Polyarteritis Nodosa (PAN) is a systemic necrotizing vasculitis first described by Adolph Kussmaul and Rudolph Maier in 1866.
PAN is usually a disease of middle-aged and older adults.
Men tend to be more affected than women.
Typically affects medium-sized arterial vessels but small vessels may also be affected.
Unlike other small-sized arterial vessel vasculitides, polyarteritis nodosa is not typically associated with anti-neutrophil cytoplasmic antibodies (ANCA).
Most cases of PAN are idiopathic.
Secondary PAN is usually associated with hepatitis B, hepatitis C, and malignancies such as hairy cell leukemia.
PAN affects multiple organs including kidneys, skin, nerves, skeletal muscle, heart, gastrointestinal tract, and gonads.
Gastrointestinal tract involvement may be manifest as abdominal pain early in the disease process. The pain may be continuous or intermittent but often occurs after meals "intestinal angina." Nausea, vomiting, melena, pseudo- obstruction and diarrhea can also be seen. GI symptoms are a result of mesenteric arteritis, predominantly affecting the small intestines. Severe disease can lead to ischemia and perforation.
There is no definitive laboratory test for PAN.
Diagnosis is mainly clinical and organ specific supportive investigations.
A biopsy of an affected organ is gold standard for the diagnosis which typically shows segmental transmural necrotizing non-granulomatous inflammation of small to medium sized arteries of different stages.
Treatment: Depends on the severity of the disease and organs involved.
Prognosis: Untreated PAN has a poor prognosis; however, outcomes have significantly improved with treatment.
References
De Virgilio A, Greco A, Magliulo G, et al. Polyarteritis nodosa: A contemporary overview. Autoimmun Rev. 2016;15:564-70.
Guillevin L, Mahr A, Callard P, et al. French Vasculitis Study Group. Hepatitis B virus-associated polyarteritis nodosa: clinical characteristics, outcome, and impact of treatment in 115 patients. Medicine (Baltimore). 2005;84:313-22.
Hasler P, Kistler H, Gerber H. Vasculitides in hairy cell leukemia. Semin. Arthritis Rheum. 1995;25:134-42.
Pagnoux C, Mahr A, Cohen P, Guillevin L. Presentation and outcome of gastrointestinal involvement in systemic necrotizing vasculitides: analysis of 62 patients with polyarteritis nodosa, microscopic polyangiitis, Wegener granulomatosis, Churg-Strauss syndrome, or rheumatoid arthritis-associated vasculitis. Medicine (Baltimore). 2005;84:115-28.
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