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FURTUNA IMUNOLOGICA PE CER SENIN

FURTUNA IMUNOLOGICA PE CER SENIN. DR. HUIDIU NICOLETA MEDIC REZIDENT REUMATOLOGIE AN I SPITALUL CLINIC „ DR. I. CANTACUZINO”. U.C . femeie 27 ani. 28.04.2014. MOTIVELE INTERNARII febra vesperala (38⁰C) astenie fizica marcata mialgii membre inferioare si superioare

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FURTUNA IMUNOLOGICA PE CER SENIN

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  1. FURTUNA IMUNOLOGICA PE CER SENIN DR. HUIDIU NICOLETA MEDIC REZIDENT REUMATOLOGIE AN I SPITALUL CLINIC „DR. I. CANTACUZINO”

  2. U.C. femeie 27 ani 28.04.2014 MOTIVELE INTERNARII • febravesperala (38⁰C) • asteniefizicamarcata • mialgiimembreinferioaresisuperioare • artralgii radio-ulno-carpienebilaterale • epistaxis, gingivoragiisimenoragie Peparcursulinternarii a prezentatgreata, varsaturialimentare

  3. APP • Alopecietotala de la 12 ani (peladauniversalis) ISTORICUL BOLII(03.2014) • Leucopenie3200/mm3 • Infectieurinaracu Klebsiella spp. Antibioterapiefebra(38-39⁰C) care se mentinesidupa switch-ulantibioticului, cu asociereaunuisindromdispeptic • internareclinicaBoliInfectioase CLINIC ● febra● sensibilitate la palpare in HD si E PARACLINIC ●Pancitopenie(leucopenie 1400/mm3 cu neutropeniesilimfopenie, trombocitopenie107 000/mm3 sianemieHb 10,5g/dl )● Sindrominflamatornespecific(VSH 60mm/h)● Sindromde citolizahepatica (TGO 252UI/ml, TGP 329UI/ml) ● Sindromde colestaza(GGT 180UI/ml) ⃰ Ag Hbs, ac anti HCV, ac anti HAV, virus Epstein Barr , VDRL negative ⃰ Hemoculturi negative

  4. ISTORICUL BOLII • ECOGRAFIE ABDOMINALA - Ficat cu dimensiunisistructuranormale, colecistdublucudatsiseptat, faradilatatii de CBP, VP, splina 12cm, faraadenopatii • ELASTOGRAFIE FICAT - 0 • ECOGRAFIE CORD - Faramodificari • ECOGRAFIE TIROIDA - Tiroidanormala, miciadenopatiilatero-cervicale cu caracterreactiv Manifestariclinicenespecifice+ anomaliiparaclinicenejustificate de trigger infectiosridicasuspiciuneauneiboliautoimune • profil ANA:ac. anti ADNdc, anti nucleozomi, anti histone, anti proteina P ribozomalapozitivi • Ac anti U1 RNP, anti Ro, anti La, anti Scl70, antiJo1, anti centromere, AMA M2 negative PacientaesteindrumataindrumatacatreClinica de Reumatologie

  5. INVESTIGATII PARACLINICE • Hb 8,5g/dl, trombocite68 400/mm3, leucocite1972/mm3 , reticulocite 3,02% • TGO 675,32UI/l, TGP 607UI/l • CK 292,72UI/L, LDH 502,58UI/l, PA 310,80 UI/l • GGT 1671,68UI/L, BT 1,66mg/dl, BD 1,24mg/dl • Trigliceride322,92mg/dl • Proteinetotale6g/dl • D dimeri4,72mcg/ml • Complement seric C3 0,24g/l • Fibrinogen 155g/dl • INR 0,87, PT 120,80% • Farasindrominflamator biologic • FR absent • Uree, creatininaglicemie, ionogramain limitenormale • Sumarurina: leucocite 75/mcl, hematii 1mg/dl, proteine 100mg/dl/, cilindrigranulosi/hialinirelativfrecventi, flora microbianarara • Proteinurie 664mg/24h

  6. INVESTIGATII PARACLINICE • ECG - tahicardiesinusala, 110bpm • EDS faramodificari • RMN cranio-cerebral - Mica leziune 4mm subcorticala frontal dreapta cu aspect nespecific (probabilmicroangiopatic)

  7. pacientatanara • alopecietotala • tablouclinic dominat de simptomegenerale, nespecifice (febra, asteniefizica, mialgiidifuze, inapetenta), artralgii RUC neinsotite de tumefactiisisindromhemoragipar • multipliparametribiologicimodificati (afectare hepatica severa, pancitopenie, coagulopatie, hipertrigliceridemie, proteinuriesiserologieANA pozitiva).

  8. DIAGNOSTIC DIFERENTIAL SEPSIS • Febra, disfunctie de organ, dar cu hemoculturinegative, raspuns favorabil la medicatieimunosupresiva CAUZE DE HEPATITA (trigger viral/ sindrom Reye/ toxicitatemedicamentoasa/autoimunitate) • Disfunctie hepatica, coagulopatie, darfaraicter, hepatomegalie, marker virali/ encefalopatie hepatica, edem cerebral, hipoglicemie, administrare de aspirina sau trigger infectios, modificarilebiologice au persistat la oprireamedicatiei/ farasindrominflamator, asocierea cu multiple alte anomaliibiologice HEMOPATII (limfom/ limfohistiocitozahemofagociticafamiliala) • Febra, microadenopatii, splenomegalie, citopenie, darfarascadereingreutate, transpiratiinocturne, trigger viral (EBV), serologiepozitivaspecifica LES, asocierea cu multiple alte anomaliibiologice

  9. DIAGNOSTICDIFERENTIAL BOALA STILL A ADULTULUI • Febra, artralgii, darfaraeruptiecutanatacaracteristica, leucocitoza, faringita* POLIMIOZITA • Asteniefizicamarcata, artralgii,CK, LDH crescute, darmialgiidifuze ale membrelor, farasindrominflamator biologic, ac. Anti Jo1 negativ, profilimunologic specific LES** LUPUS ERITEMATOS SISTEMIC • afectare renala, anemie, leucopenie cu limfopenie, trombocitopenie; ANA, ac. AntiADNdcpozitivi, complement C3 scazut (4C, 3I)*** SINDROM DE ACTIVARE MACROFAGICA • Disfunctie hepatica severa, pancitopenie, coagulopatie, hipertrigliceridemie, hipofibrinogenemie, hipoproteinemie, farasindrominflamator biologic, asociata cu o boalaautoimuna sistemica *cf. crt. Fautrel B et al, Medicine2002, 81 : 194-200 ** cf. crtBohan A, Peter JB. Polymyositis and dermatomyositis, N Engl J Med 1975; 292:344-347, 403-407 ***cf. crt. SLICC Petri M, et al. "Arthritis ad Rheumatism" aug. 2012 Totusi, nu toatemanifestarileclinico-biologice se pot datorauneisingurepatologii cu mecanismimunologic.

  10. DIAGNOSTIC POZITIV LUPUS ERITEMATOS SISTEMIC CU MANIFESTARI HEMATOLOGICE, MUSCULARE, ARTICULARE, RENALE ASOCIAT CU SINDROM DE ACTIVARE MACROFAGICA INFECTIE URINARA JOASA IN ANTECEDENTELE RECENTE PELADA UNIVERSALIS

  11. SINDROM DE ACTIVARE MACROFAGICA • SE ASOCIAZA CU LUPUS ERITEMATOS SISTEMIC, BOALA STILL A ADULTULUI, VASCULITE, ARTRITA JUVENILA IDIOPATICA; UN STATUS AUTOIMUN CU O INFECTIE SUPRAPUSA POATE DETERMINA HEMOFAGOCITOZA • ACTIVAREA HAOTICA SI PROLIFERAREA LIMFOCITELOR T SI A MACROFAGELOR, CU DIMINUAREA ACTIVITATII NK SI LTc, CU HIPERPRODUCTIA CITOKINELOR (INF, TNF, IL6, IL10, M CSF) SI HEMOFAGOCITOZA CU CITOPENIE • ENTITATE RARA, DIN GRUPUL LIMFOHISTIOCITOZELOR HEMOFAGOCITARE • NU EXISTA CRITERII CLARE DE DIAGNOSTIC • PROGNOSTIC NEFAVORABIL (MORTALITATE 20%), POTENTIAL RECURENTA • TRATAMENT IMUNOSUPRESOR IN DOZA MARE iv

  12. ATITUDINE TERAPEUTICA In cursulinternarii: • METILPREDNISOLON iv 250MG/ZI 6 ZILE • DEXAMETAZONA iv 8MG/ZI 4 ZILE • CICLOFOSFAMIDA iv 200MG/ZI 4 ZILE • PLASMA PROASPATA CONGELATA iv 1U/ZI 4 ZILE • CEFTRIAXONA iv FL 1G/12H • DALTEPARINA iv 7500U/ZI • ARGININA iv FI 1G/ZI • RANITIDINA iv 50MG/ZI la nevoie • METOCLOPRAMID iv 10MG la nevoie

  13. ATITUDINE TERAPEUTICA La externare: • Regimigieno-dietetic (exercitiufizic, regimalimentarnormocaloric, hiposodat, cura de diureza) • Tratamentmedicamentos cu: • Prednison 5mg 5 cp/zi cu scadereatreptata a dozelor (1/2cp la 2 saptamani) • Azatioprina 50mg 2cp/zi • Hidroxicloroquina 200mg 2cp/zi • Acid acetilsalicilic 75mg 1cp/zi • Acid ursodeoxicolic 500mg 2cp/zi • Silimarina 1g 1cp/zi • Alpha D3 0,5mcg 1cp/zi MONITORIZARE LA 2 SAPTAMANI A TESTELOR DE LABORATOR + REEVALUARE 4-6 SAPTAMANI

  14. EVALUARE ULTERIOARA (29.05.2014) CLINIC - Evolutie favorabila,castig ponderal,afebrila PARACLINIC • Hb10,2g/dl • Trombocite186 000/mm3 • Leucocite3230/mm3 • TGO 12,64UI/l • TGP 17,27 UI/l • CK 41,11UI/l • LDH 207UI/l Ddimeri0,51mcg/ml Complement sericC3 0,57g/l Fibrinogen 349mg/dl GGT 392UI/L BT 0,52 mg/dl Trigliceride123mg/dl Proteinetotale 6,01g/dl Proteinurie 931,50 mg/24h

  15. PARTICULARITATEA CAZULUI • LUPUS ERITEMATOS SISTEMIC ASOCIAT CU SINDROM DE ACTIVARE MACROFAGICA, CU INTRICAREA MANIFESTARILOR • LA O PACIENTA CU PELADA UNIVERSALIS (MECANISM PATOGEN AUTOIMUN) • PATOLOGIE COMPLEXA, IDIOPATICA • CU EVOLUTIE FAVORABILA SUB TRATAMENTUL ACTUAL INSTITUIT PROMPT • MONITORIZARE PENTRU PREINTAMPINAREA RECIDIVEI

  16. VĂ MULȚUMESC PENTRU ATENȚIE!

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