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Palarie-ntr-un picior, ghici ciuperca ce-i ?

Palarie-ntr-un picior, ghici ciuperca ce-i ?. Dr. Corina Rosca , Dr. Ioana Saulescu Sp.Cl . “ Sf.Maria ” Bucuresti. Motivele internarii. B, 73 ani Tumefactii maini bilateral debutate brusc de o saptamana Artralgii cu caracter inflamator RCC bilateral, umar stg Impotenta functionala.

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Palarie-ntr-un picior, ghici ciuperca ce-i ?

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  1. Palarie-ntr-un picior, ghici ciuperca ce-i? Dr. CorinaRosca, Dr. IoanaSaulescu Sp.Cl. “Sf.Maria” Bucuresti

  2. Motiveleinternarii • B, 73 ani • Tumefactii maini bilateral debutate brusc de o saptamana • Artralgii cu caracter inflamator RCC bilateral, umar stg • Impotenta functionala

  3. Istoric (1) • HTA esentialast II • 2005- Tromboza de arteracentrala a retinei OD • Aprilie 2010- oligoartritaasimetrica-> FR -, PCR =23 mg/dl, VSH=98 mm/h, FB=600 mg/dl -> Poliartritareumatoidaseronegativa-> Dexametazona intra-articular umardr + SSZ 2 g/zi-> ineficienteidupa 2 luni se renunta -> continua cu AINS -> Adenom de prostata -> Infectieurinara cu E. Coli- AB -> Diabetzaharat tip II – dieta

  4. Istoric (2) • Aprilie 2011- artralgiiglezne, coate, umeri + tumefactiigleznebilateral, debutate de aprox 5 luni, VSH=105 mm/h, FB=935 mg/dl, CRP=141 mg/l, FR=- -> Puls –terapieSolu Medrol 1,5 g- evolutiefavorabila-> MTX 7,5 mg/sapt+Prednison40 mg/zi cu doze scazande ->la Prednison 20 mg/zi - reaparesimptomatologia

  5. Examen clinic Edeme importante maini bilateral, cu godeu

  6. Examenlaborator • Hb=10.8 g/dl, Ht=33%,limfocitopenie, • VSH=67 mm/h, • CRP=112.22 mg/l • FB= 439.4mg/dl • FR=6.3UI/I • Chol=163 mg/dl, TG=77 mg/dl, • Ionograma =VN • Calciu=9.4 mg/dl • CK =33 U/L, CK-MB=1 U/L • Creatinina =0,9 mg/dl, uree =52 mg/dl • Glicemie= 134mg/l, • FA=57 UI/I, LDH=134 U/I, • Proteine serice totale=7g/dl, • TGO,TGP=VN • A. uric, uricozurie-VN • C3=1.36 g/l, C4= 0.36 g/l-VN

  7. Diagnostic diferential VSH=67 mm/h, CRP=112.22 mg/l FB= 439.4mg/dl FR=6.3UI/I Ac anti CCP-negativi Poliartritareumatoida Ecografie maini: Rg maini: Rg antepicioare: RCC stg -sinovita cu minim semnal Doppler RCC dr : fara eroziuni, fara sinovita, + tenosinovita de extensori cu semnal Doppler intens, tenosinovita flexori cu semnal Doppler de intensitate mai mica, important edem celular subcutan. modificari artrozice interfalangiene elemente artrozice MTF haluce bilat.

  8. Diagnostic diferential Spondilartrita Rg coloana lombara profil Rg bazin Debut cu oligoartrita asimetrica VSH=67 mm/h, CRP=112.22 mg/l FB= 439.4mg/dl Ag HLA B27-negativ Spatii SI pastrate Discaratroze

  9. Diagnostic diferential Sdr. paraneoplazic • Eco abd: ficat : discretafibrozaperi-portala, pancreas: mascat de gaze, prostata: calcificari in ambiilobi, ambiirinichi: faramodificari • EDS:esofag de aspect normal, stomac cu mucoasa in potiuneaverticalamaiedematiata, in special pe fata anterioara, intr-o ariesubcardiala, centrata de un micpolipsesil, diam 7 mm, care se biopsiaza. Portiuneamediegastrica cu mucoasaatrofica, DI, II, cu rare exudate punctiforme. Concluzii: gastritacronica, micpolipisesil gastric subcardial, duodenitaexudativa. • Biopsiepolip gastric: poliphiperplazic-HP prezent (3+) • Colonoscopie: aspect endoscopic normal pana in cec. Hemoroiziinternicongestionati • FA=57 UI/I • PSA=negativ

  10. Diagnostic diferential Sdr antifosfolipidic Sarcoidoza • Tomboza de artera centrala retiniana OD • Ac anti cardipolipina-negativi • Anticoagulantul lupic-echivoc, ulterior negativ • Faraleziunipleuro-pulmonare, adenopatiiaxilare • Angiotensin convertaza-VN

  11. Diagnostic diferential Polimialgie Condrocalcinoza • Manifestari simetrice • Rg-modificari degenerative, fara calcificari • Varstnic • VSH=67 mm/h, • CRP=112.22 mg/l • Absenta mialgiilor la nivelul centurilor

  12. Diagnostic diferential Panarterita nodoasa • VSH=67 mm/h • CRP=112.22 mg/l • Anemie normocroma, normocitara • Ag HBs, Ac anti HCV-negativi • P-ANCA, c-ANCA- negativi

  13. Diagnostic diferential Sdr. RS3PE (remitting seronegative symmetrical synovitis with pitting oedema) Ecografie maini RCC stg -sinovita cu minim semnal Doppler RCC dr : faraeroziuni, farasinovita, +tenosinovita de extensori cu semnal Doppler intens, tenosinovitaflexori cu semnal Doppler de intensitatemai mica, important edemcelularsubcutan Aspectul clinic al mainilor –edem cu godeu Debut brusc al simptomatologiei FR -negativ

  14. Diagnostic sitratament BAVP (maini, antepicioare, spondiloartroza) Tomboza de ACR OD DZ tip II controlat prin dieta Adenom de prostata HTA esentiala st II BCI – angor de efort Gastrita cronica Duodenita exudativa Angiopatie HTA st II Sdr(RS3PE)-sinovitasimetrica remisa cuedem parti moi Puls-terapie Solu-Medrol 1,5 g Remiterea completa a simpt. CRP= 17.98mg/l Calciu 500 mg 2 cp/zi Renitec 2 cp/zi Olicard 2 cp/zi Aspenter 75 mg 1 cp/zi Omeprazol 20 mg 1 cp/zi Aspacardin 2 cp/zi Plaquenil 200 mg 2 cp/zi Prednison 5 mg 2 cp/zi Vigantol 2 pic/zi sau 1 cp/zi 1000U

  15. Evaluare la 1 luna Ecografie maini • Hb=13.5 g/dl • VSH=38mm/h, • CRP=20.59mg/l, • Fibrinogen= 426.8mg/dl, • FR=1.3UI/I tenosinovita la nivelul extensorilor bilateral cu minim semnal Doppler. Plaquenil 2 cp/zi Prednison 5 mg 1 cp/zi

  16. Sdr. RS3PE- 1985 • Nu se cunoasteincidenta/prevalenta • Raport B/F=2/1 • Se asociaza cu HLA B7, A2, B27 • In patologiesunt implicate : VEGF (vascular endothelial growth factor), responsabilpentruhipervascularizatie (sinovita) sipermeabilitateacapilara (edemsubcutanat)/ Tenosinovitaextensoriloresteresponsabila de aparitiaedemului • Criterii de diagnostic: • pitting oedemaambelemaini, • debut brusc al poliartritei, • varstapeste 50 ani, • FR negativ • Poateasociafebrasiasteniefizica Mc Carty DJ, O' Duffy JD, Pearson L, Hunter JB. RS3PE syndrome. JAMA.  1985;254:2763–2767. doi: 10.1001/jama. 254.19.2763. 

  17. Sdr. RS3PE • Rg- absentaeroziunilor • “Hallmark”: tenosinovita de extensorisiflexori • RS3PE se poateasocia cu: tumorigastrice, pancreatice, limfoamenon-Hodgkin, PR, Sp A, condrocalcinoza, amiloidoza • RS3PE –considerat un subset al poliartriteireumatoide, cu prognostic favorabil • Formele care raspundgreu la GC pot fi paraneoplazice Remitting seronegative symmetrical synovitis with pitting oedema (RS3PE) syndrome: a prospective follow up and magnetic resonance imaging study-F. Cantini, C. Salvarani, I. Olivieri, L. Barozzi, L. Macchioni, L. Niccoli, A. Padula, P. Pavlica, and L. Boiard Ann Rheum Dis. 1999 April; 58(4): 230–236

  18. PROGNOSTIC • Foarte bun -> remiterea in totalitate a simptomatologiei, fara ca pacientul sa ramana cu deficit functional (desi poate sa persiste o usoara contractura a flexorilor de la nivelul mainilor). • 20 % din pacientii -> recaderi, dar care raspund la corticoterapie in doze mici • La un nr. mic de pacienti acest sindrom a precedat aparitia limfomului cu celule T si a sindromului mielodisplazic RUBEN QUEIRO, MD, PhD; MERCEDES ALPERI, MD; JOSE LUIS RIESTRA, MD, PhD; JAVIER BALLINA, MD, PhD, Rheumatology Service, Hospital Universitario Central de Asturias, C/ Celestino Villamil s/n, 33006 Oviedo, Asturias, Spain

  19. CONCLUZII 1. Sindromul RS3PE este o afectiunefoarterara. 2. Diagnosticulacesteiafectiuni se face prinexcludereaaltorboli. 3. Sindromul RS3PE poatesapreceadaaparitiaaltorbolireumatologicesineoplazice. 4.Pacientii trebuiescatentmonitorizati in eventualitateadezvoltariineoplaziilor.

  20. VA MULTUMESC!

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