UPDATES ON VASCULITIS IN CHILDREN
Asmaa Mahmoud Ahmed;
Abstract
V
asculitis is defined as the presence of inflammation in a blood vessel that may occur as a primary process or secondary to an underlying disease. The primary Vasculitisare unusual conditions with some of them such as HSP and KD being seen almost exclusively and commonly in children. These are defined by both the size of vessels involved and the type of inflammatory response.
The classification of childhood vasculitis are modified and adapted from the adult classification into large, medium and small vessel disease with the latter being subclassified into granulomatous and non-granulomatous varieties.
Clinical features consist of multi-organ involvement on a background of constitutional features reflecting the size and location of the blood vessels involved. While the commoner diseases such as HSP and KD are largely or wholly clinical diagnosis, the rarer varieties require sophisticated imaging and other investigations (auto antibodies) to reveal the correct diagnosis.
The clinical picture should give a clue to the choice of investigations with DSA or similar angiographic procedures being chosen for large/medium vessel disease and deep skin biopsies being helpful for medium and small vessel disease. Serology plays a small but distinct role in the identification of some vasculitides such as AAV.
Early detection and aggressive treatment is crucial as many of the vasculitides cause significant morbidity or mortality. Treatment options range from symptomatic therapy, immunosuppresive agents, intravenous immunoglobulin (IVIG) or biologic agents and are determined by the type of vasculitis, the severity of the inflammation, and the organ systems affected.
For some forms of vasculitis, such as KD, there are specific guidelines for treatment. For other vasculitides, the spectrum of care may range from supportive care with NSAIDs to immunosuppressive therapy with steroids alone or in combination with cyclophosphamide, azathioprine or other cytotoxic medications depending on the severity, the presence of organ involvement and which vascular bed is involved.
Biologic agents are slowly becoming available as efficacy is being shown in some of the childhood vasculitides. These agents bring with them a promise of better control of the more severe forms of vasculitis in children.
asculitis is defined as the presence of inflammation in a blood vessel that may occur as a primary process or secondary to an underlying disease. The primary Vasculitisare unusual conditions with some of them such as HSP and KD being seen almost exclusively and commonly in children. These are defined by both the size of vessels involved and the type of inflammatory response.
The classification of childhood vasculitis are modified and adapted from the adult classification into large, medium and small vessel disease with the latter being subclassified into granulomatous and non-granulomatous varieties.
Clinical features consist of multi-organ involvement on a background of constitutional features reflecting the size and location of the blood vessels involved. While the commoner diseases such as HSP and KD are largely or wholly clinical diagnosis, the rarer varieties require sophisticated imaging and other investigations (auto antibodies) to reveal the correct diagnosis.
The clinical picture should give a clue to the choice of investigations with DSA or similar angiographic procedures being chosen for large/medium vessel disease and deep skin biopsies being helpful for medium and small vessel disease. Serology plays a small but distinct role in the identification of some vasculitides such as AAV.
Early detection and aggressive treatment is crucial as many of the vasculitides cause significant morbidity or mortality. Treatment options range from symptomatic therapy, immunosuppresive agents, intravenous immunoglobulin (IVIG) or biologic agents and are determined by the type of vasculitis, the severity of the inflammation, and the organ systems affected.
For some forms of vasculitis, such as KD, there are specific guidelines for treatment. For other vasculitides, the spectrum of care may range from supportive care with NSAIDs to immunosuppressive therapy with steroids alone or in combination with cyclophosphamide, azathioprine or other cytotoxic medications depending on the severity, the presence of organ involvement and which vascular bed is involved.
Biologic agents are slowly becoming available as efficacy is being shown in some of the childhood vasculitides. These agents bring with them a promise of better control of the more severe forms of vasculitis in children.
Other data
| Title | UPDATES ON VASCULITIS IN CHILDREN | Other Titles | الحديـث فــي التهــاب الأوعيـة الدمويــة لـــدي الأطفـــال | Authors | Asmaa Mahmoud Ahmed | Issue Date | 2014 |
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