{{Short description|Recurrent inflammation and clotting of blood vessels in the hands and feet}} {{MCN|date=May 2024}} {{Redirect-distinguish|Buerger disease|Berger's disease}} {{Infobox medical condition (new) | name = | synonyms = Buerger disease, Buerger's disease, Winiwarter-Buerger disease, presenile gangrene<ref name=ferri>{{cite book |last=Ferri |first=Fred F. |name-list-style = vanc |year=2003 |title=Ferri's Clinical Advisor 2004: Instant Diagnosis and Treatment |edition=6th |isbn=978-0-323-02668-0|page=840}}</ref> | image = M.Buerger 1.JPG | caption = Complete occlusion of the right and stenosis of the left [[femoral artery]] as seen in a case of thromboangiitis obliterans | pronounce = | field = | symptoms = | complications = | onset = | duration = | types = | causes = | risks = | diagnosis = | differential = | prevention = | treatment = | medication = | prognosis = | frequency = | deaths = }}
'''Thromboangiitis obliterans''', also known as '''Buerger disease''' (English {{IPAc-en|ˈ|b|ɜːr|ɡ|ər}}; {{IPA|de|ˈbʏʁɡɐ|lang}}) or '''Winiwarter-Buerger disease''', is a recurring progressive [[inflammation]] and [[thrombosis]] (clotting) of small and medium [[artery|arteries]] and [[vein]]s of the hands and feet. It is strongly associated with use of [[tobacco]] products,<ref>{{cite journal | vauthors = Joyce JW | title = Buerger's disease (thromboangiitis obliterans) | journal = Rheumatic Disease Clinics of North America | volume = 16 | issue = 2 | pages = 463–70 | date = May 1990 | doi = 10.1016/S0889-857X(21)01071-1 | pmid = 2189162 }}</ref> primarily from [[Tobacco smoking|smoking]], but is also associated with [[smokeless tobacco]].<ref>{{cite web | url = http://www.mayoclinic.org/diseases-conditions/buergers-disease/home/ovc-20179160 | title = Overview of Buerger's disease | work = Mayo Clinic | author = Mayo Clinic Staff | access-date = 13 February 2016 }}</ref><ref>{{cite web | url = https://medlineplus.gov/ency/article/000172.htm | title = Thromboangiitis obliterans | work = Medline Plus | publisher = U.S. National Library of Medicine | access-date = 13 February 2016 }}</ref>
==Signs and symptoms== There is a recurrent acute and chronic [[inflammation]] and [[thrombosis]] of [[artery|arteries]] and [[vein]]s of the hands and feet. The main symptom is [[pain]] in the affected areas, at rest and while walking ([[claudication]]).<ref name=ferri /> The impaired circulation increases sensitivity to cold. Peripheral [[pulse]]s are diminished or absent. There are color changes in the extremities. The colour may range from [[Cyanosis|cyanotic blue]] to reddish blue. Skin becomes thin and shiny. Hair growth is reduced. [[Ulcer (dermatology)|Ulcerations]] and [[gangrene]] in the extremities are common [[Complication (medicine)|complication]]s, often resulting in the need for [[amputation]] of the involved extremity.<ref>{{cite book|last=Porth|first=Carol| name-list-style = vanc |title=Essentials of Pathophysiology: Concepts of Altered Health States |url=https://archive.org/details/essentialspathop00port_175|url-access=limited| edition = 2nd |year=2007 |publisher=Lippincott Williams&Wilkins |isbn=978-0-7817-7087-3 |page=[https://archive.org/details/essentialspathop00port_175/page/n269 264] }}</ref>
==Pathophysiology== There are characteristic [[pathology|pathologic]] findings of acute inflammation and thrombosis (clotting) of arteries and veins of the hands and feet (the lower limbs being more common). The mechanisms underlying Buerger's disease are still largely unknown, but smoking and tobacco consumption are major factors associated with it. It has been suggested that the tobacco may trigger an immune response in susceptible persons or it may unmask a clotting defect, either of which could incite an inflammatory reaction of the vessel wall.<ref>{{cite journal | vauthors = Tanaka K | title = Pathology and pathogenesis of Buerger's disease | journal = International Journal of Cardiology | volume = 66 | pages = S237-42 | date = October 1998 | issue = Suppl 1 | pmid = 9951825 | doi = 10.1016/s0167-5273(98)00174-0 }}</ref> A possible role for ''[[Rickettsia]]'' in this disease has been proposed.<ref name=Fazeli2011>{{cite journal | vauthors = Fazeli B, Ravari H, Farzadnia M | title = Does a species of Rickettsia play a role in the pathophysiology of Buerger's disease? | journal = Vascular | volume = 20 | issue = 6 | pages = 334–6 | date = December 2012 | pmid = 21803838 | doi = 10.1258/vasc.2011.cr0271 | s2cid = 22660338 }}</ref>
==Diagnosis==
A concrete diagnosis of thromboangiitis obliterans is often difficult as it relies heavily on exclusion of other conditions. The commonly followed diagnostic criteria are outlined below although the criteria tend to differ slightly from author to author. Olin (2000) proposes the following criteria:<ref>{{cite journal | vauthors = Olin JW | title = Thromboangiitis obliterans (Buerger's disease) | journal = The New England Journal of Medicine | volume = 343 | issue = 12 | pages = 864–9 | date = September 2000 | pmid = 10995867 | doi = 10.1056/NEJM200009213431207 }}</ref> # Typically between 20 and 40 years old and male, although recently females have been diagnosed.<ref>{{cite book |title=Atlas of Clinical Diagnosis | edition = 2nd |year=2003 |publisher=Elsevier Health Sciences |isbn=978-0-7020-2668-3|page=238}}</ref> # Current (or recent) history of tobacco use. # Presence of distal extremity [[ischemia]] (indicated by [[claudication]], pain at rest, ischemic ulcers or gangrene) documented by noninvasive vascular testing such as ultrasound. # Exclusion of other autoimmune diseases, hypercoagulable states, and diabetes mellitus by laboratory tests. # Exclusion of a proximal source of emboli by echocardiography and arteriography. # Consistent arteriographic findings in the clinically involved and noninvolved limbs.
Buerger's disease can be mimicked by a wide variety of other diseases that cause diminished blood flow to the extremities. These other disorders must be ruled out with an aggressive evaluation, because their treatments differ substantially from that of Buerger's disease, for which there is no treatment known to be effective.{{citation needed|date=February 2021}}
Some diseases with which Buerger's disease may be confused include [[atherosclerosis]] (build-up of cholesterol plaques in the arteries), [[endocarditis]] (an infection of the lining of the heart), other types of [[vasculitis]], severe [[Raynaud's phenomenon]] associated with connective tissue disorders (e.g., [[lupus]] or [[scleroderma]]), [[thrombophilia|clotting disorders]] or the production of [[thrombosis|clots]] in the blood.{{citation needed|date=February 2021}}
Angiograms of the upper and lower extremities can be helpful in making the diagnosis of Buerger's disease. In the proper clinical setting, certain angiographic findings are diagnostic of Buerger's. These findings include a "corkscrew" appearance of arteries that result from vascular damage, particularly the arteries in the region of the wrists and ankles. Collateral circulation gives "tree root" or "spider leg" appearance.<ref name=ferri /> Angiograms may also show occlusions (blockages) or stenosis (narrowings) in multiple areas of both the arms and legs. Distal [[plethysmograph]]y also yields useful information about circulatory status in [[Digit (anatomy)|digits]]. To rule out other forms of vasculitis (by excluding involvement of vascular regions atypical for Buerger's), it is sometimes necessary to perform angiograms of other body regions (e.g., a mesenteric angiogram).{{citation needed|date=February 2021}}
Skin biopsies of affected extremities are rarely performed because of the frequent concern that a biopsy site near an area poorly perfused with blood will not heal well.{{citation needed|date=June 2022}}
==Prevention== {{Further|Thrombosis prophylaxis}} The cause of the disease is thought to be autoimmune in nature and heavily linked to tobacco use in patients with Buerger's as primary disease.{{clarify|date=February 2016}}
==Treatment== Smoking cessation has been shown to slow the progression of the disease and decrease the severity of amputation in most patients, but does not halt the progression.{{citation needed|date=June 2022}}
[[File:Barokomora Nis hyperbaric center.JPG|thumb|Treatment by 100% hyperbaric oxygen]]In [[Acute (medicine)|acute]] cases, drugs and procedures which cause vasodilation are effective in reducing pain experienced by patient. For example, [[prostaglandins]] like Limaprost<ref>{{cite journal | vauthors = Matsudaira K, Seichi A, Kunogi J, Yamazaki T, Kobayashi A, Anamizu Y, Kishimoto J, Hoshi K, Takeshita K, Nakamura K | display-authors = 6 | title = The efficacy of prostaglandin E1 derivative in patients with lumbar spinal stenosis | journal = Spine | volume = 34 | issue = 2 | pages = 115–20 | date = January 2009 | pmid = 19112336 | doi = 10.1097/BRS.0b013e31818f924d | s2cid = 22190177 }}</ref> are vasodilators and give relief of pain, but do not help in changing the course of disease. [[Epidural anesthesia]] and [[hyperbaric oxygen therapy]] also have vasodilator effect.<ref name=ferri /> There is moderate certainty evidence that intravenous [[iloprost]] ([[prostacyclin]] analogue) is more effective than aspirin for relieving rest pain and healing [[Arterial insufficiency ulcer|ischemic ulcers]].<ref name=":0">{{Cite journal|last1=Cacione|first1=Daniel G.|last2=Macedo|first2=Cristiane R.|last3=do Carmo Novaes|first3=Frederico|last4=Baptista-Silva|first4=Jose Cc|date=4 May 2020|title=Pharmacological treatment for Buerger's disease|journal=The Cochrane Database of Systematic Reviews|volume=5|issue=5 |article-number=CD011033|doi=10.1002/14651858.CD011033.pub4|issn=1469-493X|pmc=7197514|pmid=32364620}}</ref> No difference have been detected between iloprost or clinprost (prostacyclin) and [[alprostadil]] (prostaglandin analogue) for relieving pain and healing ulcers.<ref name=":0" />
In [[Chronic (medicine)|chronic]] cases, lumbar [[sympathectomy]] may be occasionally helpful.<ref>{{cite book|title=Clinical Surgery | edition = 2nd |year=2012 |publisher=John Wiley & Sons |isbn=978-1-118-34395-1}}</ref> It reduces vasoconstriction and increases blood flow to limb. It aids in healing and giving relief from pain of ischemic ulcers.<ref name=ferri /> [[Vascular surgery|Bypass]] can sometimes be helpful in treating limbs with poor perfusion secondary to this disease. Use of vascular growth factor and stem cell injections have been showing promise in clinical studies. There may be a benefit of using bone marrow-derived stem cells in healing ulcers and improving pain-free walking distance, but larger, high-quality trials are needed.<ref>{{cite journal | vauthors = Cacione DG, do Carmo Novaes F, Moreno DH | title = Stem cell therapy for treatment of thromboangiitis obliterans (Buerger's disease) | journal = The Cochrane Database of Systematic Reviews | volume = 2018 | article-number = CD012794 | date = October 2018 | issue = 10 | pmid = 30378681 | pmc = 6516882 | doi = 10.1002/14651858.CD012794.pub2 | editor-last = Cochrane Vascular Group }}</ref> [[Debridement]] is done in necrotic ulcers. In gangrenous [[Digit (anatomy)|digit]]s, amputation is frequently required. Below-knee and [[above-knee amputation]] is rarely required.<ref name=ferri />
[[Streptokinase]] has been proposed as adjuvant therapy in some cases.<ref name=pmid8473086>{{cite journal | vauthors = Hussein EA, el Dorri A | title = Intra-arterial streptokinase as adjuvant therapy for complicated Buerger's disease: early trials | journal = International Surgery | volume = 78 | issue = 1 | pages = 54–8 | year = 1993 | pmid = 8473086 }}</ref>
Despite the clear presence of inflammation in this disorder, [[anti-inflammatory]] agents such as [[corticosteroids]] have not been shown to be beneficial in healing, but do have significant anti-inflammatory and pain relief qualities in low dosage intermittent form. Similarly, strategies of [[anticoagulation]] have not proven effective. physical therapy: interferential current therapy to decrease inflammation.{{citation needed|date=February 2021}}
==Prognosis== Buerger's is not immediately fatal. Amputation is common and major amputations (of limbs rather than fingers/toes) are almost twice as common in patients who continue to smoke. Prognosis markedly improves if a person quits smoking. Female patients tend to show much higher longevity rates than men. The only known way to slow the progression of the disease is to abstain from all tobacco products.{{citation needed|date=February 2021}}
==Epidemiology== Buerger's is more common among men than women. Although present worldwide, it is more prevalent in the Middle East and Far East.<ref name="Piazza 1858–1861">{{cite journal | vauthors = Piazza G, Creager MA | title = Thromboangiitis obliterans | journal = Circulation | volume = 121 | issue = 16 | pages = 1858–61 | date = April 2010 | pmid = 20421527 | pmc = 2880529 | doi = 10.1161/CIRCULATIONAHA.110.942383 }}</ref> Incidence of thromboangiitis obliterans is 8 to 12 per 100,000 [[adults]] in the [[United States]] (0.75% of all patients with [[peripheral vascular disease]]).<ref name="Piazza 1858–1861"/>
==History== Buerger's disease was first described by [[Felix von Winiwarter]] in 1879 in [[Austria]].<ref>{{cite journal |author=v. Winiwarter F |title=Ueber eine eigenthümliche Form von Endarteriitis und Endophlebitis mit Gangrän des Fusses |journal=Archiv für Klinische Chirurgie |volume=23 |pages=202–226 |year=1879 |url=https://babel.hathitrust.org/cgi/pt?id=mdp.39015070536068&view=1up&seq=216 }}</ref> It was not until 1908, however, that the disease was given its first accurate pathological description, by [[Leo Buerger]] at [[Mount Sinai Hospital, New York|Mount Sinai Hospital]] in [[New York City]], who referred to the condition as "presenile spontaneous gangrene".<ref>{{cite journal |author= Buerger L |title=Thrombo-angiitis obliterans: a study of the vascular lesions leading to presenile spontaneous gangrene |journal=American Journal of the Medical Sciences |volume=136 |pages=567–580 |date=1908 |doi=10.1097/00000441-190810000-00011 |s2cid=31731903 |url=https://archive.org/details/sim_american-journal-of-the-medical-sciences_1908-10_136_4/page/567/ }}</ref>
==Notable people affected== As reported by Alan Michie in ''God Save the Queen'', published in 1952 (see pages 194 and following), King [[George VI]] was diagnosed with the disease on 12 November 1948. Both legs were affected, the right more seriously than the left. The king's doctors prescribed complete rest and electric treatment to stimulate circulation, but as they were either unaware of the connection between the disease and smoking (the king was a heavy smoker) or unable to persuade the king to stop smoking, the disease failed to respond to their treatment. On 12 March 1949, the king underwent a lumbar [[sympathectomy]], performed at [[Buckingham Palace]] by [[James R. Learmonth]]. The operation, as such, was successful, but the king was warned that it was a palliative, not a cure, and that there could be no assurance that the disease would not grow worse. From all accounts, the king continued to smoke.{{citation needed|date=June 2022}}
The author and journalist [[John McBeth]] describes his experiences of the disease, and treatment for it, in the chapter "Year of the Leg" in his book ''Reporter: Forty Years Covering Asia''.<ref>{{Cite book |vauthors=McBeth J |year=2011 |chapter=Year of the Leg |title=Reporter: Forty Years Covering Asia |location=Singapore |publisher=Talisman Publishing |pages=254–264 |isbn=978-981-08-7364-6}}</ref>
Former [[Philippines|Philippine]] [[President of the Philippines|president]] [[Rodrigo Duterte]] disclosed in 2015 that he has Buerger's disease.<ref>{{Cite news |last1=Frialde |first1=Mike | name-list-style = vanc |title=Duterte: I may not last 6 years in office |url= http://www.philstar.com/headlines/2015/12/10/1531028/duterte-i-may-not-last-6-years-office |access-date=December 17, 2015 |work=The Philippine Star |date=December 10, 2015}}</ref>
[[M. F. K. Fisher]]'s second husband, [[Dillwyn Parrish]], developed Buerger's disease when they lived in Switzerland in the early 1940s.<ref>{{Cite book |last=Starr |first=Kevin |author-link=Kevin Starr |url=https://books.google.com/books?id=ww6e8ucHmksC&dq=%22dillwyn+parrish%22+%22buerger%27s+disease%22&pg=PA381 |title=Material Dreams: Southern California Through the 1920s |date=1990 |publisher=Oxford University Press |isbn=978-0-19-504487-4 |pages=381 |language=en}}</ref>
== References == {{Reflist}}
== Further reading == {{Refbegin}} * {{Cite journal |author=Richards RL |title=Thrombo-angiitis. Clinical diagnosis and classification of cases |journal=British Medical Journal |volume=1 |issue=4808 |pages=478–481 |date=February 1953 |pmid=13009253 |pmc=2015385 |doi=10.1136/bmj.1.4808.478 }} * {{Cite journal |author=Anon |title=Thromboangiitis obliterans|journal=Indian Medical Gazette |volume=88 |issue=7 |pages=395–396 |date=July 1953 |pmid=29015658 |pmc=5202473 }} * {{Cite journal |author=Arkkila PET |title=Thromboangiitis obliterans (Buerger's disease) |journal=Orphanet Journal of Rare Diseases |volume=1 |pages=14pp |date=April 2006 |article-number=14 |pmid=16722538 |pmc=1523324 |doi=10.1186/1750-1172-1-14 |doi-access=free }} * {{Cite journal |vauthors=Aktoz T, Kaplan M, Yalcin O, Atakan IH, Inci O |title=Penile and scrotal involvement in Buerger's disease |journal=Andrologia |volume=40 |issue=6 |pages=401–403 |date=December 2008 |pmid=19032693 |doi=10.1111/j.1439-0272.2008.00859.x |s2cid=33681507 |doi-access= }} {{Refend}}
{{Medical resources | DiseasesDB = 1762 | ICD10 = {{ICD10|I|73|1|i|70}} | ICD9 = {{ICD9|443.1}} | ICDO = | OMIM = 211480 | MedlinePlus = 000172 | eMedicineSubj = med | eMedicineTopic = 253 }}
{{Vascular diseases}} {{Authority control}}
{{DEFAULTSORT:Buerger's Disease}} [[Category:Diseases of arteries, arterioles and capillaries]] [[Category:Vascular-related cutaneous conditions]] [[Category:Ailments of unknown cause]]