Transcript Henoch Schonlein Purpura

CASE CONFERENCE
July 18, 2012
15 year old male with a rash
HISTORY
4 days PTC
Developed a red rash on the palms and
soles
Intensely itchy
Discomfort while walking
2 days PTC
(+) Mild throat discomfort
(+) Low grade fever
Sought consult at the ED:
Impression – Coxsackie Virus infection
Tx: Diphenhydramine
Day of Admission
No relief from Diphenhydramine
Worsening of the rash
Difficulty in walking because of b/l ankle
pain
History
Review of
Systems
Denies vomiting, abdominal pain, changes
in bowel habits, and changes in urine
output
Past Medical
History
Nodular acne; has been on Doxycycline
100 mg daily x 5 months
Family History
Denies any medical/surgical problems
among immediate family members
Social History
Child lives in an apartment with parents
and siblings. (+) Pets at home. No recent
travel. HEAADDSS history noncontributory to the case
Physical Examination
General Appearance
Alert and awake, not in distress.
Cooperative
Vital Signs
Afebrile, 100/60, HR80; RR 20
Head, Eyes, Ears, Nose Throat,
Neck
NCAT, pinkish conjunctivae, anicteric
sclerae, nasal septum midline, TM’s intact,
dry oral mucosa, non-hyperemic OP,
supple neck, no CLAD
Chest and Cardiovascular
CTAB, no wheezes, +S1/S2, no murmurs
Abdominal Exam
Flat abdomen, normoactive bowel sounds,
no tenderness to palpations, no CVA
tenderness
Extremities
No edema, no cyanosis, brisk capillary
refill; No limitation in ROM
Neurologic Exam
No focal neurologic findings; Gait
difficulties
Physical Examination
ED Management
 Concerns for vasculitis – Basic labs sent, which included
coagulation panels
 Strep infection partially ruled out with RST
 Urinalysis
 RPR, Rickettsial antibodies
 ANA, RF
 Patient booked for admission for observation
Laboratory Tests
CBC
Chemistries
Parameter Result
s
Normal
Paramete
r
Result
s
Normal
WBC count
6.1
4.5-13.5
Na+
136
133-146
Hemoglobi
n
13.8
13-14.5
K+
4.1
3.4-4.7
Hematocrit
41.1
36-43
Cl-
106
98-107
Platelets
306
150-350
Bicarb
28
20-28
N
42
BUN
7
5-18
L
41
Crea
0.6
0.5-1
M
10
Glucose
97
60-100
Calcium
9.3
8.6-10
Laboratory Tests
CHEMISTRIES
Parameter
OTHERS
Result
s
Normal
ALT
16
10-40
AST
20
15-45
Bilirubin
0.5
0.3-1.2
Albumin
3.8
3.2-5.1
Total
Protein
7.3
6.0-7.9
Parameter
Results
C3
126
C4
32
RPR
Non-reactive
Rickettsial
Negative
ANA
Negative
RF
Negative
Laboratory Tests
Urinalysis
Parameter
Results
Color
Yellow
Clarity
Clear
SPG
1.029
pH
5.5
Proteins
TR
Glucose
Negative
Blood
Negative
WBC
3/hpf
RBC
1/hpf
Sq Cells
< 1/hpf
Henoch Schonlein Purpura
Vincent Patrick Tiu Uy, MD
PGY-2
History
Epidemiology
 Peak age of onset: 3-15
years old
 Exceedingly rare in the
adult population
 Males>Females
 Very common during the
cooler months and rare
during the summer
Pathogenesis
Possible Etiologies
 Upper Respiratory Tract Infections (~75%)
 Streptococcal infections
 Other infections
 Vaccinations
 Medications
 Insect Bites
Clinical Manifestations
Rash of HSP
Arthritis and Arthralgias
 Typically presents in 84% of patients with HSP, and is
the presenting manifestation in 15% of the cases.
 Oligoarticular (1-4 joints); Migratory; Mild
 > Ankles/Knees
 Usually no joint effusion and no swelling will be seen
 Toddlers and younger children will refuse to ambulate
 Does not cause permanent joint deformities
Gastrointestinal Symptoms
 Can range from mild
symptoms of
nausea/vomiting and
pain to significant events
like bowel angina and GI
bleeding.
 Colicky pain
 Massive GI hemorrhage
is rare
 Submucosal hemorrhage
and bleeding 
Mesenteric vasculitis
 Intussusception
Renal Disease
 20-54% of cases; usually in patients with persistent
rashes
 Long-term outcome determined by extent of kidney
involvement.
 Most common presentation is nephritic syndrome with
hematuria and mild/absence of proteinuria.
 Nephrotic range proteinuria and altered kidney function
tests predict a more progressive kidney disease
 Watch out for high blood pressure – this may be a clue!
 Refer to Renal
 Findings on kidney punch biopsy = IgA nephropathy
Nephritic vs Nephrotic Syndrome
Nephritic Syndrome
Nephrotic Syndrome
 Hematuria
 24 hour urine protein
>50 mg/kg/day
 Hypertension
 Azotemia
 Oliguria
 Low serum albumin
 Hypertension
 Hyperlipidemia
Other Presentations
 Scrotal Pain
 Central Nervous System
 Peripheral Nervous System
 Respiratory Tract
 Eyes
Differential Diagnosis
Condition
Presentation
AHEI
4 mos – 2 years; (+) Fever, purpura,
ecchymosis and edema; Resolves
spontaneously
Hypersensitivity
Vasculitis
After drug exposure; Fever, urticaria,
lymphadenopathy & arthralgias; Skin
biopsy has no IgA
Rocky Mountain
Spotted Fever
Presents with fever and rashes on the
palms and soles; caused by insect bite
SLE
Must satisfy 4/11 criteria for SLE
Meningococcemia
Patient appears more septic; may be
unvaccinated; Larger purpura and
ecchymosis
ITP/HUS
Platelet abnormalities are present
Reasons Behind Ancillary Procedures
Test
Reason
Complete Blood Count
Check platelets;
anemia/bleeding
Coagulation Studies
Bleeding
disorders/Coagulopathy
Urinalysis
Check for
hematuria/proteinuria
Serum Creatinine
Should be obtained if
urinalysis is abnormal;
always obtain in older
patients
Abdominal Ultrasound
(+) Severe abdominal pain
Skin Biopsy
Usually not necessary unless
manifestations are unclear
SUPPORTIVE Treatment of HSP
 Most patients may be treated on an outpatient basis
 Advise patients to rest until symptoms wear off
 Prognosis is generally good, especially if no renal
involvement
 STRICT Follow-up should be advised
Criteria for Hospitalization
1. Inability to maintain adequate hydration orally
2. Severe anemia requiring transfusion
3. Severe abdominal pain
4. Significant GI bleeding
5. Changes in mental status
6. Severe joint involvement limiting ability to move
7. Renal insufficiency, hypertension and nephrotic
syndrome
SYMPTOMATIC Treatment of HSP
 Pain control may be achieved with NSAIDS.
 No studies that relate worsening of GI bleeding in patients
given NSAIDS or cyclooxygenase inhibitors
 May give Naproxen, Acetaminophen or Ibuprofen
 Glucocorticoid use is controversial
 May be considered in hospitalized patients, symptoms that
are severe enough to prevent oral fluid intake or severe
joint symptoms that prevent ambulation.
 Not enough data to support that steroid provide rapid
improvement
Follow-up
Recovery
Weekly or
bi-weekly
BP + UA for
blood
Monthly BP
+ UA for
blood
Bi -monthly
BP + UA for
blood
2 months
~ 6 months
12 months
Obtain SERUM CREATININE
anytime if (+) abnormalities
HSP of the BRAIN leading to
CONFUSION!
As the expert in Pediatric Henoch-Schonlein Purpura in St.
Barnabas Hospital, you are called to see a 10 year old
female who presented with palpable purpura of the
buttocks and legs with pain on both knees. The doctor was
convinced that this is HSP – and she apparently sent for
labs. Which of the following laboratory work-up will make
the diagnosis of HSP stronger?
A. Complete Blood Count and Coagulation studies
B. CBC and Urinalysis
C. Urine Dipstick
D. Abdominal Ultrasound
E. Anti-Nuclear Antibodies
HSP of the BRAIN leading to
BRAIN INFARCT!!!
An otherwise healthy 15 year old male was seen in the ED
for rashes, arthralgia and abdominal pain. A diagnosis of
HSP was made and the ED attending booked him for
admission. You are the admitting resident on the floor.
Which of the following situation warrants admission?
A. A hemoglobin level of 12.0 mg/dL with nosebleed for 1
minute
B. Rash involving the face, upper trunk and groin in
addition to the typical leg and buttock rash
C. Patient was not responding to acetaminophen
D. Blood pressure of 140/80 with no proteinuria on dipstick
E. Fever of 101.2F and positive Guaiac test
THANK YOU!
I would like to thank Dr. Pertubal and Dr. Bhopi for the H&P
& Dr. Shafaghi for her guidance while managing this case