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SARCOIDOZA
SARCOIDOZA
Definitie o Boala multisistemica cu cauza necunoscuta o Afecteaza tineri si adultii de varsta mijlocie
Definitie
o
Boala multisistemica cu cauza necunoscuta
o
Afecteaza tineri si adultii de varsta mijlocie
o
Caracterizata histopatologic de granulomul
epitelioid necazeificant
o
Localizata frecvent la nivelul ganglionilor
hilari, plamanului, ochiului si pielii.
o
Alte localizari mai rar intalnite: ficat splina,
noduli limfatici, glande salivare, cord, sistem
nervos, muschi, oase,
Istoric o 1877 Jonathon Hutchinson descrie leziunile cutanate Sarcoidosis: Historical Time Line 1877 “Livid
Istoric
o 1877 Jonathon Hutchinson descrie leziunile cutanate
Sarcoidosis: Historical Time Line
1877
“Livid papillary psoriasis”
1889
“Lupus pernio”
1899
“Mutiple sarcoide cutanate cu character benign”
1914
Natura mutisistemica a bolii
1952
Primul raport despre beneficiul tratamentului cu coticoizi
2004
Etiologia ramane necunoscuta
PowerPoint® presentation from AZ-Air
Epidemiologie o Este depistata cu predilectie la adulti sub 40 ani o Cu “peak” la
Epidemiologie
o
Este depistata cu predilectie la adulti sub 40
ani
o
Cu “peak” la 20-29 ani
o
In tarile Scandinave si Japonia “peak”-ul este
la 50 ani
o
Frecventa usor mai crescuta la femei
(6.3♀/5.9♂ la 100000)
o
Populatia de culoare din America, Danemarca
si Suedia are prevalenta cea mai inalta din
lume (40/10.9 la 100000) pe cand in tarile
mediteraneene si arabe apare rareori []
Epidemiologie o PowerPoint ® presentation from AZ-Air Sarcoidosis: Geographic Prevalence Rates Prevalence/100,000
Epidemiologie
o PowerPoint ® presentation from AZ-Air
Sarcoidosis: Geographic Prevalence Rates
Prevalence/100,000 persons
Country
Men
Women
Study
United States
White
African American
United Kingdom
British born
Irish born
Caribbean born
Finland
Japan
Sweden
5.9
6.3
5.0
Henke et al.[20]
Gundelfinger and Britten [21]
47.8
Gundelfinger
and Britten [21]
27
27
97
213
197
170
22
34.4
4.9
7.0
16.5
21.7
James and Hosoda [22]; Brett [23]
James and Hosoda [22]; Brett [23]
James and Hosoda [22]; Brett [23]
Pietinalho et al. [24]
Pietinalho et al. [24]
Hillerdal et al. [25]
Etiopatogeneza o Factorul genetic n Predispozitia de agregare familiala intalnita la unele cazuri o Agenti
Etiopatogeneza
o
Factorul genetic
n Predispozitia de agregare familiala
intalnita la unele cazuri
o
Agenti din mediu:
n Infectiosi virali : Herpes S.
Epstein Barr
retrovirus
coxsackie B
citomegalovirus
Etiopatogeneza n Infectiosi bacterieni: Borellia burdorferi Propionibacterium acnes M Tuberculosis Mycoplasma n
Etiopatogeneza
n
Infectiosi bacterieni: Borellia burdorferi
Propionibacterium
acnes
M Tuberculosis
Mycoplasma
n
Substante anorganice: aluminiu,
zirconiu,
talc
n
Agenti organici: polen de pin
argila
Etiopatogeneza o T-cell receptor(TCR) n Rearanjarea genelor ce codifica receptorul limfocitelor T implicat in
Etiopatogeneza
o
T-cell receptor(TCR)
n Rearanjarea genelor ce codifica receptorul
limfocitelor T implicat in recunoasterea
antigenului din Complexul Major de
histocompatibilitate.
o
Factori imunologici:
n
Reactia imediata→ acumularea de celule T
activate si macrofage la locul inflamtiei
n
Majoritatea celulelor T sunt CD4 helper
→Eliberarea de IFNγ, IL2 si alte citokine
Etiopatogeneza n Hiperreactivitatea limfocitului B→ productie crescuta de imunoglobuline n Acumularea de
Etiopatogeneza
n
Hiperreactivitatea limfocitului B→
productie crescuta de imunoglobuline
n
Acumularea de monocite-macrofage cu
capacitete crescuta de prezentare a
antigenului si de exprimare a moleculelor
de adeziune
n
Eliberarea de citokine ce favorizeaza
fibrogeneza
SARCOIDOZA Pathogenesis of the inflammatory alterations in sarcoidosis PowerPoint ® presentation from AZ-Air
SARCOIDOZA Pathogenesis of the inflammatory alterations in sarcoidosis
PowerPoint ® presentation from AZ-Air
Patologia - granulomul epitelioid noncazeificant PowerPoint ® presentation from AZ-Air
Patologia - granulomul epitelioid noncazeificant
PowerPoint ® presentation from AZ-Air
Patologia o Localizarea granuloamelor: n Ganglioni llimfatici mai ales intratoracici n Plaman n Ficat n
Patologia
o
Localizarea granuloamelor:
n
Ganglioni llimfatici mai ales intratoracici
n
Plaman
n
Ficat
n
Splina
n
Piele
n
Ochi
o
Evolutia este spre fibroza
Transbronchial lung biopsy taken from a patient with pulmonary sarcoidosis. The photomicrograph shows well-formed
Transbronchial lung biopsy taken from a patient with pulmonary sarcoidosis. The photomicrograph shows
well-formed epithelioid granulomas adjacent to otherwise largely normal-appearing pulmonary interstitium.
(Courtesy of Dr. Robert Homer, Associate Professor of Pathology, Yale School of Medicine.) PowerPoint ®
presentation from AZ-Air
Transbronchial biopsy taken from a patient with atypical tuberculosis infection. This photomicrograph demonstrates a
Transbronchial biopsy taken from a patient with atypical tuberculosis infection. This photomicrograph demonstrates a
granuloma with central caseation, typical of mycobacterial infection. B, Transbronchial biopsy taken from a patient with
sarcoidosis. This photomicrograph demonstrates a typical sarcoid granuloma with a prominent multinucleated giant cell
without evidence of caseation. (Courtesy of Dr. Robert Homer, Associate Professor of Pathology, Yale School of
Medicine.) PowerPoint ® presentation from AZ-Air
Manifestari clinice o Manifestari clinice generale: n Febra, n Fatigabilitate n Malaise n Scadere ponderala
Manifestari clinice
o Manifestari clinice generale:
n
Febra,
n
Fatigabilitate
n
Malaise
n
Scadere ponderala
n
Transpiratii nocturne
Manifestari clinice o Manifestari clinice pulmonare: n Dispnee n Tuse seaca n Dureri toracice n
Manifestari clinice
o Manifestari clinice pulmonare:
n
Dispnee
n
Tuse seaca
n
Dureri toracice
n
Rareori hemoptizie
n
Rareori pleurezie
PowerPoint ® presentation from AZ-Air Staging of Sarcoidosis by Chest Radiography Stage Radiographic findings
PowerPoint ® presentation from AZ-Air
Staging of Sarcoidosis by Chest Radiography
Stage
Radiographic findings
Prognosis
0
Normal
I
Hilar, mediastinal, or paratracheal adenopathy
II
Hilar, mediastinal, or paratracheal adenopathy with
pulmonary parenchymal abnormalities
III
Pulmonary parenchymal abnormalities without
adenopathy
IV
Fibrobullous pulmonary parenchymal disease
Variable, depending on
extrapulmonary organ involvement
50%–75% have spontaneous
resolution
10%–40% remain stable
15%–30% progress to higher stage
Up to 60% have spontaneous
improvement
40%–60% remain stable or have
progressive disease
15%–35% have spontaneous
improvement
50%–60% remain stable
5%–10% progress to severe lung
disease
Irreversible
Manifestari clinice o Ganglionare: n adenopatii periferice palpabile, mobile, nedureroase n cervical, axilar,
Manifestari clinice
o
Ganglionare:
n
adenopatii periferice palpabile, mobile,
nedureroase
n
cervical, axilar, epitrohlear, inghinal
o
Splenomegalie minima
o
Cardiace:
n
aritmie,
n
bloc de grad inalt,
n
moarte subita.
Manifestari clinice o Hepatice: n usoara hepatomegalie n Modificari usoare ale testelor hepatice o Cutanate:
Manifestari clinice
o
Hepatice:
n
usoara hepatomegalie
n
Modificari usoare ale testelor hepatice
o
Cutanate:
n Eritemul nodos:
o
Leziuni nodulare, rosii, usor sensibile
o
Localizate pe fata anterioara a gambelor
o
Articulatii adiacente tumefiate si dureroase
o
Expresie a sarcoidozei acute
ERS Monography 2006 –Sarcoidosis cp 10
ERS Monography 2006 –Sarcoidosis cp 10
Manifestari clinice n Lupus pernio: o o o o o o Expresie a sarcoidozei cronice
Manifestari clinice
n Lupus pernio:
o
o
o
o
o
o
Expresie a sarcoidozei cronice
Placi indurate cu decolorare
Localizare la nivelul fetei (nas, obraji,
buze), urechi
Mai frecventa la femei Afro-
Americane
Afectarea mucoasei nazale
Nu sunt dureroase, pruriginoase si
nu se ulcereaza
ERS Monography 2006 –Sarcoidosis cp 10
ERS Monography 2006 –Sarcoidosis cp 10
Manifestari clinice o Oculare: n Uveita anterioara acuta n Uveita cronica→ glaucom, cataracta, o Neurologice:
Manifestari clinice
o
Oculare:
n
Uveita anterioara acuta
n
Uveita cronica→ glaucom, cataracta,
o
Neurologice:
n
Pareza faciala
o
Musculo-scheletale:
n
n
n
Dureri articulare genunchi, glezne, coate,
pumn,
Rareori miopatie in formele cronice.
Chiste osoase asociate leziunilor cutanate
cronice
ERS Monography 2006 –Sarcoidosis cp 12
ERS Monography 2006 –Sarcoidosis cp 12
Manifestari clinice o Gastrointestinale: n Afecteaza mai ales stomacul n Poate mima boala Crohn, TBC,
Manifestari clinice
o
Gastrointestinale:
n
Afecteaza mai ales stomacul
n
Poate mima boala Crohn, TBC, infectii
fungice
o
Hematologice:
n
Usoara anemie
n
Leucopenie
o
Parotidiene:
n Tumefierea glandelor parotide + febra +
pareza faciala + uveita anterioara = Sdr.
Heerfordt
Manifestari clinice o Endocrine: n Hipercalcemie, hipercalciurie (anomalii de productie a Calcitriolului) →
Manifestari clinice
o
Endocrine:
n
Hipercalcemie, hipercalciurie (anomalii
de productie a Calcitriolului) →
nefrocalcinoza, litiaza renala.
n
Diabet insipid← afectarea axului
hipotalamo-pituitar
n
Hipo/hipertiroidism
o
Renale:
n
Rareori nefrita interstitiala,
n
Insuficienta renala prin nefrocalcinoza
Sarcoidoza la copil o Are manifestari si localizari similare cu ale adultului o Prognostic mai
Sarcoidoza la copil
o Are manifestari si localizari similare cu
ale adultului
o
Prognostic mai bun decat adultul
Sarcoidoza varstnicului
o
Rareori se manifesta dupa 65 de ani
o
Poate mima patologia tumorala
Sarcoidoza in sarcina o Nu afecteaza major sarcina o Se poate agrava postpartum
Sarcoidoza in sarcina
o Nu afecteaza major sarcina
o Se poate agrava postpartum
Metode de diagnostic o Aspecte clinice + prezenta granulomului necazeificant + excluderea altor boli cu
Metode de diagnostic
o Aspecte clinice + prezenta granulomului
necazeificant + excluderea altor boli cu
manifestari clinice sau histologie similare
stage I sarcoidosis.
stage I sarcoidosis.
stage II sarcoidosis
stage II sarcoidosis
Stage III sarcoidosis.
Stage III sarcoidosis.
Stage IV sarcoidosis.
Stage IV sarcoidosis.
ERS Monography 2006 –Sarcoidosis cp 14
ERS Monography 2006 –Sarcoidosis cp 14
Endoscopia bronsica o Biopsia transbronsica o Biopsia de mucoasa bronsica → granulom necazeificant o Lavaj
Endoscopia bronsica
o
Biopsia transbronsica
o
Biopsia de mucoasa bronsica
→ granulom necazeificant
o Lavaj bronhoalveolar → CD4/CD8
>3,5
Diagnostice diferentiale o Tuberculoza (pentru orice localizare a granulomului) o Mycobacterioze o Granulomatoze o
Diagnostice diferentiale
o
Tuberculoza (pentru orice localizare
a granulomului)
o
Mycobacterioze
o
Granulomatoze
o
Alte boli infectioase
o
Formatiuni tumorale
Evolutia naturala o Remisie spontana in 2/3 cazuri o 1/3 -1/2 din cazuri sunt tratate
Evolutia naturala
o
Remisie spontana in 2/3 cazuri
o
1/3 -1/2 din cazuri sunt tratate cu
corticosteroizi
o
10-20% raman cu sechele
o
Formele cardiace, neurologice si renale
necesita tratament sistemic
o
Formele cutanate si oculare pot
beneficia de tratament topic
Tratamentul o Corticosteroizii o Metotrexat (in formele rebele la corticoid sau cronice) o Ciclofosfamida o
Tratamentul
o
Corticosteroizii
o
Metotrexat (in formele rebele la
corticoid sau cronice)
o
Ciclofosfamida
o
Azathioprina
o
Hidroxiclorochina (pt formele cutanate)
o
Ketokonazolul (in nefocalcinoze←
scade Ca seric)
PowerPoint ® presentation from AZ-Air Treatment for Pulmonary Sarcoidosis Observe patients with: Stage I di
PowerPoint ® presentation from AZ-Air
Treatment for Pulmonary Sarcoidosis
Observe patients with:
Stage I di s eas e, gi ven the li keli hood of s tabi li ty or
s pontaneous remiss i on;
Stage II or III disease who are asymptomatic and have normal pulmonary function; or
Stage II or III disease with minimal symptoms and mild pulmonary function abnormalities
Consider treatment with glucocorticoids in patients with:
Stage II or III disease with symptoms and/or pulmonary function abnormalities that do not
spontaneously improve after a period of observation (months)
Initiate treatment with glucocorticoids in patients with:
Stage II or III disease who are asymptomatic and have normal pulmonary function at baseline but who
develop symptomatic or physiologic worsening during a period of observation (months)
Stage II or III disease with symptoms and/or pulmonary function abnormalities at baseline that are
progressively worsening
PowerPoint ® presentation from AZ-Air Extrapulmonary Sarcoidosis : Treatment Opt ions other than Glucocorticoids Organ
PowerPoint ® presentation from AZ-Air
Extrapulmonary
Sarcoidosis : Treatment Opt ions other than Glucocorticoids
Organ
Cli nical
scenario
Treatment options (ot her than oral
corticosteroids)
Skin
Eye
Lupus pernio, disfiguring
skin lesions
Anterior chamber disease
Hydroxychloroquine; intralesion corticosteroid
injection
Topical corticosteroid alone may be adequate.
Liver
Transaminase elevation
Neurologic
Variable presentations
Patients with abnormal liver function tests in the
absence of symptoms may not require treatment.
Anticonvulsants if indicated.
system
Heart
Endocrine
Conduction system disease,
arrhythmia, congestive heart
failure
Hypercalcemia,
hypercalciuria
Pacemaker or implantable cardio?defibrillator
device placement; antiarrhythmic m edications;
treatment for left ventricular dysfunction
Reduction in calcium and vitamin D intake;
hydration
Pituitary
Pituitary insufficiency,
diabetes insipidus
Hormone replacement; water replacement for
diabetes insipidus
Musculoskeletal
Acute or chronic arthritis
Nonglucocorticoid anti? inflammatory agents
Bibliografie:
Bibliografie: