Catastrophic Kawasaki Disease or Juvenile Polyarteritis Nodosa?

Yamazaki‐Nakashimada, Marco Antonio; Espinosa-Lopez, Margarita; Hernández-Bautista, Víctor Manuel; Espinosa-Padilla, Sara; Espinosa-Rosales, Francisco

doi:10.1016/j.semarthrit.2006.02.002

Cited by 21 publications

(18 citation statements)

References 47 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Both entities are vasculitides affecting small and medium vessels in children [8]. Early in the course either disease can have non-specific signs and symptoms such as fever or malaise.…”

Section: Discussionmentioning

confidence: 99%

Giant coronary artery aneurysms in juvenile polyarteritis nodosa: a case report

et al. 2012

View full text Add to dashboard Cite

Juvenile polyarteritis nodosa (PAN) is a rare, necrotizing vasculitis, primarily affecting small to medium-sized muscular arteries. Cardiac involvement amongst patients with PAN is uncommon and reports of coronary artery aneurysms in juvenile PAN are exceedingly rare. We describe a 16 year old girl who presented with fever, arthritis and two giant coronary artery aneurysms, initially diagnosed as atypical Kawasaki disease and treated with IVIG and methylprednisolone. Her persistent fevers, arthritis, myalgias were refractory to treatment, and onset of a vasculitic rash suggested an alternative diagnosis. Based on angiographic abnormalities, polymyalgia, hypertension and skin involvement, this patient met criteria for juvenile PAN. She was treated with six months of intravenous cyclophosphamide and high dose corticosteroids for presumed PAN related coronary vasculitis. Maintenance therapy was continued with azathioprine and the patient currently remains without evidence of active vasculitis. She remains on anticoagulation for persistence of the aneurysms. This case illustrates a rare and unusual presentation of giant coronary artery aneurysms in the setting of juvenile PAN.

show abstract

“…Both entities are vasculitides affecting small and medium vessels in children [8]. Early in the course either disease can have non-specific signs and symptoms such as fever or malaise.…”

Section: Discussionmentioning

confidence: 99%

Giant coronary artery aneurysms in juvenile polyarteritis nodosa: a case report

et al. 2012

View full text Add to dashboard Cite

show abstract

“…The vascular occlusion is considered to be, at least in part, due to activation of blood platelets [2]. Further complications of arteritis include anemia [1,[3][4][5]. At least in theory, anemia during arteritis may result from stimulation of eryptosis, the suicidal death of erythrocytes characterized by cell shrinkage and cell membrane scrambling with phosphatidylserine exposure at the erythrocyte surface [6].…”

Section: Introductionmentioning

confidence: 99%

Stimulated Suicidal Erythrocyte Death in Arteritis

Bissinger

Kempe-Teufel

Honisch

et al. 2016

Cell Physiol Biochem

View full text Add to dashboard Cite

Background/Aims: Arteritis is an inflammatory disease of the vascular wall leading to ischemia and vascular occlusion. Complications of arteritis include anemia, which could, at least in theory, result from suicidal erythrocyte death or eryptosis, which is characterized by erythrocyte shrinkage and phosphatidylserine (PS) exposure at the erythrocyte surface. Cellular mechanisms involved in the stimulation of eryptosis include increased cytosolic Ca²⁺-concentration ([Ca²⁺]_i), oxidative stress and ceramide formation. The present study explored whether and how arteritis influences eryptosis. Methods: Blood was drawn from patients suffering from arteritis (n=17) and from healthy volunteers (n=21). PS exposure was estimated from annexin V-binding, erythrocyte volume from forward scatter, [Ca²⁺]_i from Fluo3-fluorescence, reactive oxygen species (ROS) from DCFDA fluorescence and ceramide abundance from FITC-conjugated antibody binding in flow cytometry. The patients suffered from anemia despite 2.8±0.4% reticulocytes. Results: The percentage of PS-exposing erythrocytes was significantly higher in patients (1.1±0.1%) than in healthy volunteers (0.3±0.1%). The increase in PS exposure was paralleled by increase in oxidative stress and [Ca²⁺]_i but not by significant changes of ceramide abundance. Erythrocyte PS exposure and ROS production were significantly enhanced in erythrocytes exposed to patient plasma as compared to exposure to plasma from healthy volunteers. Conclusion: Arteritis is associated with enhanced eryptosis due to increased [Ca²⁺]_i and oxidative stress. The eryptosis contributes to or even accounts for the anemia in those patients. As eryptotic erythrocytes adhere to endothelial cells of the vascular wall, they could impede microcirculation and thus contribute to vascular occlusion.

show abstract

“…Although we suspected PAN rather than KD as the causative disease, we were unable to confirm PAN because further evaluation including skin biopsy was not done. PAN and KD are known to exhibit overlapping clinical and pathological features and result in systemic vasculitis; however, they are considered to be different diseases with different clinical manifestations [4].…”

Section: Discussionmentioning

confidence: 99%