classic polyarteritis nodosa (PAN)
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Introduction
Classic form described in 1866 by Kussmaul & Maier.
Etiology
- associated diseases:
- hepatitis B infection
- hepatitis B surface antigen (HBSAg) positive (20-50%)
- hepatitis C (5%)
- hairy cell leukemia
- rheumatoid arthritis
- Sjogren's syndrome
- hepatitis B infection
Epidemiology
- mean age of onset: 48 years
- male:female ratio 1.6:1
- uncommon: incidence 4.6-9/million
- all racial groups affected
Pathology
- multisystem necrotizing vasculitis
- necrotizing inflammation of small & medium sized muscular arteries
- lesions are transmural, segmental & occur at bifurcations
- neutrophils & monocytes invading the vessel walls
- fibrinoid necrosis of vessels
- thrombosis of necrosed vessels with infarction of affected tissue
- renal artery vasculitis & mesenteric artery vasculitis characteristic
- kidney disease but no glomerulonephritis
- no granulomas
- no eosinophilia
- pathology may be initiated by immune complexes
Genetics
Clinical manifestations
- general:
- fever
- weight loss
- malaise
- weakness
- night sweats
- hypertension
- symptoms develop over days to months
- central nervous system: (23%)
- peripheral nervous system: (50%)
- peripheral neuropathy (asymmetric)
- mononeuritis multiplex
- gastrointestinal: (44%)
- renal: (60%)
- proteinuria (rarely in nephrotic range)
- hypertension
- acute renal failure is rare
- no glomerulonephritis
- renal artery vasculitis[3]
- flank pain
- cardiac: (36%)
- musculoskeletal: (64%)
- genitourinary: (25%)
- testicular pain, orchitis
- epididymal pain
- ovarian pain
- renal disease resulting from ischemia rather than glomerulonephritis[3]
- skin: (43%)
- eye: scleritis
- respiratory tract rarely involved[3]
- sensorineural hearing loss[3]
- pulmonary disease & eosinophila are not features
Laboratory
- complete blood count (CBC):
- elevated erythrocyte sedimentation rate (ESR) & serum C-reactive protein
- liver function tests
- hepatitis B panel (30% positive for HBSAg)
- hepatitis C serology
- anti neutrophil cytoplasmic antibody (ANCA) may be positive
- p or peripheral type (pANCA) in polyarteritis nodosa
- MKSAP19 makes no mention of this[3]
- c or central type (cANCA) in Wegener's granulomatosis
- p or peripheral type (pANCA) in polyarteritis nodosa
- serum complement levels are normal[3]
- biopsy
- skin biopsy (skin nodule)*
- painful testes
- muscle biopsy
- nerve biopsy (sural nerve)*
- microscopy of medium sized muscular arteries for diagnosis (see Pathology:)
- urinalysis:
- proteinuria (rarely nephrotic range), hematuria, no erythrocyte casts[3]
- see ARUP consult[5]
* gold standards for diagnosis[3]
Radiology
- abdominal CT angiography may demonstrate microaneurysms, stenosis, occlusion, & beading in small & medium sized arteries, especially of the renal arteries & mesenteric arteries
- MKSAP19 suggests MRI angiography diagnostic test of choice
Complications
- see clinical manifestations
Differential diagnosis
Management
- many patients respond to high-dose glucocorticoids
- prednisone 1 mg/kg/day
- combination therapy
- prednisone 1 mg/kg PO QD + cyclophosphamide 2 mg/kg PO QD
- recommended initial therapy[14]
- cyclophosphamide formerly reserved for patients without hepatitis B who do not respond to prednisone or have significant renal, gastrointestinal, cardiac or neurologic manifestations[3]
- prednisone + azathioprine or methotrexate controversial[14]
- 90% long term remission after discontinuation of therapy
- HBSAg+ polyarteritis nodosa
- antiviral therapy
- prednisone for 2 weeks, followed by plasmapheresis, then antiviral therapy
- apparently outdated recommendation[3]
- patients generally respond to therapy, but become chronic carriers of hepatitis B & may later die of cirrhosis or variceal bleeding
- control of hypertension reduces renal, cardiac & CNS complications
- prognosis
- prognosis of untreated polyarteritis nodosa is poor
- fulminant deterioration
- slow progression with intermittent exacerbations
- 5 year survival is 13% if untreated
- predictors of poor prognosis with treatment
- renal, gastrointestinal, CNS or cardiac involvement
- 5 year survival is 65% with any of these predictors & 90% without[3]
- prognosis of untreated polyarteritis nodosa is poor
- hepatitis B vaccine reduces incidence of polyarteritis nodosa[3]
More general terms
References
- ↑ Harrison's Principles of Internal Medicine, 13th ed. Isselbacher et al (eds), McGraw-Hill Inc. NY, 1994, pg 1671-2
- ↑ Harrison's Principles of Internal Medicine, 14th ed. Fauci et al (eds), McGraw-Hill Inc. NY, 1998, pg 1912-14
- ↑ 3.00 3.01 3.02 3.03 3.04 3.05 3.06 3.07 3.08 3.09 3.10 3.11 3.12 3.13 3.14 3.15 3.16 Medical Knowledge Self Assessment Program (MKSAP) 11, 14, 15, 16, 17, 18,19. American College of Physicians, Philadelphia 1998, 2006, 2009, 2012, 2015, 2018, 2022.
Medical Knowledge Self Assessment Program (MKSAP) 19 Board Basics. An Enhancement to MKSAP19. American College of Physicians, Philadelphia 2022 - ↑ Khasnis A and Langford CA. Update on vasculitis. J Allergy Clin Immunol 2009 Jun; 123:1226. PMID: https://www.ncbi.nlm.nih.gov/pubmed/19501230
- ↑ 5.0 5.1 ARUP Consult: Polyarteritis Nodosa - PAN The Physician's Guide to Laboratory Test Selection & Interpretation https://arupconsult.com/content/polyarteritis-nodosa
- ↑ Henegar C, Pagnoux C, Puachal X et al A paradigm of diagnostic criteria for polyarteritis nodosa: analysis of a series of 949 patients with vasculitides. Arthritis Rheum. 2008 May;58(5):1528-38. PMID: https://www.ncbi.nlm.nih.gov/pubmed/18438816
- ↑ 7.0 7.1 Guillevin L, Mahr A, Callard P et al Hepatitis B virus-associated polyarteritis nodosa: clinical characteristics, outcome, and impact of treatment in 115 patients. Medicine (Baltimore). 2005 Sep;84(5):313-22. PMID: https://www.ncbi.nlm.nih.gov/pubmed/16148731
- ↑ Pagnoux C, Seror R, Henegar C et al Clinical features and outcomes in 348 patients with polyarteritis nodosa: a systematic retrospective study of patients diagnosed between 1963 and 2005 and entered into the French Vasculitis Study Group Database. Arthritis Rheum. 2010 Feb;62(2):616 PMID: https://www.ncbi.nlm.nih.gov/pubmed/20112401
- ↑ Ebert EC, Hagspiel KD, Nagar M, Schlesinger N Gastrointestinal involvement in polyarteritis nodosa. Clin Gastroenterol Hepatol. 2008 Sep;6(9):960-6 PMID: https://www.ncbi.nlm.nih.gov/pubmed/18585977
- ↑ 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. PMID: https://www.ncbi.nlm.nih.gov/pubmed/21586205
- ↑ De Virgilio A, Greco A, Magliulo G et al Polyarteritis nodosa: A contemporary overview. Autoimmun Rev. 2016 Jun;15(6):564-70. Review. PMID: https://www.ncbi.nlm.nih.gov/pubmed/26884100
- ↑ Forbess L, Bannykh S. Polyarteritis nodosa. Rheum Dis Clin North Am. 2015;41(1):33-46, vii. Review. PMID: https://www.ncbi.nlm.nih.gov/pubmed/25399938
- ↑ Alibaz-Oner F, Koster MJ, Crowson CS Clinical Spectrum of Medium-Sized Vessel Vasculitis. Arthritis Care Res (Hoboken). 2017 Jun;69(6):884-891. PMID: https://www.ncbi.nlm.nih.gov/pubmed/27564269 Free Article
- ↑ 14.0 14.1 14.2 Chung SA, Gorelik M, Langford CA et al. 2021 American College of Rheumatology/Vasculitis Foundation guideline for the management of polyarteritis nodosa. Arthritis Rheumatol 2021 Aug; 73:1061-1070. PMID: https://www.ncbi.nlm.nih.gov/pubmed/34235889 https://onlinelibrary.wiley.com/doi/10.1002/acr.24633