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Henoch-Schonlein purpura :ribbon: (:leaves: S&S (Triad of symptoms…
Henoch-Schonlein purpura :ribbon:
:zap:
Def
One of the most common forms of Systemic vasculitis manifested in childhood
aseptic Systemic vasculitis & by deposition of Immune complexes that contains Ab
IgA
at
small vss
(arterioles & venules)
Also known as:
IgA vasculitis
Anaphylactoid purpura
Purpura rheumatica
Henoch-Schonlein purpura :star: M/C use this
Clinical Dx
:hearts:
:earth_americas:
Epidemiology
Common in
children
:male_sign: predominance
Associated with Inciting infections — Group A Streptococcus or other exposure
Occurs most frequently in spring & summer — ช่วงอากาศชื้น but Thailand เจอตลอดอยู่ละเพราะอากาศชื้น
:radioactive_sign:
Possible etiology
Infections
(มีนำมาก่อน)
Upper respiratory tract infection
Streptococcal infection
Others — food poisonings
Vaccinations
Insect bites
:runner::skin-tone-4:
Precipitating Ag
Infections
:grapes:
Drugs
:grapes:
:leaves:
S&S
Triad
of symptoms
Purpuric raised rash
@ skin of legs & buttocks
Abdominal pain
- bleeding into bowel wall and renal involvement
Arthritis
of ankles, knees & elbows
Palpable purpura
Renal involvement
Joint symptoms
Abdominal pain
Acute hemorrhagic edema of childhood (AHEC)
:gear:
PathoPSO
Complexes of IgA & C3 complements deposition on
small vss
(arterioles, capillaries, and venules)
High serum lvl of IgA (found only 50% of cases)
Involves skin, CNT, scrotum, joints, GI tracts & kidneys
:male-scientist::skin-tone-4:
Lab
no single test to confirm HSP
CBC: mayb mild leukocytosis; normal plt count
IgA levels
elevated
(only 50% of cases)
Skin Bx (then view via immunofluorescence)
Renal Bx
(only when Renal involvements เยอะ cuz its invasive — not done in every pt)
:bulb:
Dx
:star:Clinical ;
no need to Bx for Dx
Blood tests
Skin Bx
Renal Bx
Dx criteria
:grapes:
Needs
≥ 2
of these criteria
Palpable purpura
not related to thrombocytopenia
Age ≤ 20 yrs at ds onset
Acute abdominal pain
Granulocytes in the wall of small arterioles & venules on
Bx
(no need to immunofluorescence stain unlike old Dx criteria)
:lightning:
Ddx
:grapes:
:deciduous_tree:
Rx
Recovery in ~90%
Reccurence commoner in Older child & Adults
Adults go on to ESRD ~10%
:koala:
Supportive
Duration of illness usu 6 - 16 wks;
5-10% of pt will hv persistent or recurrent ds
:koala:
Drugs
Antispasmodics
ATBs
Anti-inflammatory drugs
include Systemic
corticosteroid
Corticosteroids
(Cons)
not been shown bene in Kidney ds or Dermal manifestations
doesn’t :arrow_down: chance of recurrence
doesn’t shorten duration of ds
(Bene) May
:arrow_down: tissue edema
aid in arthralgia & abd. pain
:green_heart:
I/C
Severe renal involvement — nephrotic syndrome
Severe abd. pain
GI bleed
Severe body edema
Severe swollen scrotum
Neuro symptoms
Pulmonary hemorrhage
:koala:
Plasmaphoresis (severe cases)