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| '''For patient information, click [[Polyarteritis nodosa (patient information)|here]]''' | | '''For patient information, click [[Polyarteritis nodosa (patient information)|here]]''' |
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| | '''For the heart in Polyarteritis Nodosa click[[The Heart in Polyarteritis Nodosa| here]]''' |
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| {{DiseaseDisorder infobox | | | {{DiseaseDisorder infobox | |
| Name = Polyarteritis nodosa | | | Name = Polyarteritis nodosa | |
| ICD10 = {{ICD10|M|30|0|m|30}} |
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| ICD9 = {{ICD9|446.0}} |
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| ICDO = |
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| Image = PAN Grade 6 atherosclerosis.jpg| | | Image = PAN Grade 6 atherosclerosis.jpg| |
| Caption = Lung: Arteriosclerosis Grade 6: Micro med mag H&E; an excellent example of pulmonary polyarteritis nodosa. 4 yo male with primary pulmonary hypertension <br> <small> [http://www.peir.net Image courtesy of Professor Peter Anderson DVM PhD and published with permission © PEIR, University of Alabama at Birmingham, Department of Pathology] </small>| | | Caption = Lung: Arteriosclerosis Grade 6: Micro med mag H&E; an excellent example of pulmonary polyarteritis nodosa. 4 yo male with primary pulmonary hypertension <br> <small> [http://www.peir.net Image courtesy of Professor Peter Anderson DVM PhD and published with permission © PEIR, University of Alabama at Birmingham, Department of Pathology] </small>| |
| OMIM = |
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| OMIM_mult = |
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| MedlinePlus = 001438 |
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| DiseasesDB = 10220 |
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| MeshID = D010488 |
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| }} | | }} |
| {{Search infobox}} | | {{Polyarteritis nodosa}} |
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| {{CMG}}; '''Associate Editor-In-Chief:''' {{CZ}}; [[User:Haritha|Haritha Machavarapu, M.B.B.S.]] | | {{CMG}} {{APM}}; {{AE}}{{SSW}}{{OO}} {{CZ}}; [[User:Haritha|Haritha Machavarapu, M.B.B.S.]] |
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| {{SK}} PAN; [[Adolph Kussmaul|Kussmaul]] disease; Kussmaul-Meier disease; periarteritis nodosa | | {{SK}} PAN; [[Adolph Kussmaul|Kussmaul]] disease; Kussmaul-Meier disease; periarteritis nodosa |
| ===[[The Heart in Polyarteritis Nodosa|For the heart in Polyarteritis Nodosa click here]]===
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| ==Overview== | | ==[[Polyarteritis nodosa overview|Overview]]== |
| Polyarteritis nodosa is a [[vasculitis]] of medium-sized [[arteries]], which become swollen and damaged from attack by rogue immune cells. | |
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| ==Historical Perspective== | | ==[[Polyarteritis nodosa historical perspective|Historical Perspective]]== |
| Polyarteritis nodosa is also called '''[[Adolph Kussmaul|Kussmaul]] disease''' or '''Kussmaul-Meier disease'''<ref>{{WhoNamedIt|synd|764}}</ref> after Kussumaul and Maier, who first described the syndrome in 1866. | |
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| ==Pathophysiology== | | ==[[Polyarteritis nodosa classification|Classification]]== |
| Polyarteritis nodosa is a disease of unknown cause that affects arteries, the blood vessels that carry oxygenated blood to organs and tissues. It occurs when certain immune cells attack the affected arteries.Inflammation starts in the vessel intima and results in [[fibrinoid necrosis]] by destroying the internal and external elastic lamina. Aneurysms and thrombi may develop at the site of lesions. One hypothesis is that this condition is caused by antibodies against HBV, via a [[Hypersensitivity#Type_3_-_immune_complex | type IIII hypersensitivity reaction]]. [[Hepatitis C]] associated polyarteritis nodosa is the most common type among the hepatitis C associated vasculitis and has a severe clinical presentation.<ref name="pmid20981809">{{cite journal |author=Saadoun D, Terrier B, Semoun O, ''et al.'' |title=Hepatitis C virus-associated polyarteritis nodosa |journal=Arthritis Care Res (Hoboken) |volume=63 |issue=3 |pages=427–35 |year=2011 |month=March |pmid=20981809 |doi=10.1002/acr.20381 |url=}}</ref> | |
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| ==Causes== | | ==[[Polyarteritis nodosa pathophysiology|Pathophysiology]]== |
| Polyarteritis nodosa is a disease of unknown cause that affects arteries, the [[blood vessels]] that carry [[oxygenated blood]] to [[organs]] and [[tissues]]. It occurs when certain [[immune cells]] attack the affected arteries.More adults than children get this disease. It damages the tissues supplied by the affected arteries because the tissues aren't receiving the oxygen and nourishment they need. People with active [[hepatitis B]] and [[hepatitis C]] may develop this disease. | |
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| ==Epidemiology and Demographics== | | ==[[Polyarteritis nodosa causes|Causes]]== |
| The condition affects adults more frequently than children. It damages the tissues supplied by the affected arteries because they don't receive enough oxygen and nourishment without a proper blood supply. Polyarteritis nodosa is more common in people with [[hepatitis B]] infection.
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| ==Natural History, Prognosis, and Complications== | | ==[[Differentiating Polyarteritis nodosa from other diseases|Differentiating Polyarteritis nodosa from other Diseases]]== |
| 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. Without treatment, the outlook is poor. Guillevin and coworkers have described five prognostic factors that predict high probability of mortality and are considered indications for another immunosuppressive drug in addition to prednisone.
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| #Proteinuria >1g/day
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| #Azotemia
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| #Cardiomyopathy
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| #Gastrointestinal involvement
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| #Central nervous system disease <p>With none of these factors, 5-year mortality is 12%. With 2 or more 5-year mortality is 46%<ref>Kelley's Textbook of Rheumatology,8th edition</ref>
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| | ==[[Polyarteritis nodosa epidemiology and demographics|Epidemiology and Demographics]]== |
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| Complications include the following:
| | ==[[Polyarteritis nodosa risk factors|Risk Factors]]== |
| * [[Stroke]]
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| * [[Kidney failure]]
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| * [[myocardial infarction|Heart attack]]
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| * Intestinal [[necrosis]] and perforation
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| ==Diagnosis== | | ==[[Polyarteritis nodosa screening|Screening]]== |
| ===Symptoms===
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| * [[Fatigue (physical)|Fatigue]]
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| * [[Weakness]]
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| * [[Fever]]
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| * [[Abdominal pain]]
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| * [[Decreased appetite]]
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| * Unintentional [[weight loss]]
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| * [[Muscle aches]]
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| * [[Joint aches]]
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| * Skin ulcers
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| In this disease, symptoms result from damage to affected organs, often the skin, heart, kidneys, and nervous system.
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| Generalized symptoms include [[fever]], [[fatigue]], [[weakness]], [[loss of appetite]], and [[weight loss]]. Muscle and joint aches are common. Cutaneous polyarteritis nodosa most frequently manifests as nodules in the lower legs. They may not leave any residual changes but sometimes [[livedo reticularis]] is observed. Ulcerations and splinter hemorrhages are the frequent complications.<ref name="pmid17544958">{{cite journal |author=Díaz-Pérez JL, De Lagrán ZM, Díaz-Ramón JL, Winkelmann RK |title=Cutaneous polyarteritis nodosa |journal=Semin Cutan Med Surg |volume=26 |issue=2 |pages=77–86 |year=2007 |month=June |pmid=17544958 |doi=10.1016/j.sder.2007.02.003 |url=}}</ref>
| | ==[[Polyarteritis nodosa natural history, complications and prognosis|Natural History, Complications and Prognosis]]== |
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| Nerve involvement may cause sensory changes with [[numbness]], pain, burning, and [[weakness]].Frequently affected nerves are peroneal, median, ulnar and sural nerves. Central nervous system polyarteritis nodosa presents in three major forms : [[diffuse encepalopathy]],[[focal neurological deficits]] and [[seizures]]. Diffuse encepahalopathy is characterised by loss of intellectual capacity, disorientation and occasionally [[psychosis]] with [[visual hallucinations]].Cerebrovascular accidents in cerebrum, cerebellum or brain stem cause focal defecits.<ref name="pmid1979706">{{cite journal |author=Rosenberg MR, Parshley M, Gibson S, Wernick R |title=Central nervous system polyarteritis nodosa |journal=West. J. Med. |volume=153 |issue=5 |pages=553–6 |year=1990 |month=November |pmid=1979706 |pmc=1002622 |doi= |url=}}</ref>.
| | == Diagnosis == |
| Kidney involvement can produce varying degrees of [[renal failure]].Arteritis in the renal circulation leads to [[hypertension]].Sometimes patients present with severe flank pain because of renal [[infarction]] or rupture of intrarenal [[aneurysm]].
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| | [[Polyarteritis nodosa diagnostic criteria|Diagnostic Criteria]] | [[Polyarteritis nodosa history and symptoms| History and Symptoms]] | [[Polyarteritis nodosa physical examination | Physical Examination]] | [[Polyarteritis nodosa laboratory findings | Laboratory Findings]] | [[Polyarteritis nodosa electrocardiography | Electrocardiography]] | [[Polyarteritis nodosa chest x ray | Chest X Ray]] | [[Polyarteritis nodosa CT|CT]] | [[Polyarteritis nodosa MRI|MRI]] | [[Polyarteritis nodosa other diagnostic studies|Other Diagnostic Studies]] | [[Polyarteritis nodosa other imaging findings|Other Imaging Findings]] |
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| Involvement of the arteries of the heart may cause a [[heart attack]], [[heart failure]], and inflammation of the sac around the heart ([[pericarditis]]).
| | ==Treatment== |
| [[Vasculitis]] of [[testicular vessels]] can cause testicular infarction, manifesting as [[testicular pain]] or tenderness.<ref name="pmid18651100">{{cite journal |author=Meeuwissen J, Maertens J, Verbeken E, Blockmans D |title=Case reports: testicular pain as a manifestation of polyarteritis nodosa |journal=Clin. Rheumatol. |volume=27 |issue=11 |pages=1463–6 |year=2008 |month=November |pmid=18651100 |doi=10.1007/s10067-008-0970-5 |url=}}</ref>. | | [[Polyarteritis nodosa medical therapy|Medical Therapy]] | [[Polyarteritis nodosa surgery|Surgery]] | [[Polyarteritis nodosa primary prevention|Primary Prevention]] | [[Polyarteritis nodosa secondary prevention|Secondary Prevention]] |[[Polyarteritis nodosa cost-effectiveness of therapy|Cost-Effectiveness of Therapy]] | |[[Polyarteritis nodosa future or investigational therapies|Future or Investigational Therapies]] |
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| [[Abdominal pain]],weight loss, food avoidance, gastrointestinal bleeding, [[peritonitis]] are seen in [[mesentric]] [[vasculitis]].<ref name="pmid7653948">{{cite journal |author=Guillevin L, Lhote F, Gallais V, ''et al.'' |title=Gastrointestinal tract involvement in polyarteritis nodosa and Churg-Strauss syndrome |journal=Ann Med Interne (Paris) |volume=146 |issue=4 |pages=260–7 |year=1995 |pmid=7653948 |doi= |url=}}</ref>
| | ==Case Studies== |
| | [[Polyarteritis nodosa case study one|Case #1]] |
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| ===Laboratory Findings===
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| There are no specific lab tests for diagnosing polyarteritis nodosa. Diagnosis is generally based upon the physical examination and a few laboratory studies that help to confirm the diagnosis:
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| * [[Complete blood count|CBC]] (may demonstrate an elevated white blood count)
| | [[Category:Rheumatology]] |
| * [[Erythrocyte sedimentation rate|ESR]] (often elevated)
| | [[Category:Nephrology]] |
| * 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.
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| * Tissue biopsy (reveals inflammation in small arteries, called [[arteritis]])
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| * Elevated [[c reactive protein]]
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| *[[EMG]] and nerve conduction studies can be done in cases of nerve involvement and help in nerve biopsy.<ref name="pmid17989592">{{cite journal |author=Lacomis D, Zivković SA |title=Approach to vasculitic neuropathies |journal=J Clin Neuromuscul Dis |volume=9 |issue=1 |pages=265–76 |year=2007 |month=September |pmid=17989592 |doi=10.1097/CND.0b013e31815202b3 |url=}}</ref>
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| *Angiography and arteriograms help in identifying aneurysms in mesentry.<ref name="pmid11798986">{{cite journal |author=Hughes LB, Bridges SL |title=Polyarteritis nodosa and microscopic polyangiitis: etiologic and diagnostic considerations |journal=Curr Rheumatol Rep |volume=4 |issue=1 |pages=75–82 |year=2002 |month=February |pmid=11798986 |doi= |url=}}</ref>
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| *CT and MRI of abdomen in GI involvement.
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| *MRI in CNS involvement helps in identifying hemorrhage and ischemia.
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| ===Diagnostic Criteria===
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| A patient is said to have polyarteritis nodosa if he or she has 3 of the 10 following signs:
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| #Weight loss ≥4 kg.
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| #Livedo reticularis (a mottled purplish skin discoloration over the extremities or torso).
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| #Testicular pain or tenderness. (occasionally, a site biopsied for diagnosis).
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| #Muscle pain, weakness, or leg tenderness.
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| #Nerve disease (either single or multiple).
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| #Diastolic blood pressure greater than 90mmHg (high blood pressure).
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| #Elevated kidney blood tests (BUN greater than 40 mg/dl or creatinine greater than 1.5 mg/dl).
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| #Hepatitis B virus tests positive (for surface antigen or antibody).
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| #Arteriogram (angiogram) showing the arteries that are dilated (aneurysms) or constricted by the blood vessel inflammation.
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| #Biopsy of tissue showing the arteritis (typically inflamed arteries).<ref>Shiel, Jr., William C, http://www.medicinenet.com/polyarteritis_nodosa/article.htm</ref>
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| ==Diagnostic x-ray images==
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| [http://www.peir.net Images shown below are courtesy of Professor Peter Anderson DVM PhD and published with permission © PEIR, University of Alabama at Birmingham, Department of Pathology]
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| <div align="left">
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| <gallery heights="150" widths="160">
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| Image:Polyarteritis nodosa mesenteric artery arteriogram.jpg|Polyarteritis nodosa mesenteric artery arteriogram
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| Image:Polyarteritis nodosa liver arteriogram.jpg|Polyarteritis nodosa liver arteriogram
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| </gallery>
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| </div>
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| <div align="left">
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| <gallery heights="150" widths="160">
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| Image:Polyarteritis nodosa kidney arteriogram.jpg|Polyarteritis nodosa kidney arteriogram
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| Image:Polyarteritis nodosa kidney arteriogram 2.jpg|Polyarteritis nodosa kidney arteriogram
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| </gallery>
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| </div>
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| ==Treatment==
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| Treatment involves medications to suppress the immune system such as [[prednisone]] and [[cyclophosphamide]]. Addition of immunosuppressants like [[cyclophosphamide]], [[methotrexate]], [[azathioprine]] to corticosteroid therapy has better prognosis.<ref name="pmid42314">{{cite journal |author=Leib ES, Restivo C, Paulus HE |title=Immunosuppressive and corticosteroid therapy of polyarteritis nodosa |journal=Am. J. Med. |volume=67 |issue=6 |pages=941–7 |year=1979 |month=December |pmid=42314 |doi= |url=}}</ref>.
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| Patients with [[hepatitis B]] associated polyarteritis nodosa are treated with [[corticosteroid therapy]], [[antiviral agents]] and [[plasma exchanges]].<ref name="pmid10214615">{{cite journal |author=Mouthon L |title=[Periarteritis nodosa induced by hepatitis B virus] |language=French |journal=Pathol. Biol. |volume=47 |issue=3 |pages=237–44 |year=1999 |month=March |pmid=10214615 |doi= |url=}}</ref>.<ref name="pmid11334492">{{cite journal |author=Trepo C, Guillevin L |title=Polyarteritis nodosa and extrahepatic manifestations of HBV infection: the case against autoimmune intervention in pathogenesis |journal=J. Autoimmun. |volume=16 |issue=3 |pages=269–74 |year=2001 |month=May |pmid=11334492 |doi=10.1006/jaut.2000.0502 |url=}}</ref>. Unlike hepatitis b associated PAN, [[Hepatitis C]] associated PAN is treated with [[Rituximab]] and corticosteoid therapy without antiviral agents, emphasizing the [[B cell]] targeted therapy.<ref name="pmid22089995">{{cite journal |author=Néel A, Masseau A, Hervier B, ''et al.'' |title=Life-threatening hepatitis C virus-associated polyarteritis nodosa successfully treated by rituximab |journal=J Clin Rheumatol |volume=17 |issue=8 |pages=439–41 |year=2011 |month=December |pmid=22089995 |doi=10.1097/RHU.0b013e31823a58d7 |url=}}</ref>
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| Mild cases of cutaneous polyarteritis nodosa are treated with [[nonsteroidal anti-inflammatory drugs]]<ref name="pmid20618492">{{cite journal |author=Morgan AJ, Schwartz RA |title=Cutaneous polyarteritis nodosa: a comprehensive review |journal=Int. J. Dermatol. |volume=49 |issue=7 |pages=750–6 |year=2010 |month=July |pmid=20618492 |doi=10.1111/j.1365-4632.2010.04522.x |url=}}</ref>. Severe cases are treated with corticosteroid and adjunctive therapy. Antibiotics are added to the treatment of patients with antecedent streptococcal infections or [[high ASO titer]].<ref name="pmid17544958">{{cite journal |author=Díaz-Pérez JL, De Lagrán ZM, Díaz-Ramón JL, Winkelmann RK |title=Cutaneous polyarteritis nodosa |journal=Semin Cutan Med Surg |volume=26 |issue=2 |pages=77–86 |year=2007 |month=June |pmid=17544958 |doi=10.1016/j.sder.2007.02.003 |url=}}</ref>.
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| [[Intravenous immunoglobulin]] is suggested for treatment of corticosteroid therapy resistant cases, but the effect is transient.<ref name="pmid11586025">{{cite journal |author=Kroiss M, Hohenleutner U, Gruss C, Glaessl A, Landthaler M, Stolz W |title=Transient and partial effect of high-dose intravenous immunoglobulin in polyarteritis nodosa |journal=Dermatology (Basel) |volume=203 |issue=2 |pages=188–9 |year=2001 |pmid=11586025 |doi= |url=}}</ref><ref name="pmid17428360">{{cite journal |author=Balbir-Gurman A, Nahir AM, Braun-Moscovici Y |title=Intravenous immunoglobulins in polyarteritis nodosa restricted to the limbs: case reports and review of the literature |journal=Clin. Exp. Rheumatol. |volume=25 |issue=1 Suppl 44 |pages=S28–30 |year=2007 |pmid=17428360 |doi= |url=}}</ref>.It is also used in the treatment of [[Parvovirus B19]] associated polyarteritis nodosa.<ref name="pmid11357850">{{cite journal |author=Viguier M, Guillevin L, Laroche L |title=Treatment of parvovirus B19-associated polyarteritis nodosa with intravenous immune globulin |journal=N. Engl. J. Med. |volume=344 |issue=19 |pages=1481–2 |year=2001 |month=May |pmid=11357850 |doi=10.1056/NEJM200105103441919 |url=}}</ref>
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| ==References==
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| {{reflist|2}}
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| [[Category:Diseases involving the fasciae]]
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| [[Category:Rheumatology]]
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| [[Category:Disease]]
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| [[ja:結節性多発動脈炎]]
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