University of Zagreb. School of Medicine. Department of Gynecology and Obstetrics.
Abstract
Mijastenija gravis (MG) je stečena autoimuna bolest koja nastaje kao posljedica
stvaranja protutijela na acetilkolinske receptore (AChR) završne motorne ploče čime
se narušava prijenos signala neuromuskularne spojnice. Usprkos nepoznavanju
točnog uzroka smatra se da je bolest multifaktorijalne etiologije. Dobna raspodjela MG
koja svoj vrh doseže u reproduktivnoj dobi žene zahtjeva pojačanu prekoncepcijsku,
antenatalnu i postpartalnu skrb. Prema raširenosti simptoma MG razlikuju se okularni
oblik s razvojem ptoze i diplopije te generalizirani oblik koji uključuje slabost
ekstraokularnih mišića. Mijastenička kriza sa zatajenjem disanja predstavlja hitno
neurološko stanje, a često je provocirana trudnoćom i postpartalnim razdobljem.
Dijagnoza MG se postavlja na temelju anamneze, fizikalnog pregleda, serologije te
dodatnih farmakoloških i elektrofizioloških ispitivanja. Liječenje uključuje inhibitore
acetilkolinesteraze, imunosupresivnu terapiju, timektomiju te intravenske
imunoglobuline i plazmaferezu. Prekoncepcijsko savjetovanje žena je neophodno
kako bi se osigurala kontrola majčine bolesti, a istovremeno izbjegle teratogene
posljedice imunosupresivne terapije na fetus. Trudnoća ima različite učinke na tijek
MG, a moguće su egzacerbacije, remisije ili nepromijenjen tijek bolesti. Tijekom
antenatalne skrbi provode se redoviti testovi plućne funkcije, individualna prilagodba
terapije te fetalni ultrazvuk u svrhu određivanja pokreta ploda. Zbog hemodinamskih
promjena prilagođava se doza lijeka, a odabir sigurne terapije u trudnoći može
predstavljati izazov. Prilikom odabira načina porođaja u većini slučajeva se pristupa
spontanom vaginalnom porođaju u epiduralnoj anesteziji. Dojenje se podupire uz
praćenje stanja novorođenčeta i majke zbog mogućnosti postpartalne egzacerbacije
u 30% slučajeva. Tranzitorna neonatalna mijastenija gravis (TNMG) nastaje kao
posljedica transplacentarnog prijenosa majčinih protutijela, a prepoznaje se unutar
prvih 12-48 h praćenjem kvalitete disanja novorođenčeta.Myasthenia gravis (MG) is an acquired autoimmune disease which occurs from the
production of antibodies targeting the acetylcholine receptors on the motor end plate,
thereby disrupting signal transmission of neuromuscular junction. Even though the
exact cause is still unknown, MG is considered to have multifactorial etiology. The age
distribution of MG that reaches its peak in the female's reproductive age indicates the
need for enhanced preconceptional, antenatal and postpartum care. According to the
distribution of symptoms, MG is classified into the ocular MG with the development of
ptosis and diplopia and the generalized MG which includes extraocular muscles
weakness. Myasthenic crisis with respiratory failure presents urgent neurological
condition and is frequently provoked during pregnancy and postpartum period. The
diagnosis is based on medical history, physical examination, serology, and additional
pharmacological and electrophysiological testing. Treatment includes
acetylcholinesterase inhibitors, immunosuppressive therapy, thymectomy,
intravenous immunoglobulins and plasmapheresis. Preconception counseling is
mandatory in an attempt to ensure disease control and to prevent teratogenic effect of
immunosuppressants at the same time. Pregnancy has various effects on the course
of the disease. Remission and exacerbation are possible while the severity of the
disease may remain unchanged. Antenatal care includes regular pulmonary function
tests, individual dose adjustment and fetal ultrasound to determine fetal movements.
Due to hemodynamic changes during pregnancy, drug dose adjustment and deciding
on a safe therapy in pregnancy can be very challenging. Spontaneous vaginal delivery
under epidural anesthesia is usually encouraged while choosing the mode of delivery.
Breastfeeding is recommended with continuous monitoring of the newborn and mother
due to the possibility of postpartum exacerbation in 30% of the cases.
Transient neonatal myasthenia gravis (TNMG) occurs as a result of transplacental
transmission of maternal antibodies and is recognized within the first 12-48 h when it
is important to monitor the quality of respiration