Pediatric emergency medicine trisk 741
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TABLE 101.7
COMPLICATIONS OF POLYARTERITIS NODOSA
Clinical entity
Renal failure
Symptoms and
signs
Investigations
Treatment
Usually insidious; Urinalysis (serial); Fluid, electrolyte
no symptoms
BUN; creatinine;
management
until uremia sets
creatinine
Treatment of
in
clearance; serum
hypertension
electrolytes
Peritoneal dialysis
Hemodialysis
Renal infarction Flank pain
Urinalysis; BUN;
Management of renal
creatinine
failure as given
High blood
above, hemodialysis
pressure
Renal arteriogram
Renal artery
Severe, sudden
Serial hematocrit
Management of shock
aneurysm with
flank pain; gross Renal arteriogram Surgical consult
hemorrhage
hematuria;
shock; palpable
abdominal mass
Hypertension
Asymptomatic or Serial measurement Diuretics
headache; retinal of BP; BUN;
Antihypertensive
changes;
creatinine,
agents
encephalopathy
creatinine
clearance; IVP
(or) renal
arteriogram
Pericarditis
Chest pain;
EKG; radiograph
Rest, steroids, removal
pericardial rub;
chest;
of fluid (if
pulsus
echocardiogram;
tamponade)
paradoxus (if
removal of fluid Caution: If tamponade
tamponade)
for analysis
is sudden, it may be
caused by ruptured
aneurysm with blood
in pericardium
Myocardial
Sudden chest pain; EKG (continuous
Pain relief; oxygen
infarction
shock;
monitor);
Circulatory support
arrhythmia;
echocardiogram; Heparin, thrombolytic
dyspnea;
thallium scan;
agents
congestive
coronary
failure
arteriography
Gastrointestinal
Abdominal pain;
Plain radiograph
Treat shock; block
hemorrhage
Gastrointestinal
perforation
Aneurysm with
rupture (intraabdominal)
Central nervous
system lesions
vomiting;
melena,
hematemesis, or
hematochezia;
shock;
tenderness and
guarding of
abdomen; bowel
sounds absent
Sudden abdominal
pain; shock;
guarding,
tenderness, and
rigidity of
abdomen; absent
bowel sounds
Abdominal pain
(chronic) with
acute
exacerbation
Palpable mass
Sudden onset of
shock
Convulsions;
gradual onset of
loss of
consciousness;
hemiparesis
abdomen
Peritoneal
aspiration
Endoscopy
Celiac arteriogram
bleeding vessel
during angiography;
surgical ligation
Plain radiograph
abdomen
(upright)
Treat shock
Surgical repair
Ultrasound
Celiac arteriogram
Treat shock
Surgical repair
Exclude
hypertensive
encephalopathy
CT scan, MRI
Carotid
arteriography
Supportive care
Control BP
Anticonvulsants
High-dose
corticosteroids
and/or
immunosuppressives
BUN, blood urea nitrogen; BP, blood pressure; IVP, intravenous pyelogram; EKG, electrocardiogram; CT, computed
tomography; MRI, magnetic resonance imaging.
Mesenteric thrombosis with infarction of the bowel may present with sudden
abdominal pain, vomiting, hematemesis or hematochezia, and shock. Exquisite
tenderness of the abdomen and absent bowel sounds are the major findings.
Hemorrhage from a ruptured aneurysm (mesenteric, hepatic, or renal) with
hemoperitoneum is heralded by sudden onset of severe pain, vomiting, tachycardia, and
shock. The abdomen is tender and tense, and bowel sounds are diminished or absent.
Initial management of each of these GI catastrophes includes volume replacement,
gastric decompression, and stress doses of corticosteroids. Very large volumes of IV
fluids may be required. Technetium scan, angiography of the celiac axis vessels, and
peritoneal aspiration may be indicated in some cases, and direct examination of the GI
tract by endoscopy may yield valuable information concerning the nature, location, and
extent of lesions. Surgical consultation should be obtained immediately, and in the
presence of bleeding aneurysms or infarcted bowel, exploratory laparoscopy or
laparotomy should be performed as soon as the patient can be stabilized.
CNS Complications. Clinical signs of CNS disease are less frequent than those of
peripheral nervous system involvement. Seizures and hemiparesis are the most
common manifestations of CNS involvement in PAN. CT angiogram, MRI with MRA,
and/or carotid angiography may help localize the lesion.
Management of hypertensive encephalopathy and increased intracranial pressure are
described elsewhere (see Chapters 97 Neurologic Emergencies and 100 Renal and
Electrolyte Emergencies ). Surgical correction of a ruptured aneurysm should be
undertaken if the bleeding vessel can be localized and is accessible.
Miscellaneous Complications. As with all vasculitides, PAN may involve testicular
vessels, leading to acute scrotal pain and purpura and accompanying dysuria. Once
other causes of scrotal pain are excluded, including epididymitis and testicular torsion,
treatment may proceed with steroids and immunosuppressive medications.
JUVENILE DERMATOMYOSITIS
CLINICAL PEARLS AND PITFALLS
Patients are at risk for aspiration pneumonia and respiratory insufficiency
because of muscular weakness, including weakness of the diaphragm.
Poorly controlled disease may be associated with GI manifestations of
vasculitis, including GI bleeding.
A rare complication is the development of cardiac conduction abnormalities.
Current Evidence
JDM is a rare rheumatic disorder characterized by inflammation of the blood vessels,
skin, and striated muscle. The annual incidence rate is roughly three cases per 1 million
children in the United States. Girls are more often affected than boys (2:1), as is typical
of most autoimmune conditions. The mean age of onset is estimated at 6.9 years in the
United States, with almost 20% of patients diagnosed at 4 years of age or younger.
Before steroid therapy was available, as many as one-third of patients died of the
disease and another one-third developed permanent disabilities. More recently, the
