0
AANPAANP
2010-72010-7
Robert Corliss, MD, Shahriar Salamat,Robert Corliss, MD, Shahriar Salamat,
MD PhD, Sara Zydowicz, DO...
2010-72010-7
 HistoryHistory
 A 3 1/2 old previously healthy girl had a sudden episode ofA 3 1/2 old previously healthy ...
H&E
10x
Diagnosis?Diagnosis?
DiscussionDiscussion
 Special Stains and immunohistochemical studiesSpecial Stains and immunohistochemical studies
and EMand EM
 PulmonaryPul...
 Differential diagnosis of granulomatousDifferential diagnosis of granulomatous
inflammation and aneurysm in medium sized...
 Diagnosis:Diagnosis:
 Rupture of vertebrobasilar artery aneurysmRupture of vertebrobasilar artery aneurysm
due to isola...
2010-72010-7
 Intracranial aneurysms in the pediatricIntracranial aneurysms in the pediatric
populationpopulation
 Rare ...
Pathogenesis of Giant Cell Arteritis (GCA)Pathogenesis of Giant Cell Arteritis (GCA)
 Innate and adaptive immune response...
REFERENCESREFERENCES
 Piggot, K. et alPiggot, K. et al Vascular damage in giant cell artertitisVascular damage in giant c...
During the DSS discussion, testing forDuring the DSS discussion, testing for
possible viral etiologies was recommended.pos...
AANP 2010-7 Robert Corliss, MD, Shahriar Salamat, MD PhD ...
AANP 2010-7 Robert Corliss, MD, Shahriar Salamat, MD PhD ...
AANP 2010-7 Robert Corliss, MD, Shahriar Salamat, MD PhD ...
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  • Mural thickening resulting from prominent intimal proliferation and active fibroplasia. Numerous isolated and clustered multi-nucleated giant cells, most notably in areas of inflammation. Although immunolabeling and silver stains revealed evidence of pulmonary histoplasmosis, but both were negative in the aneurysm
  • Transcript of "AANP 2010-7 Robert Corliss, MD, Shahriar Salamat, MD PhD ..."

    1. 1. AANPAANP 2010-72010-7 Robert Corliss, MD, Shahriar Salamat,Robert Corliss, MD, Shahriar Salamat, MD PhD, Sara Zydowicz, DOMD PhD, Sara Zydowicz, DO
    2. 2. 2010-72010-7  HistoryHistory  A 3 1/2 old previously healthy girl had a sudden episode ofA 3 1/2 old previously healthy girl had a sudden episode of unresponsiveness witnessed by her mother. Imaging studies ofunresponsiveness witnessed by her mother. Imaging studies of the still unresponsive child revealed severe, bilateral pulmonarythe still unresponsive child revealed severe, bilateral pulmonary edema (flash edema) and diffuse anoxic brain injury.edema (flash edema) and diffuse anoxic brain injury. Aggressive resuscitative efforts were unsuccessful and she wasAggressive resuscitative efforts were unsuccessful and she was pronounced dead.pronounced dead.  External exam was unremarkable. Internal examination of theExternal exam was unremarkable. Internal examination of the head revealed diffuse, acute basilar subarachnoid bloodhead revealed diffuse, acute basilar subarachnoid blood originating from a collapsed 2 cm irregular aneurysm along theoriginating from a collapsed 2 cm irregular aneurysm along the length of the basilar artery closer to the point of anastamosislength of the basilar artery closer to the point of anastamosis with the vertebral artery. Additionally, multiple calcifiedwith the vertebral artery. Additionally, multiple calcified subpleural, parenchymal and hilar pulmonary granulomas weresubpleural, parenchymal and hilar pulmonary granulomas were found.found.
    3. 3. H&E 10x
    4. 4. Diagnosis?Diagnosis? DiscussionDiscussion
    5. 5.  Special Stains and immunohistochemical studiesSpecial Stains and immunohistochemical studies and EMand EM  PulmonaryPulmonary  Multinucleated giant cells and lymphocytesMultinucleated giant cells and lymphocytes  Fungal organisms consistent withFungal organisms consistent with HistoplasmaHistoplasma speciesspecies  AneurysmAneurysm  No evidence of fungal organisms viaNo evidence of fungal organisms via special stains and immunohistochemicalspecial stains and immunohistochemical studiesstudies  No microorganisms via EMNo microorganisms via EM
    6. 6.  Differential diagnosis of granulomatousDifferential diagnosis of granulomatous inflammation and aneurysm in medium sizedinflammation and aneurysm in medium sized muscular arteriesmuscular arteries  Takayasu’s arteritisTakayasu’s arteritis  Kawasaki diseaseKawasaki disease  Varicella-zoster virus (VSV)Varicella-zoster virus (VSV)  Isolated angiitis of the CNSIsolated angiitis of the CNS  Polyarteritis nodosaPolyarteritis nodosa  Churg-Strauss syndromeChurg-Strauss syndrome  Wegener’s granulomatosisWegener’s granulomatosis  SLESLE  Sjogren’s syndromeSjogren’s syndrome  Rheumatoid arthritisRheumatoid arthritis  Bechet’s diseaseBechet’s disease  NeurosarcoidosisNeurosarcoidosis  Various infectionsVarious infections
    7. 7.  Diagnosis:Diagnosis:  Rupture of vertebrobasilar artery aneurysmRupture of vertebrobasilar artery aneurysm due to isolated intracranial giant cell arteritisdue to isolated intracranial giant cell arteritis
    8. 8. 2010-72010-7  Intracranial aneurysms in the pediatricIntracranial aneurysms in the pediatric populationpopulation  Rare during the first few years of lifeRare during the first few years of life  Usually symptomaticUsually symptomatic  Usually involvesUsually involves  Carotid arteryCarotid artery  Anterior communicating arteryAnterior communicating artery  Vertebrobasilar systemVertebrobasilar system  Case reports describe loss of tunica media andCase reports describe loss of tunica media and absent or discontinuous internal elastic mediaabsent or discontinuous internal elastic media
    9. 9. Pathogenesis of Giant Cell Arteritis (GCA)Pathogenesis of Giant Cell Arteritis (GCA)  Innate and adaptive immune responsesInnate and adaptive immune responses lead to vascular damage in GCAlead to vascular damage in GCA  Injury of the vascular wall is the result ofInjury of the vascular wall is the result of the cumulative effect of a cascade ofthe cumulative effect of a cascade of immune eventsimmune events  Early ‘danger’ signals trigger arteryEarly ‘danger’ signals trigger artery intrinsic dendritic cells (DC)intrinsic dendritic cells (DC)  Adaptive immune system is induced withAdaptive immune system is induced with resultant granulomatous infiltratesresultant granulomatous infiltrates  Vascular wall responds with maladaptiveVascular wall responds with maladaptive remodeling endangering dependent organsremodeling endangering dependent organs  Vascular DC dictate the arrangement andVascular DC dictate the arrangement and composition of T-cell responses in thecomposition of T-cell responses in the arterial wallarterial wall  Triggering of the DC by various TLRTriggering of the DC by various TLR ligands leading to vessel wall inflammationligands leading to vessel wall inflammation  TLR4 ligands induce release of CCL20TLR4 ligands induce release of CCL20 with subsequent recruitment of CCR6with subsequent recruitment of CCR6++ T-T- cells and establishment of wall-penetratingcells and establishment of wall-penetrating inflammationinflammation  TRL5 agonists facilitate recruitment of T-TRL5 agonists facilitate recruitment of T- cells with clustering in the adventitiacells with clustering in the adventitia  Immune cells and vessel wall cellsImmune cells and vessel wall cells collaborate in mediating vascular damagecollaborate in mediating vascular damage in GCAin GCA
    10. 10. REFERENCESREFERENCES  Piggot, K. et alPiggot, K. et al Vascular damage in giant cell artertitisVascular damage in giant cell artertitis. Autoimmunity,. Autoimmunity, October 2009; 42(7): 596-604October 2009; 42(7): 596-604  Lasjaunias, P. et alLasjaunias, P. et al Intracraninal aneurysms in children aged under 15Intracraninal aneurysms in children aged under 15 years: review of 59 consecutive children with 75 aneurysmsyears: review of 59 consecutive children with 75 aneurysms. Childs Nervous. Childs Nervous system (2005) 21: 437-350system (2005) 21: 437-350  Love, S. et alLove, S. et al Ruptured vertebrobasilar aneurysm associated with giant cellRuptured vertebrobasilar aneurysm associated with giant cell arteritis in a young boyarteritis in a young boy. Clinical Neurology and Neurosurgery 110 (2008). Clinical Neurology and Neurosurgery 110 (2008) 92-9692-96  Salvarani, C. et alSalvarani, C. et al Giant Cell Arteritis: Involvement of Intracranial ArteriesGiant Cell Arteritis: Involvement of Intracranial Arteries Arthritis & Rheumatism Vol. 55, No. 6, December 2006, pp 985-989Arthritis & Rheumatism Vol. 55, No. 6, December 2006, pp 985-989
    11. 11. During the DSS discussion, testing forDuring the DSS discussion, testing for possible viral etiologies was recommended.possible viral etiologies was recommended. Both the aneurysm and selected CNSBoth the aneurysm and selected CNS samples were probed for varicella zostersamples were probed for varicella zoster virus (immunohistochemically & by tissuevirus (immunohistochemically & by tissue PCR), as well as herpes simpex virus,PCR), as well as herpes simpex virus, Epstein-Barr virus, and cytomegalovirusEpstein-Barr virus, and cytomegalovirus (tissue PCR). All viral studies were negative.(tissue PCR). All viral studies were negative.
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