Transcript Vasculitides

Vasculitides
Premchand Anne, MD, MPH
PGY II (aka the class that rocks)
Internal Medicine/Pediatrics
Objectives
basic principles of vasculitis
 cases/discussion/didactic
 questions
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Vasculitis
Inflammation of blood vessels and its
consequences
 Classification
 Pathology of vasculitis
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– Role of Immune complexes, complement,
in vasculitis
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Presentation: purpura vs. nodule
Doctor, Doctor, my son has a
rash for three days.
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8 year old male who is very active in sports, presents with
complaints of rash and swelling of the feet and ankles.
Pt’s mother noticed a progressive bumpy red rash and the
swelling over the past three days. Rash is also present on
buttocks bilaterally and onset similar to that of the feet.
Low grade fevers for a week, no nausea or vomiting but
reported frothy urine and intermittent abdominal pain over
the past few hours. History of a URI in the family,
including the child, 2-3 weeks ago. No family history. No
PMHx.
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QUESTIONS?
DIFFERENTIAL DIAGNOSES?
WORK UP?
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Henoch Schonlein Purpura
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Self limited vasculitis of the small vessels
Occurrence 9/100000 with male predominance
2-8 years of age, most common
IgA and C3a mediated disease and can occur with
other
Renal involvement leads to nephrotic syndrome
Clinical diagnosis
– Arthritis in knees and ankles
– Angioedema in dependent areas with palpable lesions
– Intermittent abdominal pain with possibility of
intussusception (3%) with currant jelly stools.
Henoch Schonlein Purpura cont...
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Differential diagnosis
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PAN with neuro and cardiology involvement
Meningococcemia for the rash
Kawasaki disease-rash limited to LE, high fever
JRA-salmon pink rash that is fleeting
Flea and spider bites
Labs: Def. Dx: bx of lesion shows leukoclastic
angiitis, Bx of renal shows IgA nephropathy and
segmental GN with crescents, elevated ESR, and
elevated serum IgM and IgG
Therapy: symptomatic; high dose steroids if
significant GI, Neuro, and renal involvement and
baby aspirin if hypercoaguble
Doctor, Doctor, my child has a
high fever and a red tongue
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3 year old Asian girl is brought in for persistent fevers of
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102-104 F for more than two weeks. Patient was taken to
primary pediatrician and was given antibiotics for a
sorethroat 10days ago but no improvement in fevers, neck
masses or the redness of the tongue and oral cavity.
Fevers are improving but the girl has conjunctival injection
and has periungual and perianal desquamation and
incredibly irritable and poor appetite. No N/V/D/myalgias
but reporting joint pains.
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QUESTIONS?
DIFFERENTIAL DIAGNOSES?
WORK UP?
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Kawasaki Disease
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Aka MUCOCUTANEOUS LYMPH NODE SYNDROME
febrile vasculitis of childhood
cause is unknown
unlikely in <4 months due to maternal Ab
80% are under 5 years of age
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Path: severe inflammation of medium sized vessels, with
strong affinity for the coronary arteries, leading to
destruction of the intimal layer (all 3 layers involved in
severe cases)
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Cardiac disease: MOST COMMON, decreased ventricular
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function 2 myocarditis, pericarditis, and coronary
aneurysms, thrombosis, stenosis, and sudden death.
Kawasaki disease cont...
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Clinical manifestations:
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Acute phase: 1-2 weeks with fevers upto 104F and
unresponsive to Antibiotics
Subacute phase: irritability, anorexia, conjuctival
infection, desquamation of periungual and perineal
regions and arthritis
Convalescent stage: 6-8 weeks post onset of disease
bilateral bulbar conjunctival injection without exudate
strawberry tongue and oral erythema
erythema and edema of arms and legs
CERVICAL LYMPHADENOPATHY > 1.5 cm
Rash
Differential Diagnoses:
– Scarlet fever, TSSS, Measles, SJS, JRA, and RMSF
Kawasaki disease cont...
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Labs: 2D-Echo absolutely pertinent due to extensive
coronary involvement; Ig profile due to elevations in
subacute phase, coag studies. Follow up 2D
absolutely recommended
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Therapy:
– IVIG: decreases CAD by 75% (2g/kg over 10 hours)
– ASA for hypercoagulable state due to thrombocytosis
Doctor, Doctor, I have painful
marks on my legs
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25 y/o asian indian female presents with complaints of
nightsweats, anorexia, weight loss, fatigue, and myalgias
of few weeks duration. She reports coming to the US 3
months ago. In addition to the above symptoms, pt
noticed severely painful nodular lesions in the anterior
aspects of her legs. Pain is unremitting. Physical exam
reveals a thin women in distress secondary pain, (+)
carotid bruit on the right, widened and laterally displaced
PMI, and Left chest pain relieved with leaning forward.
AVSS. (+) splenomegaly. LE exam with nodular lesions
resembling erythema nodosum and tender to touch.
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QUESTIONS?
DIFFERENTIAL DIAGNOSES?
WORKUP?
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TAKAYASU ARTERITIS
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Vasculitis affecting mostly large caliber vessels, mainly
aortic arch branches
predominant in Asian and Indian subcontinent peoples,
women >>men predominance.
1/3 before the age of 20
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Path: preference for aorta and major branches with
significant renal disease (GN). Association with TB
exposure and HLA-B
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Differential Diagnoses:
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Cogan syndrome
Behcet’s disease
Reiter syndrome
Sarcoidosis
TAKAYASU ARTERITIS CONT...
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Clinical manifestations:
– pre-pulseless:
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nightsweats
anorexia
weight loss
fatigue
myalgia
– pulseless:
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splenomegaly
erythema nodosum, syncope, amaurosis fugax
dilated CM, myocarditis, and pericarditis
(+) vascular bruit.
Labs: ESR>60, microcytic hypochromic anemia
Confirmation of Dx: angiography for occlusive and
aneurysmal disease
TAKAYASU ARTERITIS CONT...
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Therapy
– surgical excision of stenotic lesions and
management of dilated aorta if indolent disease
– Immunosuppressives:
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prednisone (1mg/kg/day) in early stage,
cyclophosphamide and methotrexate if refractory to
steroids
Doctor, Doctor, my child needs
your help
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10 year old child presented with complaints of
persistent fevers with diagnosis of FUO, diarrhea,
skin lesions, numbness and tingling with muscle
weakness for the past few days. Physical exam
with temp of 101.1, erythematous and necrotic
skin lesions on the leg and lateral aspect of the
ankle, similar to stasis ulcers, and Left foot drop
QUESTIONS?
DIFFERENTIAL DIAGNOSES?
WORKUP?
POLYARTERITIS NODOSA
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Clinical manifestations
– FUO, mononeuritis multiplex
– depends on variety of vessels involved
– multiple systems affected, including peripheral nerves,
mesenteric vessels, heart and brain
– often associated with p-ANCA in adults but classis PAN
is ANCA negative.
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Diagnosis: Biopsy of tissue in symptomatic sites is
highly sensitive and specific and ANGIOGRAPHY for
dilatation of arteries.
Differential diagnosis
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HSP
Wegener’s
good pasture’s
Churg strauss
POLYARTERITIS NODOSA cont...
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Labs:
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ESR
abnormal urine
Hep B and C serology (cause 10-30%)
increased B-cell lymphocytes
hydralazine, allopurinol, penicillamine causes
Therapy
– Steroids
– cytoxan
– prostacyclin
Doctor, Doctor, my teenage
son coughed up blood
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15 y/o male with hx of chronic sinusitis and
congestion, here with complaints of
hemoptysis and dyspnea but stable now.
QUESTIONS?
DIFFERENTIAL DIAGNOSIS?
WORKUP
WEGENER’S
GRANULOMATOSIS
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Differential diagnosis
– sarcoid
– TB
– Churg Strauss
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Epidemiology of WGN
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mostly kidney and lung with granuolomas
adults (4th and 5th decades) >>>kids
caucasians >>>noncaucasian
initially with respiratory symptoms and then renal
insufficiency
WEGENER’S
GRANULOMATOSIS
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Diagnosis: Clinical findings of sinusitis and pulm
finding, order high resolution CT. Check ANCA:
(90% of WGN have the marker) with 70%
sensitivity
Bx:
– lung: granulomas
– renal: 10% with granulomas, segmental necrotizing GN
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RX: rapid therapy needed
– Steroids
– cyclophosphamide
OTHERS
Behcet’s disease =>f/u CT scan of brain
for ocular involvement.
 Cogan syndrome: autoimmune hearing
loss, aortitis and keratitis
 Buerger’s Disease in a male <40 yrs
smoker with claudication, rest pain
 SLE arteritis
 Temporal arteritis with polymyalgia
rheumatica
 Churg-Strauss Syndrome
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Behcet’s
Cogan’s
References:
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Nelson’s Pediatrics
Harrison’s Internal Medicine
Google search for pictures
AAP Board PREP
CMDT 2004