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Background
This vasculitis affects small to medium sized arteries usually within the kidneys, liver, gastrointestinal tract, central nervous sytem, and skin. It is a disease of adults presenting with constitutional symptoms of fever, weight loss, fatigue, arthralgia, and myalgia. Skin changes include purpura and ulceration, usually occurring on the lower extremities. There is a cutaneous variant with minimal systemic changes. Occasionally periosteal new bone formation may occur under these skin lesions.
The disease can occur in association with Crohn's disase, Kawasaki's syndrome, rheumatoid arthritis, and angioimmunoblastic lymphadenopathy. It is thought to arise from circulating immune complexes. There is a well documented association with Hepatitis B surface antigen positivity as well as a preceeding Streptococcal infection.
The disease has a variable course and may progress leading to death in 50% of cases at 5 years. Death is usually secondary to vasculitis occurring within the kidneys or gastrointestinal tract.
OUTLINE
EPIDEMIOLOGY CHARACTERIZATION AGE CHILDHOOD
- Cutaneous polyarteritis nodosa in children.
Fathalla BM, Miller L, Brady S, Schaller JG.
Division of Pediatric Rheumatology, Tufts University/New England Medical Center, Boston, Massachusetts, USA.
J Am Acad Dermatol. 2005 Oct;53(4):724-8. Abstract quote
The purpose of this study was to present the clinical courses and histologic findings of 4 children with cutaneous vasculitis characterized by tender cutaneous nodules and fever in the absence of major organ involvement.
We conducted a retrospective chart review of 4 patients with cutaneous vasculitis followed up for a mean of 68 months (range, 12-114 months). The patients included 3 boys and 1 girl (ages at onset, 2-10 years). Clinical and laboratory manifestations included tender erythematous cutaneous nodules (n = 4/4), fever 39 degrees C or higher (4/4), nondeforming arthritis (3/4), leukocytosis and elevated erythrocyte sedimentation rate (4/4), positive antinuclear antibodies (1/4), and elevated streptococcal enzymes (3/4).
Skin biopsy results showed inflammation of medium-sized cutaneous arteries with a mixed inflammatory cell infiltrate consistent with cutaneous polyarteritis nodosa (4/4). Patients were treated with prednisone with good initial response, but exacerbation occurred once prednisone was tapered. Additional medications given were methotrexate (2/4), dapsone (2/4), colchicine (1/4), and cyclophosphamide (1/4). One patient is in clinical remission after 48 months of disease; the others have continuing disease that requires treatment. Patients with evidence of streptococcal infection received oral penicillin prophylaxis; two of the three patients had recurrent attacks of vasculitis despite penicillin. No patients have developed major organ system involvement after 12 to 114 months of follow-up.
Cutaneous polyarteritis nodosa in children is a recognizable entity characterized by painful nodules, fever, absence of major organ involvement, and chronic or recurrent course. Patients should be screened for streptococcal infection and treated with antibiotics when needed.
LABORATORY/
RADIOLOGYCHARACTERIZATION CYTOKINES, SERUM
- Angiogenic cytokines in serum and cutaneous lesions of patients with polyarteritis nodosa.
Kikuchi K, Hoashi T, Kanazawa S, Tamaki K.
Department of Dermatology, Faculty of Medicine, University of Tokyo, Tokyo, Japan.
J Am Acad Dermatol. 2005 Jul;53(1):57-61. Abstract quote
BACKGROUND: Basic fibroblast growth factor (bFGF) and vascular endothelial growth factor (VEGF) are angiogenic cytokines, that have been reported to elevate serum levels in patients with collagen diseases such as systemic sclerosis and dermatomyositis as well as in those with inflammatory bowel diseases.
OBJECTIVE: In this study, the serum levels of bFGF and VEGF were measured by using enzyme-linked immunosorbent assay in 20 patients with polyarteritis nodosa (PAN; 5 with systemic PAN and 15 with cutaneous PAN) and in 20 control subjects. We also investigated the expression of bFGF and VEGF in cutaneous lesions of patients by using immunohistochemical methods.
RESULTS: Basic FGF was undetectable in the serum of control subjects, but detectable levels were found in 4 of 5 patients with classical PAN and 3 of 15 patients with cutaneous PAN. The serum bFGF level in these patients with systemic PAN was significantly elevated in comparison with that in healthy control subjects. The VEGF level was 178 +/- 41 pg/mL in the serum of healthy individuals. The mean VEGF level in patients with systemic or cutaneous PAN was significantly higher than that in healthy controls. Serum bFGF and VEGF levels in patients with systemic PAN were significantly elevated in comparison with those having cutaneous PAN. Immunohistochemical studies showed elevated bFGF expression on damaged endothelial cells in necrotizing vasculitis lesions. Elevated expression of bFGF was also observed on fibroblasts around the vasculitis. Some of the infiltrating cells around vasculitis lesions expressed VEGF.
LIMITATIONS: The study was small.
CONCLUSIONS: Serum bFGF and VEGF levels may be useful markers of the disease activities of PAN.
PROGNOSIS AND TREATMENT CHARACTERIZATION PROGNOSIS Long-term followup of polyarteritis nodosa, microscopic polyangiitis, and Churg-Strauss syndrome: analysis of four prospective trials including 278 patients.
Gayraud M, Guillevin L, le Toumelin P, Cohen P, Lhote F, Casassus P, Jarrousse B
French Vasculitis Study Group. H pital Avicenne, Bobigny, France.
Arthritis Rheum 2001 Mar;44(3):666-75 Abstract quote
OBJECTIVE: To determine the long-term outcome of patients with polyarteritis nodosa (PAN), microscopic polyangiitis (MPA), and Churg-Strauss syndrome (CSS), to compare the long-term outcome with the overall French population, to evaluate the impact on outcome of the type of vasculitis, prognostic factors, and treatments administered at diagnosis, and to analyze treatment side effects and sequelae.
METHODS: Data from PAN, MPA, and CSS patients (n = 278) who were enrolled between 1980 and 1993 were collected in 1996 and 1997 and analyzed. Two prognostic scoring systems, the Five-Factors Score (FFS) and the Birmingham Vasculitis Activity Score (BVAS), were used to evaluate all patients at the time of diagnosis.
RESULTS: The mean (+/- SD) followup of the entire population was 88.3 +/- 51.9 months (range 3 days to 192 months). Of the 85 deaths recorded, at least 41 were due to progressive vasculitis or its consequences. Death rates reflected disease severity, as assessed by the FFS (P = 0.004) and the BVAS (P < 0.0002), and the 2 scores were correlated (r = 0.69). Relapses, rarer in hepatitis B virus (HBV)-related PAN (7.9%) than in MPA (34.5%) (P = 0.004), occurred in 56 patients (20.1%) and did not reflect disease severity. Survival curves were similar for the subpopulation of 215 patients with CSS, MPA, and non-HBV-related PAN who were given first-line corticosteroids (CS) with or without cyclophosphamide (CYC). However, CS with CYC therapy significantly prolonged survival for patients with FFS scores > or =2 (P = 0.041). Relapse rates were similar regardless of the treatment regimen; only patients treated with CS alone had uncontrolled disease. CYC was associated with a greater frequency of side effects (P < 0.00001).
CONCLUSION: Rates of mortality due to PAN (related or unrelated to HBV), MPA, and CSS reflected disease severity and were higher than the mortality rate in the general population (P < 0.0004). Rates of relapse, more common in MPA than HBV-related PAN patients, did not reflect disease severity. Survival rates were better among the more severely ill patients who had received first-line CYC. Based on these findings, we recommend that the intensity of the initial treatment be consistent with the severity of the disease. The use of the FFS and BVAS scores improved the ability to evaluate the therapeutic response.
TREATMENT INFLIXIMAB
- Refractory polyarteritis nodosa successfully treated with infliximab.
Al-Bishri J, Riche NL, Pope JE.
Division of Rheumatology, Department of Medicine, University of Western Ontario, London, Ontario, Canada.
J Rheumatol. 2005 Jul;32(7):1371-3. Abstract quote
We describe a woman with severe polyarteritis nodosa (PAN) with visceral involvement unresponsive to multiple immunosuppressive drugs. Infliximab treatment was very effective in this case. Infliximab may potentially be used as an alternative agent for the treatment of patients with PAN refractory to conventional therapy.
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Last Updated October 4, 2010
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