Medscape is available in 5 Language Editions – Choose your Edition here.


Polyarteritis Nodosa Clinical Presentation

  • Author: Dana Jacobs-Kosmin, MD, FACP; Chief Editor: Herbert S Diamond, MD  more...
Updated: Jan 12, 2016


Polyarteritis nodosa (PAN) is an acute multisystem disease with a relatively short prodrome (ie, weeks to months).[5] Delays in diagnosis are not uncommon. The spectrum of disease ranges from single-organ involvement to fulminant polyvisceral failure. Pertinent and common historical features of PAN include the following:

Constitutional and musculoskeletal symptoms

Constitutional and musculoskeletal symptoms of PAN include the following:

  • Fever
  • Malaise
  • Fatigue
  • Anorexia and weight loss
  • Myalgia
  • Arthralgia in large joints or, less commonly, arthritis

Central nervous system symptoms

Transient symptoms of cerebral ischemia, including typical spells of transient monocular blindness, are the most common presenting CNS deficits of PAN. Cerebral arteritis usually presents late in the course of the disease, usually in the second to third year of the vasculitis. Cerebral arteritis may cause arterial thrombosis with cerebral ischemia or intraparenchymal or subarachnoid hemorrhage.

Global CNS dysfunction with encephalopathy and seizures results from metabolic derangements secondary to multiple organ failure. Acute or subacute myelopathy with paraparesis can occur at any cord level. Myelopathy may result, although rarely, from cord compression by an extramedullary hematoma secondary to a ruptured spinal aneurysm. Although CNS lesions usually occur 2-3 years after the onset of PAN, earlier CNS involvement has been reported.

Peripheral nervous system symptoms

Peripheral neuropathy develops in as many as 60% of patients. Vasculitic neuropathy is often asymmetrical and presents as (1) mononeuritis multiplex, (2) distal polyneuropathy, or (3) cutaneous neuropathy. It can take the form of a pure motor, pure sensory, or mixed sensorimotor polyneuropathy.

Cutaneous symptoms

Dermatologic symptoms are very common in PAN, and about 40% of patients manifest with skin lesions including rash, purpura, gangrene, nodules, cutaneous infarcts, livido reticularis, and Raynaud phenomenon. Skin involvement, which can be painful, occurs most frequently on the legs.

Gastrointestinal symptoms

GI involvement usually presents as nonspecific symptoms and signs such as abdominal pain (which may be postprandial) and nausea and vomiting, with or without obvious GI bleeding. Rare and more serious complications of PAN include bowel infarction and perforation, cholecystitis, hepatic infarction, or pancreatic infarction.

Renal symptoms

About 60% of patients with PAN have renal involvement. Flank pain may be present. Ischemic changes in the glomeruli and renal artery vasculitis can cause renal failure, hypertension, or both. A small percentage of patients may require dialysis.

Additional symptoms

Less common symptoms reported in PAN include the following:

  • Genitourinary - Patients may develop pain over the testicular or ovarian area. In rare cases, testicular infarction may occur; testicular pain is usually unilateral
  • Cardiac - Chest pain, dyspnea, palpitations, pericarditis, myocardial infarction, and congestive heart failure; cardiac disease affects 35% of patients with PAN, but most affected patients are asymptomatic
  • Ophthalmologic - Blurred vision
  • Neuropsychiatric - Headache, psychosis, [39] and depression

Physical Examination

Because polyarteritis nodosa (PAN) is a systemic disease, a complete examination is essential for diagnosis.

The emergence of multiple mononeuropathies in persons with PAN is an important clue to an underlying arteritis. Evidence of organ or extremity ischemia, including hypertension and renal insufficiency (renovascular disease), are further clues to the diagnosis. Lung involvement is rare and its presence should prompt a search for an alternative to PAN as the diagnosis.

Constitutional and musculoskeletal symptoms

Fever may be observed; Raynaud phenomenon may occur. A nondeforming, asymmetrical arthritis, usually involving the larger joints of the lower extremities, has been reported in PAN.

Neurologic symptoms

Neurologic symptoms in PAN include the following:

  • Sensory and/or motor neuropathies - When these occur, they are usually asymmetrical
  • Mononeuritis multiplex (multiple mononeuropathy) - This is the successive ischemia or infarction of "named nerves" (eg, ulnar, radial, peroneal, sural). Although nerve involvement is initially asymmetrical, the development of additional nerve lesions can cause the clinical picture to resemble symmetrical polyneuropathy. (A history of asymmetry at the onset and by electrodiagnostic studies can be helpful in this case.) [40]
  • CNS involvement - Although rare (≤10% of cases), encephalopathy, focal deficits, strokes, seizures, and, sometimes, brain hemorrhages can occur. [40, 41]

Cutaneous symptoms

Cutaneous symptoms in PAN include the following[5] (see the images below):

  • Livedo reticularis that does not blanch with active pressure
  • Ulcerations - Especially on the lower extremities, near the malleoli and on the calf
  • Digital ischemia - May be accompanied by splinter hemorrhages and, sometimes, gangrene
  • Nodules - Usually on the lower extremities (like ulcers); nodules are the least common skin manifestation of PAN
    Tender, hyperpigmented, firm subcutaneous nodules Tender, hyperpigmented, firm subcutaneous nodules with a background of livedo reticularis common in cutaneous polyarteritis nodosa (PAN).
    Tender erythematous nodules with central "punched Tender erythematous nodules with central "punched out" ulcerations common in cutaneous polyarteritis nodosa (PAN).
    Livedo reticularis in polyarteritis nodosa (PAN). Livedo reticularis in polyarteritis nodosa (PAN).

Gastrointestinal symptoms

Gastrointestinal symptoms include the following[29] :

  • Tender abdomen with or without rigidity, guarding, or diminished bowel sounds
  • GI bleeding
  • Bowel infarction
  • Cholecystitis

Renal symptoms

Renal symptoms include the following:

  • Hypertension
  • Costophrenic tenderness
  • Retroperitoneal or intraperitoneal hemorrhage
  • Renal failure

Cardiac symptoms

Cardiac symptoms include the following:

  • Hypertension
  • Tachycardia out of proportion to fever
  • Pericardial friction rub
  • Arrhythmias
  • Congestive heart failure

Ophthalmologic symptoms

Ophthalmologic symptoms of PAN include the following:

  • Retinal vasculitis
  • Retinal detachment
  • Cotton-wool spots

Additional symptoms

The patient may experience testicular tenderness. Psychiatric symptoms, specifically psychosis and depression, may occur.

Unusual presentations

PAN has been reported to present in the muscles alone. One patient had features suggesting polymyositis[42] ; an additional patient had disease limited to the soleus and gastrocnemius muscles of his calf.[43]

Contributor Information and Disclosures

Dana Jacobs-Kosmin, MD, FACP Attending Physician, Department of Medicine, Division of Rheumatology, Einstein Medical Center; Clinical Assistant Professor of Medicine, Jefferson Medical College of Thomas Jefferson University

Dana Jacobs-Kosmin, MD, FACP is a member of the following medical societies: American College of Physicians, American College of Rheumatology, American Medical Association

Disclosure: Nothing to disclose.


J Mark Jackson, MD Clinical Professor of Medicine/Dermatology, Division of Dermatology, University of Louisville School of Medicine

J Mark Jackson, MD is a member of the following medical societies: American Academy of Dermatology, American Medical Association, Kentucky Medical Association, Texas Medical Association

Disclosure: Nothing to disclose.

Chief Editor

Herbert S Diamond, MD Visiting Professor of Medicine, Division of Rheumatology, State University of New York Downstate Medical Center; Chairman Emeritus, Department of Internal Medicine, Western Pennsylvania Hospital

Herbert S Diamond, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians, American College of Rheumatology, American Medical Association, Phi Beta Kappa

Disclosure: Nothing to disclose.


Mahendra Agraharkar, MD, MBBS, FACP, FASN, Clinical Associate Professor of Medicine, Baylor College of Medicine; President and CEO, Space City Associates of Nephrology

Mahendra Agraharkar, MD, MBBS, FACP, FASN is a member of the following medical societies: American College of Physicians, American Society of Nephrology, and National Kidney Foundation

Disclosure: South Shore DaVita Dialysis Center Ownership interest

Bruce A Baethge, MD, Faculty, Texas A&M Medical School

Bruce A Baethge, MD is a member of the following medical societies: Alpha Omega Alpha; American College of Physicians; American College of Rheumatology and Arthritis Foundation

Disclosure: Nothing to disclose.

Kanwarpreet Baweja, MD, Fellow in Nephrology, Division of Renal Diseases and Hypertension, University of Texas Health Science Center

Kanwarpreet Baweja, MD is a member of the following medical societies: American Medical Association, American Society of Nephrology, Medical Council of India, and National Kidney Foundation

Disclosure: Nothing to disclose.

Steve Chung, MD, Director, Clinical Epilepsy Research, Assistant Professor of Clinical Neurology, Residency Director, Department of Neurology, Barrow Neurological Institute

Steven Chung, MD is a member of the following medical societies: American Academy of Neurology, American Epilepsy Society, American Medical Association, California Medical Assocation, and Phi Beta Kappa

Disclosure: Nothing to disclose.

Elliot Goldberg, MD Dean of the Western Pennsylvania Clinical Campus, Professor, Department of Medicine, Temple University School of Medicine

Elliot Goldberg, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians, and American College of Rheumatology

Disclosure: Nothing to disclose.

B Mark Keegan, MD, FRCPC, Assistant Professor of Neurology, College of Medicine, Mayo Clinic; Master's Faculty, Mayo Graduate School; Consultant, Department of Neurology, Mayo Clinic, Rochester

B Mark Keegan, MD, FRCPC is a member of the following medical societies: American Academy of Neurology, American Medical Association, and Minnesota Medical Association

Disclosure: Novartis Consulting fee; Bionest Consulting fee

Howard S Kirshner, MD, Professor of Neurology, Psychiatry and Hearing and Speech Sciences, Vice Chairman, Department of Neurology, Vanderbilt University School of Medicine; Director, Vanderbilt Stroke Center; Program Director, Stroke Service, Vanderbilt Stallworth Rehabilitation Hospital; Consulting Staff, Department of Neurology, Nashville Veterans Affairs Medical Center

Howard S Kirshner, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Neurology, American Heart Association, American Medical Association, American Neurological Association, American Society of Neurorehabilitation, National Stroke Association, Phi Beta Kappa, and Tennessee Medical Association

Disclosure: Nothing to disclose.

Carlos J Lozada, MD Director of Rheumatology Fellowship Program, Professor, Department of Medicine, Division of Rheumatology and Immunology, University of Miami, Leonard M Miller School of Medicine

Carlos J Lozada, MD is a member of the following medical societies: American College of Physicians and American College of Rheumatology

Disclosure: Pfizer Honoraria Speaking and teaching; Amgen Honoraria Speaking and teaching

Christopher Luzzio, MD, Clinical Assistant Professor, Department of Neurology, University of Wisconsin at Madison School of Medicine and Public Health

Christopher Luzzio, MD is a member of the following medical societies: American Academy of Neurology

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

Vasanta P Weiss, MD, Consulting Staff, Department of Nephrology, University of Virginia Medical Center

Disclosure: Nothing to disclose.

  1. Kussmaul A, Maier R. Ueber eine bisher nicht beschriebene eigenthümliche Arterienerkrankung (Periarteritis nodosa), die mit Morbus Brightii und rapid fortschreitender allgemeiner Muskellähmung einhergeht. Dtsch Arch Klin Med. 1866. 1:484-518.

  2. Forbess L, Bannykh S. Polyarteritis nodosa. Rheum Dis Clin North Am. 2015. 41 (1):33-46, vii. [Medline].

  3. Matteson EL. A history of early investigation in polyarteritis nodosa. Arthritis Care Res. 1999 Aug. 12(4):294-302. [Medline].

  4. Davson J, Ball J, Platt R. The kidney in periarteritis nodosa. Q J Med. 1948 Jul. 17(67):175-202. [Medline].

  5. Stone JH. Polyarteritis nodosa. JAMA. 2002 Oct 2. 288(13):1632-9. [Medline].

  6. Lightfoot RW Jr, Michel BA, Bloch DA, Hunder GG, Zvaifler NJ, McShane DJ. The American College of Rheumatology 1990 criteria for the classification of polyarteritis nodosa. Arthritis Rheum. 1990 Aug. 33(8):1088-93. [Medline].

  7. Jennette JC, Falk RJ, Andrassy K. Nomenclature of systemic vasculitides. Proposal of an international consensus conference. Arthritis Rheum. 1994 Feb. 37(2):187-92. [Medline].

  8. Colmegna I, Maldonado-Cocco JA. Polyarteritis nodosa revisited. Curr Rheumatol Rep. 2005 Aug. 7(4):288-96. [Medline].

  9. Trepo C, Guillevin L. Polyarteritis nodosa and extrahepatic manifestations of HBV infection: the case against autoimmune intervention in pathogenesis. J Autoimmun. 2001 May. 16(3):269-74. [Medline].

  10. Mahr A, Guillevin L, Poissonnet M, Aymé S. Prevalences of polyarteritis nodosa, microscopic polyangiitis, Wegener's granulomatosis, and Churg-Strauss syndrome in a French urban multiethnic population in 2000: a capture-recapture estimate. Arthritis Rheum. 2004 Feb 15. 51(1):92-9. [Medline].

  11. Guillevin L, Lhote F, Cohen P, Sauvaget F, Jarrousse B, Lortholary O, et al. Polyarteritis nodosa related to hepatitis B virus. A prospective study with long-term observation of 41 patients. Medicine (Baltimore). 1995 Sep. 74(5):238-53. [Medline].

  12. Zhou Q, Yang D, Ombrello AK, Zavialov AV, Toro C, et al. Early-onset stroke and vasculopathy associated with mutations in ADA2. N Engl J Med. 2014 Mar 6. 370 (10):911-20. [Medline].

  13. Navon Elkan P, Pierce SB, Segel R, Walsh T, Barash J, et al. Mutant adenosine deaminase 2 in a polyarteritis nodosa vasculopathy. N Engl J Med. 2014 Mar 6. 370 (10):921-31. [Medline]. [Full Text].

  14. Gonzalez Santiago TM, Zavialov A, Saarela J, Seppanen M, Reed AM, Abraham RS, et al. Dermatologic Features of ADA2 Deficiency in Cutaneous Polyarteritis Nodosa. JAMA Dermatol. 2015 Nov 1. 151 (11):1230-4. [Medline].

  15. Soufir N, Descamps V, Crickx B. Hepatitis C virus infection in cutaneous polyarteritis nodosa: a retrospective study of 16 cases. Arch Dermatol. 1999 Aug. 135(8):1001-2. [Medline].

  16. Saadoun D, Terrier B, Semoun O, Sene D, Maisonobe T, Musset L, et al. Hepatitis C virus-associated polyarteritis nodosa. Arthritis Care Res (Hoboken). 2010 Oct 27. [Medline].

  17. Hughes LB, Bridges SL Jr. Polyarteritis nodosa and microscopic polyangiitis: etiologic and diagnostic considerations. Curr Rheumatol Rep. 2002 Feb. 4(1):75-82. [Medline].

  18. Somer T, Finegold SM. Vasculitides associated with infections, immunization, and antimicrobial drugs. Clin Infect Dis. 1995 Apr. 20(4):1010-36. [Medline].

  19. Patel N, Patel N, Khan T, Patel N, Espinoza LR. HIV infection and clinical spectrum of associated vasculitides. Curr Rheumatol Rep. 2011 Dec. 13(6):506-12. [Medline].

  20. Imanishi H, Tsuruta D, Oshimo T, Sowa J, Mizuno N, Nakagawa K, et al. Cutaneous polyarteritis nodosa induced by Mycobacterium tuberculosis. J Dermatol. 2012 Aug. 39(8):738-9. [Medline].

  21. Watts RA, Mooney J, Lane SE, Scott DG. Rheumatoid vasculitis: becoming extinct?. Rheumatology (Oxford). 2004 Jul. 43(7):920-3. [Medline].

  22. Nakano H, Ooka S, Shibata T, Ogawa H, Ito H, Takakuwa Y, et al. Cutaneous polyarteritis nodosa associated with HLA-B39-positive undifferentiated spondyloarthritis in a Japanese patient. Mod Rheumatol. 2012 Sep. 22(5):783-6. [Medline].

  23. Pagnini I, Simonini G, Lippi F, Azzari C, Cimaz R. Cutaneous polyarteritis nodosa and common variable immunodeficiency: a previously unreported association. Clin Exp Rheumatol. 2012 Jan-Feb. 30(1 Suppl 70):S169. [Medline].

  24. Oulego-Erroz I, Gautreaux-Minaya S, Martinez-Sáenz de Jubera J, Naranjo-Vivas D, Fernéndez-Hernández S, Muñíz-Fontán M. Coexistence of polyarteritis nodosa and psoriatic arthritis in a child: an unreported association: Polyarteritis nodosa and Psoriatic arthitritis. Eur J Pediatr. 2011 Sep. 170(9):1213-5. [Medline].

  25. Hasler P, Kistler H, Gerber H. Vasculitides in hairy cell leukemia. Semin Arthritis Rheum. 1995 Oct. 25(2):134-42. [Medline].

  26. Ambrosio MR, Rocca BJ, Ginori A, Onorati M, Fabbri A, Carmellini M, et al. Renal infarction due to polyarteritis nodosa in a patient with angioimmunoblastic T-cell lymphoma: a case report and a brief review of the literature. Diagn Pathol. 2012 May 8. 7:50. [Medline]. [Full Text].

  27. McMahon BJ, Heyward WL, Templin DW, Clement D, Lanier AP. Hepatitis B-associated polyarteritis nodosa in Alaskan Eskimos: clinical and epidemiologic features and long-term follow-up. Hepatology. 1989 Jan. 9(1):97-101. [Medline].

  28. Selga D, Mohammad A, Sturfelt G, Segelmark M. Polyarteritis nodosa when applying the Chapel Hill nomenclature--a descriptive study on ten patients. Rheumatology (Oxford). 2006 Oct. 45(10):1276-81. [Medline].

  29. Levine SM, Hellmann DB, Stone JH. Gastrointestinal involvement in polyarteritis nodosa (1986-2000): presentation and outcomes in 24 patients. Am J Med. 2002 Apr 1. 112(5):386-91. [Medline].

  30. Falcini F, La Torre F, Vittadello F, Rigante D, Martini G, Corona F, et al. Clinical overview and outcome in a cohort of children with polyarteritis nodosa. Clin Exp Rheumatol. 2014 May-Jun. 32(3 Suppl 82):S134-7. [Medline].

  31. Eleftheriou D, Dillon MJ, Tullus K, Marks SD, Pilkington CA, Roebuck DJ, et al. Systemic polyarteritis nodosa in the young: a single-center experience over thirty-two years. Arthritis Rheum. 2013 Sep. 65(9):2476-85. [Medline].

  32. Agarwal A, Bansal M, Pandey R, Swaminathan S. Bilateral subcapsular and perinephric hemorrhage as the initial presentation of polyarteritis nodosa. Intern Med. 2012. 51(9):1073-6. [Medline].

  33. Battula N, Tsapralis D, Morgan M, Mirza D. Spontaneous liver haemorrhage and haemobilia as initial presentation of undiagnosed polyarteritis nodosa. Ann R Coll Surg Engl. 2012 May. 94(4):e163-5. [Medline].

  34. Parent BA, Cho SW, Buck DG, Nalesnik MA, Gamblin TC. Spontaneous rupture of hepatic artery aneurysm associated with polyarteritis nodosa. Am Surg. 2010 Dec. 76(12):1416-9. [Medline].

  35. Yuce M, Davutoglu V, Sari I, Onat AM. Polyarteritis nodosa with multiple coronary aneurysms presenting as acute myocardial infarction. Am J Med Sci. 2011 May. 341(5):409. [Medline].

  36. Pagnoux C, Le Guern V, Goffinet F, Diot E, Limal N, Pannier E, et al. Pregnancies in systemic necrotizing vasculitides: report on 12 women and their 20 pregnancies. Rheumatology (Oxford). 2011 May. 50(5):953-61. [Medline].

  37. Kocyigit I, Koyuncu S, Mavili E, Unal A, Tokmak TT, Cilan H. Unusual clinic presentation of polyarteritis nodosa: involvement of axillary and brachial artery. Ren Fail. 2011. 33(10):1043-5. [Medline].

  38. Guillevin L, Lhote F, Gayraud M, Cohen P, Jarrousse B, Lortholary O. Prognostic factors in polyarteritis nodosa and Churg-Strauss syndrome. A prospective study in 342 patients. Medicine (Baltimore). 1996 Jan. 75(1):17-28. [Medline].

  39. Fernandes SR, Coimbra IB, Costallat LT. Uncommon features of polyarteritis nodosa: psychosis and angio-oedema. Clin Rheumatol. 1998. 17(4):353-6. [Medline].

  40. Griffin JW. Vasculitic neuropathies. Rheum Dis Clin North Am. 2001 Nov. 27(4):751-60, vi. [Medline].

  41. Rosenberg MR, Parshley M, Gibson S, Wernick R. Central nervous system polyarteritis nodosa. West J Med. 1990 Nov. 153(5):553-6. [Medline]. [Full Text].

  42. Haroon M, Bermingham N, Keohane C, Harney S. Polyarteritis nodosa presenting with clinical and radiologic features suggestive of polymyositis. Rheumatol Int. 2012 Apr. 32(4):1079-81. [Medline].

  43. Yang SN, Cho NS, Choi HS, Choi SJ, Yoon ES, Kim DH. Muscular polyarteritis nodosa. J Clin Rheumatol. 2012 Aug. 18(5):249-52. [Medline].

  44. Filippone EJ, Foy A, Galanis T, Pokuah M, Newman E, Lallas CD. Segmental arterial mediolysis: report of 2 cases and review of the literature. Am J Kidney Dis. 2011 Dec. 58(6):981-7. [Medline].

  45. Kermani TA, Ham EK, Camilleri MJ, Warrington KJ. Polyarteritis nodosa-like vasculitis in association with minocycline use: a single-center case series. Semin Arthritis Rheum. 2012 Oct. 42(2):213-21. [Medline].

  46. Bezier M, Perceau G, Reguiai Z, Remy-Leroux V, Tchen T, Durlach A. [Necrotic leg ulcers induced by vitamin K antagonists: five cases]. Ann Dermatol Venereol. 2011 Oct. 138(10):657-63. [Medline].

  47. Higuchi T, Sugimoto N, Hayama M, Tanaka E. The usefulness of 3D-CT angiography in polyarteritis nodosa. Intern Med. 2012. 51(11):1449-50. [Medline].

  48. Hervier B, Durant C, Masseau A, Ponge T, Hamidou M, Mussini JM. Use of muscle biopsies for diagnosis of systemic vasculitides. J Rheumatol. 2011 Mar. 38(3):470-4. [Medline].

  49. Ricotti C, Kowalczyk JP, Ghersi M, Nousari CH. The diagnostic yield of histopathologic sampling techniques in PAN-associated cutaneous ulcers. Arch Dermatol. 2007 Oct. 143(10):1334-6. [Medline].

  50. Gheita TA, Khairy NA, Nasrallah MM, Hussein H. Subclinical renal involvement in essential cryoglobulinemic vasculitis and classic polyarteritis nodosa. Joint Bone Spine. 2012 May. 79(3):274-80. [Medline].

  51. Demirkaya E, Ozen S, Pistorio A, Galasso R, Ravelli A, Hasija R. Performance of Birmingham Vasculitis Activity Score and disease extent index in childhood vasculitides. Clin Exp Rheumatol. 2012 Jan-Feb. 30(1 Suppl 70):S162-8. [Medline].

  52. Seri Y, Shoda H, Hanata N, Nagafuchi Y, Sumitomo S, Fujio K, et al. A case of refractory polyarteritis nodosa successfully treated with rituximab. Mod Rheumatol. 2015 Mar 12. 1-3. [Medline].

  53. Campanilho-Marques R, Ramos F, Canhão H, Fonseca JE. Remission induced by infliximab in a childhood polyarteritis nodosa refractory to conventional immunosuppression and rituximab. Joint Bone Spine. 2014 May. 81 (3):277-8. [Medline].

  54. de Luna G, et al; French Vasculitis Study Group (FVSG). Plasma exchanges for the treatment of severe systemic necrotizing vasculitides in clinical daily practice: Data from the French Vasculitis Study Group. J Autoimmun. 2015 Dec. 65:49-55. [Medline].

  55. Frohnert PP, Sheps SG. Long-term follow-up study of periarteritis nodosa. Am J Med. 1967 Jul. 43(1):8-14. [Medline].

  56. Gayraud M, Guillevin L, le Toumelin P, Cohen P, Lhote F, Casassus P. Long-term followup of polyarteritis nodosa, microscopic polyangiitis, and Churg-Strauss syndrome: analysis of four prospective trials including 278 patients. Arthritis Rheum. 2001 Mar. 44(3):666-75. [Medline].

  57. Berlit P. Diagnosis and treatment of cerebral vasculitis. Ther Adv Neurol Disord. 2010 Jan. 3(1):29-42. [Medline]. [Full Text].

  58. Fernanda F, Serena C, Giustina R, Antonella B, Alessandra D, Alessio M. Mycophenolate mofetil treatment in two children with severe polyarteritis nodosa refractory to immunosuppressant drugs. Rheumatol Int. 2012 Jul. 32(7):2215-9. [Medline].

  59. Guillevin L, Mahr A, Cohen P, Larroche C, Queyrel V, Loustaud-Ratti V, et al. Short-term corticosteroids then lamivudine and plasma exchanges to treat hepatitis B virus-related polyarteritis nodosa. Arthritis Rheum. 2004 Jun 15. 51(3):482-7. [Medline].

  60. Guillevin L, Lhote F, Leon A, Fauvelle F, Vivitski L, Trepo C. Treatment of polyarteritis nodosa related to hepatitis B virus with short term steroid therapy associated with antiviral agents and plasma exchanges. A prospective trial in 33 patients. J Rheumatol. 1993 Feb. 20(2):289-98. [Medline].

  61. Guillevin L, Lhote F, Sauvaget F, Deblois P, Rossi F, Levallois D. Treatment of polyarteritis nodosa related to hepatitis B virus with interferon-alpha and plasma exchanges. Ann Rheum Dis. 1994 May. 53(5):334-7. [Medline].

  62. Erhardt A, Sagir A, Guillevin L, Neuen-Jacob E, Häussinger D. Successful treatment of hepatitis B virus associated polyarteritis nodosa with a combination of prednisolone, alpha-interferon and lamivudine. J Hepatol. 2000 Oct. 33(4):677-83. [Medline].

  63. Wicki J, Olivieri J, Pizzolato G, Sarasin F, Guillevin L, Dayer JM. Successful treatment of polyarteritis nodosa related to hepatitis B virus with a combination of lamivudine and interferon alpha. Rheumatology (Oxford). 1999 Feb. 38(2):183-5. [Medline].

  64. Molloy PJ, Friedlander L, Van Thiel DH. Combined interferon, famciclovir and GM-CSF treatment of HBV infection in an individual with periarteritis nodosa. Hepatogastroenterology. 1999 Jul-Aug. 46(28):2529-31. [Medline].

  65. de Menthon M, Mahr A. Treating polyarteritis nodosa: current state of the art. Clin Exp Rheumatol. 2011 Jan-Feb. 29(1 Suppl 64):S110-6. [Medline].

  66. Kawakami T, Soma Y. Use of mizoribine in two patients with recalcitrant cutaneous polyarteritis nodosa. J Am Acad Dermatol. 2011 Jun. 64(6):1213-4. [Medline].

  67. Kluger N, Guillot B, Bessis D. Ulcerative cutaneous polyarteritis nodosa treated with mycophenolate mofetil and pentoxifylline. J Dermatolog Treat. 2011 Jun. 22(3):175-7. [Medline].

Nonspecific, firm, tender subcutaneous nodules without livedo reticularis and/or systemic involvement may be the first sign of polyarteritis nodosa (PAN).
Tender, hyperpigmented, firm subcutaneous nodules with a background of livedo reticularis common in cutaneous polyarteritis nodosa (PAN).
Tender erythematous nodules with central "punched out" ulcerations common in cutaneous polyarteritis nodosa (PAN).
Polyarteritis nodosa (PAN) is characterized by fibrinoid necrosis of the arterial wall with a leukocytic infiltrate. In this slide, a large, pale occlusion of a muscular artery can be seen. Within this collagenous tissue is a leukocytic infiltrate, which is the hallmark of PAN. Courtesy of Urbana Atlas of Pathology.
Livedo reticularis in polyarteritis nodosa (PAN).
All material on this website is protected by copyright, Copyright © 1994-2016 by WebMD LLC. This website also contains material copyrighted by 3rd parties.