Journal List > J Rheum Dis > v.24(1) > 1064353

Lee, Lee, Lim, Kim, Kwon, Park, and Choi: Four Cases of Polyarteritis Nodosa Presenting Initially as Pain and Pitting Edema in Both Lower Extremities

Abstract

Polyarteritis nodosa (PAN) has a broad spectrum of clinical presentation, since it affects small and medium-sized muscular arteries with microaneurysm formation, aneurysmal rupture with hemorrhage, thrombosis, and, consequently, organ ischemia or infarction. Although skeletal muscle involvement is well documented in patients with PAN, it can mimic more common diseases, and cause confusion and delays in diagnosis. PAN muscular involvement may have limited or early systemic forms with a benign course and excellent clinical response to corticosteroid therapy. Herein, we describe the clinical course and outcome of four unusual cases of PAN manifested by acute onset of pain and pitting edema in both lower extremities; in addition, we reviewed the relevant literature.

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Figure 1.
(A) Erythematous swelling in the right lower leg and (B) computed tomographic scan showed intramuscular central low density and peripheral enhancement, and superficial fas-cial thickening with subcutaneous fat edema (arrows) and (C) whole body positron emission tomography scan showed markedly increased fluorodeoxyglucose uptakes in bilateral lower leg muscles.
jrd-24-48f1.tif
Figure 2.
(A) Axial T2-weighted magnetic resonance imaging revealed diffuse edematous change and enhancement in the right thigh muscles (arrows) and (B, C) infiltration of vessel walls by neutrophils were seen histologically (H&E stain).
jrd-24-48f2.tif
Figure 3.
(A) Erythematous macular rash on the left lower extremity and (B, C) axial T2-wei-ghted magnetic resonance imaging in the right lower leg showed multifocal ill-defined signal abnormality in the muscles and subcutaneous layers (arrows).
jrd-24-48f3.tif
Figure 4.
Orbital computed tomographic scan revealed swelling and increased attenuation (arrow) of left periorbital soft tissue.
jrd-24-48f4.tif
Table 1.
Summary of clinical presentations in our case series
Variable Case 1 Case 2 Case 3 Case 4
Age (yr) 37 66 68 76
Sex Female Male Male Male
Chief complaint Calf pain Thigh pain Calf pain with fever Calf pain with fever
Onset 1 week ago 4 weeks ago 4 weeks ago 1 week ago
Associated symptoms Leg edema, arthralgia Leg edema, lower leg weakness Leg edema, scrotal swelling Left orbital swelling, scrotal swelling
CPK Elevated Normal Normal Normal
ESR/CRP Increased Increased Increased Increased
HBV serology Negative Negative Negative Negative
FFS 0 0 0 0
Biopsy site Gastroc-nemius Vastus lateralis Gastroc-nemius Gastroc-nemius
Effective treatment Prednisolone (1 mg/kg/d) Prednisolone (1 mg/kg/d) Additional methotrexate (12.5 mg/wk) Prednisolone (1 mg/kg/d) Additional methotrexate (12.5 mg/wk) Prednisolone (1 mg/kg/d) Additional methotrexate (12.5 mg/wk)

CPK: creatinine phosphokinase, ESR: erythrocyte sedimentation rate, CRP: C-reactive protein, HBV: hepatitis B virus, FFS: five-factor score.

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