Polyarteritis nodosa
| Polyarteritis nodosa | |
|---|---|
| Другие имена | Nodose Bakeris , [ 1 ] Периартерит узлоса , [ 1 ] Болезнь Куссмаула , или болезнь Куссмаул-Мейера , [ 2 ] |
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| Полиартерит узлоза: макроскопический образец сердца с обильной жировой тканью и узловыми утолщенными коронарными сосудами | |
| Специальность | Иммунология , ревматология  |
Полиартерит nodosa ( PAN ) представляет собой системное некротическое воспаление кровеносных сосудов (васкулит), среднего размера влияющие на мышечные артерии , обычно связанные с артериями почк и других внутренних органов, но, как правило, отбрасывают кровообращение легких. [ 3 ] Маленькие аневризм натянуты, как бусы с розариями , [ 4 ] Поэтому сделав этот «розарий» важной диагностической особенностью васкулита. [ 5 ] Пан иногда ассоциируется с инфекцией вирусом В или гепатита С. гепатита [ 6 ] Состояние может присутствовать у младенцев. [ 7 ]
Пан - редкая болезнь. [ 6 ] При лечении пятилетняя выживаемость составляет 80%; Без лечения пятилетняя выживаемость составляет 13%. Смерть часто является следствием почечной недостаточности , инфаркта миокарда или инсульта . [ 8 ]
Признаки и симптомы
[ редактировать ]PAN может влиять на почти на каждую систему органов и, следовательно, может представлять собой широкий спектр признаков и симптомов. [ 6 ] Эти проявления возникают в результате ишемического повреждения пораженных органов, часто кожи, сердца, почек и нервной системы. Конституционные симптомы наблюдаются у 90% пострадавших людей и включают лихорадку , усталость , слабость , потерю аппетита и непреднамеренная потеря веса. [ 6 ]
Кожа: кожа может показать сыпь, отеки, некротические язвы и подкожные узелки (комки). [ 6 ] Проявления кожи PAN включают ощутимую пурпуру и Lives Reticularis у некоторых людей. [ 6 ]
Неврологическая система: поражение нерва может вызвать сенсорные изменения с онемением, болью, сжиганием и слабостью (периферическая невропатия). периферические нервы Часто поражаются , и это чаще всего представляет собой мультиплекс мононеурита , который является наиболее распространенным неврологическим признаком PAN. [ 6 ] Мультиплекс мононеурита развивается у более чем 70% пациентов с узловой полиартеритом из -за повреждения артерий, поставляющих большие периферические нервы. Большинство случаев отмечены асимметричной полинеропатией, но прогрессирующее заболевание может привести к вовлечению симметричного нерва. Участие центральной нервной системы может вызвать инсульты или судороги . [ Цитация необходима ]
Почечная система: вовлечение почек является обычным явлением и часто приводит к смерти частей почки. [ 6 ] Вовлечение почечной артерии , которая обеспечивает почки с высокой кислородом крови, часто приводит к высоким кровяным давлением примерно в трети случаях. [ 6 ] Также можно увидеть осаждение белка или крови в моче. [ 6 ] Почти все пациенты с PAN имеют почечную недостаточность, вызванную сужением почечной артерии, тромбозом и инфарктами. [ Цитация необходима ]
Cardiovascular system: Involvement of the arteries of the heart may cause a heart attack, heart failure, and inflammation of the sac around the heart (pericarditis).[citation needed]
Gastrointestinal system: Damage to mesenteric arteries can cause abdominal pain, mesenteric ischemia, and bowel perforation. Abdominal pain may also be seen.[citation needed]
Musculoskeletal system: Muscle and joint aches are common.[6]
Complications
[edit]- Stroke[6]
- Heart failure resulting from cardiomyopathy and pericarditis[6]
- Intestinal necrosis and perforation[9]
Causes
[edit]PAN has no association with anti-neutrophil cytoplasmic antibodies,[6] but about 30% of people with PAN have chronic hepatitis B and deposits containing HBsAg-HBsAb complexes in affected blood vessels, indicating an immune complex-mediated cause in that subset. Infection with the hepatitis C virus and HIV are occasionally discovered in people affected by PAN.[6] PAN has also been associated with underlying hairy cell leukemia. The cause remains unknown in the remaining cases; there may be causal and clinical distinctions between classic idiopathic PAN, the cutaneous forms of PAN, and PAN associated with chronic hepatitis.[3] In children, cutaneous PAN is frequently associated with streptococcal infections, and positive streptococcal serology is included in the diagnostic criteria.[10]
Diagnosis
[edit]
No specific lab tests exist for diagnosing polyarteritis nodosa. Diagnosis is generally based on the physical examination and a few laboratory studies that help confirm the diagnosis:[citation needed]
- CBC (may demonstrate an elevated white blood count)
- ESR (elevated)
- Perinuclear pattern of antineutrophil cytoplasmic antibodies (p-ANCA) - not associated with "classic" polyarteritis nodosa, but is present in a form of the disease affecting smaller blood vessels, known as microscopic polyangiitis or leukocytoclastic angiitis
- Tissue biopsy (reveals inflammation in small arteries, called arteritis)
- Elevated C-reactive protein
A patient is said to have polyarteritis nodosa if he or she has three of the 10 signs known as the 1990 American College of Rheumatology (ACR)[11] criteria, when a radiographic or pathological diagnosis of vasculitis is made:
- Weight loss greater than/equal to 4.5 kg
- Livedo reticularis (a mottled purplish skin discoloration over the extremities or torso)
- Testicular pain or tenderness (occasionally, a site biopsied for diagnosis)
- Muscle pain, weakness, or leg tenderness
- Nerve disease (either single or multiple)
- Diastolic blood pressure greater than 90 mmHg (high blood pressure)
- Elevated kidney blood tests (BUN greater than 40 mg/dL or creatinine greater than 1.5 mg/dL)
- Hepatitis B (not C) virus tests positive (for surface antigen or antibody)
- Arteriogram (angiogram) showing the arteries that are dilated (aneurysms) or constricted by the blood vessel inflammation
- Biopsy of tissue showing the arteritis (typically inflamed arteries):[12] The sural nerve is a frequent location for the biopsy.
In polyarteritis nodosa, small aneurysms are strung like the beads of a rosary,[4] therefore making this "rosary sign" an important diagnostic feature of the vasculitis.[5] The 1990 ACR criteria were designed for classification purposes only, but their good discriminatory performances, indicated by the initial ACR analysis, suggested their potential usefulness for diagnostic purposes as well. Subsequent studies did not confirm their diagnostic utility, demonstrating a significant dependence of their discriminative abilities on the prevalence of the various vasculitides in the analyzed populations. Recently, an original study, combining the analysis of more than 100 items used to describe patients' characteristics in a large sample of vasculitides with a computer simulation technique designed to test the potential diagnostic utility of the various criteria, proposed a set of eight positively or negatively discriminating items to be used as a screening tool for diagnosis in patients suspected of systemic vasculitis.[13]
Differential diagnosis
[edit]Polyarteritis nodosa rarely affects the blood vessels of the lungs and this feature can help to differentiate it from other vasculitides that may have similar signs and symptoms (e.g., granulomatosis with polyangiitis or microscopic polyangiitis).[6]
Treatment
[edit]Treatment involves medications to suppress the immune system, including prednisone and cyclophosphamide. When present, underlying hepatitis B virus infection should be immediately treated. In some cases, methotrexate or leflunomide may be helpful.[14] Some patients have entered a remission phase when a four-dose infusion of rituximab is used before the leflunomide treatment is begun. Therapy results in remissions or cures in 90% of cases. Untreated, the disease is fatal in most cases. The most serious associated conditions generally involve the kidneys and gastrointestinal tract. A fatal course usually involves gastrointestinal bleeding, infection, myocardial infarction, and/or kidney failure.[15]
In case of remission, about 60% experience relapse within five years.[16] In cases caused by hepatitis B virus, however, recurrence rate is only around 6%.[17]
Epidemiology
[edit]The condition affects adults more frequently than children and males more frequently than females.[6] Most cases occur between the ages of 40 and 60.[6] Polyarteritis nodosa is more common in people with hepatitis B infection.[6]
History
[edit]The medical eponyms Kussmaul disease or Kussmaul-Maier disease reflect the seminal description of the disease in the medical literature by Adolph Kussmaul and Rudolf Robert Maier.[citation needed]
Culture
[edit]In the 1956 American film Bigger Than Life, the protagonist character played by James Mason is diagnosed with polyarteritis nodosa after experiencing excruciating chest pain and is treated with cortisone.[18]
References
[edit]- ^ Jump up to: a b Rapini, Ronald P.; Bolognia, Jean L.; Jorizzo, Joseph L. (2007). Dermatology: 2-Volume Set. St. Louis: Mosby. ISBN 978-1-4160-2999-1.
- ^ synd/764 at Who Named It?
- ^ Jump up to: a b Kumar, Vinay; K. Abbas, Abul; C. Aster, Jon (2015). Robbins and Cotran: Pathologic Basis of Disease (9th ed.). Elsevier. p. 509. ISBN 978-1-4557-2613-4.
- ^ Jump up to: a b Keen, William. Surgery, Volume 5. W. B. Saunders. p. 243.
- ^ Jump up to: a b Greenson, Joel K.; Montgomery, Elizabeth A.; Polydorides, Alexandros D. (1 September 2009). Diagnostic Pathology: Gastrointestinal: Published by Amirsys. Lippincott Williams & Wilkins. ISBN 978-1-931884-26-6. Retrieved 19 August 2013.
- ^ Jump up to: a b c d e f g h i j k l m n o p q r s Forbess, L; Bannykh, S (2015). "Polyarteritis Nodosa". Rheumatic Disease Clinics of North America. 41 (1): 33–46, vii. doi:10.1016/j.rdc.2014.09.005. PMID 25399938.
- ^ Person, A, Donald (2006-06-15). "Infantile Polyarteritis Nodosa". eMedicine. WebMD. Retrieved 24 December 2009.
- ^ Russell Goodman; Paul F. Dellaripa; Amy Leigh Miller; Joseph Loscalzo (January 2, 2014). "An Unusual Case of Abdominal Pain". N Engl J Med. 370 (1): 70–75. doi:10.1056/NEJMcps1215559. PMID 24382068.
- ^ Ebert, Ellen C.; Hagspiel, Klaus D.; Nagar, Michael; Schlesinger, Naomi (2008). "Gastrointestinal Involvement in Polyarteritis Nodosa". Clinical Gastroenterology and Hepatology. 6 (9): 960–966. doi:10.1016/j.cgh.2008.04.004. ISSN 1542-3565. PMID 18585977.
- ^ Sarah Ringold; Carol A Wallace (May 1, 2010). "Evolution of paediatric-specific vasculitis classification criteria". Annals of the Rheumatic Diseases. 69 (5): 785–86. doi:10.1136/ard.2009.127886. PMID 20388739.
- ^ "Log in | BMJ Best Practice". bestpractice.bmj.com.
- ^ Shiel, Jr., William C, http://www.medicinenet.com/polyarteritis_nodosa/article.htm
- ^ Henegar, Corneliu; Pagnoux, Christian; Puéchal, Xavier; Zucker, Jean-Daniel; Bar-Hen, Avner; Guern, Véronique Le; Saba, Mona; Bagnères, Denis; Meyer, Olivier; Guillevin, Loïc (1 May 2008). "A paradigm of diagnostic criteria for polyarteritis nodosa: Analysis of a series of 949 patients with vasculitides". Arthritis & Rheumatism. 58 (5): 1528–1538. doi:10.1002/art.23470. PMID 18438816.
- ^ Boehm, Ingrid; Bauer, R (1 February 2000). "Low-Dose Methotrexate Controls a Severe Form of Polyarteritis Nodosa". Archives of Dermatology. 136 (2): 167–9. doi:10.1001/archderm.136.2.167. PMID 10677090.
- ^ Giannini, AJ; Black, HR. Psychiatric, Psychogenic and Somatopsychic Disorders Handbook. Garden City, NY. Medical Examination Publishing, 1978. Pp. 219–220. ISBN 0-87488-596-5
- ^ Selga, D.; Mohammad, A.; Sturfelt, G.; Segelmark, M. (2006). "Polyarteritis nodosa when applying the Chapel Hill nomenclature--a descriptive study on ten patients". Rheumatology. 45 (10): 1276–1281. doi:10.1093/rheumatology/kel091. PMID 16595516.[1], Entry in Polyarteritis Nodosa Follow-up article
- ^ Guillevin, L.; Lhote, F.; Cohen, P.; Sauvaget, F.; Jarrousse, B.; Lortholary, O.; Noël, L.; Trépo, C. (1995). "Polyarteritis nodosa related to hepatitis B virus. A prospective study with long-term observation of 41 patients". Medicine. 74 (5): 238–253. doi:10.1097/00005792-199509000-00002. PMID 7565065. S2CID 38111848. [2], Entry in Polyarteritis Nodosa Follow-up article
- ^ "Bigger Than Life". The Criterion Collection. Retrieved 2021-07-16.
External links
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