類

・炎症部位の数

　単関節炎：痛風、偽痛風、離断性骨軟骨炎、血行性の化膿性関節炎のほとんど

　多関節炎：リウマチ性疾患、ウイルス性疾患、白血病での関節症状、易感染性宿主における血行性の化膿性関節炎

■関節リウマチ

　診断基準

　関節症状

　　朝のこわばり、疼痛、腫脹、関節の動揺、関節可動域制限、変形(手指、足趾、膝関節)。(SOR.211)

　　手指の近位指節間関節(PIP 関節)、中手指節関節(MP 関節)。遠位指節間関節(DIP 関節)に初発することは稀。(SOR.211)

　　左右対称性に生じることが多い。手関節、足趾、膝関節に初発する。(SOR.211)

　関節外症状

　　全身症状：発熱

　　皮膚症状：リウマトイド結節(肘の伸側、後頭部、手指)

　　眼症状：上胸膜炎(10日の経過で治癒)、強膜炎(予後不良)。稀に角膜穿孔

　　血液障害：(高頻度)貧血、白血球減少(Felty病。DMARDs投与中の場合は薬剤性の骨髄抑制を考慮)

　　アミロイドーシス：ネフローゼや下痢を来した症例で疑う。アミロイド蛋白はAA

　　腎障害：稀。アミロイドーシスの続発症か薬剤性を考慮。

　　呼吸器症状：間質性肺炎の合併多い。下肺野に好発。通常無症状。メトトレキセートなどの使用により薬剤性の急性間質性肺炎を生じることがある。

　　心・血管障害：リンパ管炎による難治性の浮腫。

　　神経症状：環軸関節亜脱臼により項部痛や脊髄症上が出現しうる。

　　骨粗鬆症

　　腱鞘滑膜炎：手指、手関節、足関節

■両側性多関節炎の鑑別疾患

　OA：遠位指節間関節を冒すのが特徴的で、近位指節間関節、中手指節関節を冒しうる。

　RA：

　SLE：軽度、症状の程度が変動する非びらん性の関節炎

　gout：単関節炎からはじまる。

　血清陰性関節炎：強直性脊椎炎、感染、ライター病：中～大関節に非対称性の関節炎。仙腸関節と遠位指節間関節にも関節炎

　急性ウイルス性関節炎：風疹。

■KEY POINTS

・RAでは遠位指節間関節は冒されない傾向がある。

・RAの全身症状は関節症状に先行することがある。

・RAの活動性と貧血及びESRは相関している。

・NSAIDsは腎機能に悪影響を与えることがある。

■initial plan

　Dx 1. 単純X線写真

　　　　・亜脱臼、関節周囲の骨萎縮(傍関節性骨骨粗鬆症,juxta-articular osteoporosis)、関節裂隙の狭小化、関節辺縁のびらん(bony erosion)

　　　　　・初期のRAで第5趾に骨びらんが一般的に見られる。

　　　　・血液検査

　　　　　・RF、抗DNA 抗体

　Tx 1. 鎮痛薬

　　　　・NSAIDs：鎮痛とこわばりの軽減

　　 2. DMARDs(メトトレキセート、レフルノミド、金製剤、ペニシラミン)

　　　　・NSAIDsで症状が治まらない場合に考慮

　　 3. 抗TNF 抗体

　　　　・重症のRAで効果がある場合がある。

■参考文献

SOR　標準整形外科学第10版医学書院

DIF　Differential Diagnosis in Primary Care Fourth Edition版 Lippincott Williams & Wilkins

「100Cases 16」

　　[★]

☆case16　膝の痛み

■glossary

indigestion 消化障害、消化不良

■症例

80歳　男性

主訴：左膝の痛みと腫脹

現病歴：左膝の痛みを2日前から認めた。膝は発熱・腫脹しており、動かすと疼痛を生じる。時々胸焼けと消化不良が見られる。6ヶ月前のhealth checkで、高血圧(172/102mmHg)と血中クレアチニンが高い(正常高値)こと以外は正常といわれた。その4週間数回血圧を測定したが、高値が継続したため、2.5mg bendrofluamethizide(UK)/ベンドロフルメチアジド bendroflumethiazide(US)で治療を開始した。最近の血圧は138/84 mmHgであった。

喫煙歴：なし。

飲酒歴：一週間に平均4unit。

既往歴：股関節に中程度(mild)の変形性関節症

家族歴：特記なし

服薬歴：アセトアミノフェン(股関節の疼痛に対して)

身体所見 examination

　血圧 142/86mmHg。体温37.5℃。脈拍88/分。grade 2 hypertensive retinopathy(高血圧症性網膜症)。心血管系、呼吸器系に検査場異常なし。手にDIPにヘバーデン結節なし。

　左膝が発熱し腫脹している。関節内に液、patellar tap陽性。90℃以上膝関節を屈曲させると痛みを生じる。右の膝関節は正常に見える。

検査　 investigation

　生化学：白血球増多、ESR 上昇、尿素高値、グルコース高値

　単純X線：関節間隙やや狭小。それ以外に異常は認めない。

■problem list

　#1 左膝の痛み

　#2 胸焼け

　#3 消化不良

　#4 高血圧

　#5 クレアチニン正常高値

　#6 股関節の変形性リウマチ

　#7 高血圧性網膜症

■考え方

　・関節痛の鑑別診断を考える。

　・VINDICATEで考えてみてもよいでしょう。

　・関節痛の頻度としては　外傷＞慢性疾患(OAなど)＞膠原病＞脊椎疾患＞悪性腫瘍

■関節痛の鑑別疾患

DIF 282

V Vascular 血友病 hemophilia, 壊血病 scurvy, 無菌性骨壊死 aseptic bone necrosis (Osgood-Schlatter diseaseとか)

I Inflammatory 淋疾 gonorrhea, ライム病 lyme disease, 黄色ブドウ球菌 Staphylococcus, 連鎖球菌 Streptococcus, 結核 tuberculosis, 梅毒 syphilis, 風疹 rubella, 単純ヘルペス herpes simplex, HIV human immunodeficiency virus, サイトメガロウイルス cytomegalovirus

N Neoplastic disorders 骨原性肉腫 osteogenic sarcoma, 巨細胞腫 giant cell tumors

D Degenerative disorders degenerative joint disease or 変形性関節症 osteoarthritis

I Intoxication 痛風 gout (uric acid), 偽痛風 pseudogout (calcium pyrophosphate), ループス症候群 lupus syndrome of hydralazine (Apresoline) and procainamide, gout syndrome of diuretics

C Congenital and acquired malformations bring to mind the joint deformities of tabes dorsalis and syringomyelia and congenital dislocation of the hip. Alkaptonuria is also considered here.