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Classical polyarteritis nodosa and microscopic polyangiitis--a clinicopathologic study
1Department of Medicine, All India Institute of Medical Sciences, New Delhi.
Objective:
To describe the clinical spectrum, laboratory features, histopathological findings and treatment outcome in patients with classical polyarteritis nodosa (PAN) and microscopic polyangiitis (MPA).
Material And Methods:
Patients with PAN and MPA seen at a large teaching hospital in north India over a period of five years (1994-99) were included in the present study.
Results:
We encountered five patients with PAN and six patients with MPA during the study period. Of the five patients with PAN, two had systemic disease while three had limited PAN. The patients with limited PAN included two with cutaneous PAN and one with PAN confined to the nerves. Constitutional symptoms, musculoskeletal complaints, peripheral neuropathy and skin lesions dominated the clinical picture. Fifty percent of the MPA patients presented as pulmonary renal syndrome. All the patients with PAN were HBsAg and ANCA negative and had normal urinalysis findings. In contrast, all patients with MPA demonstrated an active urine sediment and 83.3% were pANCA positive. Some of the rare features encountered by us were the presence of antiphospholipid syndrome and extensive interstitial lung disease in MPA, and spontaneous recovery in one patient with systemic PAN. Treatment outcome was better in PAN as compared with MPA.
Conclusions:
The clinical spectrum of PAN and MPA is quite varied. A good outcome is possible with the use of corticosteroids and cyclophosphamide.
Insights
Polyarteritis nodosa (PAN) and microscopic polyangiitis (MPA) present with diverse symptoms. Corticosteroids and cyclophosphamide offer a good prognosis for both vasculitis conditions.
Area of Science:
- Rheumatology
- Internal Medicine
- Pathology
Background:
- Polyarteritis nodosa (PAN) and microscopic polyangiitis (MPA) are distinct forms of systemic vasculitis.
- Understanding their varied clinical presentations and outcomes is crucial for effective management.
Purpose of the Study:
- To delineate the clinical spectrum, laboratory findings, histopathology, and treatment outcomes of classical PAN and MPA.
- To compare the characteristics and prognoses of patients with PAN and MPA.
Main Methods:
- Retrospective analysis of patients diagnosed with PAN and MPA at a North Indian teaching hospital from 1994-1999.
- Review of clinical data, laboratory results, urinalysis, serological markers (HBsAg, ANCA), and histopathological findings.
Main Results:
- Five patients with PAN (two systemic, three limited) and six with MPA were identified.
- Constitutional symptoms, musculoskeletal complaints, neuropathy, and skin lesions were common in PAN.
- MPA patients frequently presented with pulmonary-renal syndrome; 83.3% were pANCA positive and had active urinary sediment.
- Rarely observed: antiphospholipid syndrome and interstitial lung disease in MPA; spontaneous recovery in one systemic PAN case.
- Treatment outcomes were more favorable in PAN compared to MPA.
Conclusions:
- PAN and MPA exhibit a broad and varied clinical spectrum.
- Effective treatment with corticosteroids and cyclophosphamide can lead to favorable outcomes in both conditions.