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Miastenija gravis je bolest koja zahvaća neuromišićnu spojnicu uzrokujući abnormalnu slabost, umor i propadanje muskulature. Dolazi do gubitka ili disfunkcije acetilkolinskih receptora u najvećem slučaju zbog prisutnosti protutijela na nikotinske acetilkolinske receptore (NAChR). Timus i timom imaju posebnu ulogu u patofiziologiji miastenije gravis zbog uloge timusa u sazrijevanju i distribuciji limfocita gdje može doći do križne reaktivnosti i posljedične autoimunosti na NAChR. Najčešće korištena dijagnostička imunološka pretraga za dijagnozu miastenije gravis je mjerenje razine protutijela na NAChR što je visokospecifični test za dijagnostiku miastenije gravis. CT je metoda izbora u dijagnostici timoma. Zbog važnosti timusa u patofiziologiji miastenije gravis bolest se može liječiti kirurški, uklanjanjem timusa. Ukoliko je prisutan timom za planiranje operativog zahvata služe Masaoka i TNM klasifikacija. Metode kojima se može odstraniti timus/timom su transsternalni pristup, transcervikalna timektomija, video asistirana torakoskopska kirurgija (VATS – video-assisted thoracoscopic surgery) te robotska kirurgija. Neki od faktora koji utjeću na prognozu nakon timektomije su dob i spol, prisutnost timoma, kirurška tehnika, obujam resekcije i težina i duljina trajanja simptoma miastenije gravis. Nove metode timektomije su minimalno invazivne i s brzim oporavkom, a većina pacijenata pokazuje remisiju bolesti nakon uklanjanja timusa., Myasthenia gravis is a disease that affects the neuromuscular junction causing abnormal weakness, fatigue, and muscle decay. Loss or dysfunction of acetylcholine receptors occurs mostly due to the presence of antibodies to nicotinic acetylcholine receptors (NAChR). The thymus and thymoma have a special role in the pathophysiology of myasthenia gravis due to the role of the thymus in the maturation and distribution of lymphocytes where cross-reactivity and consequent autoimmunity to NAChR can occur. The most commonly used diagnostic method for myasthenia gravis is the measurement of antibody levels to NAChR which is a highly specific test for the diagnosis of myasthenia gravis. CT is the method of choice in the diagnosis of thymoma. Due to the importance of the thymus in the pathophysiology of myasthenia gravis the disease can be treated surgically, by removing the thymus. If there is thymoma present, to plan the operation, Masaoka and TNM classification are used. Methods that can be used to remove the thymus / thymoma are transsternal thymectomy, transcervical thymectomy, video-assisted thoracoscopic surgery (VATS) and robotic surgery. Some of the factors influencing the prognosis after thymectomy are age and sex, presence of thymoma, surgical technique, extent of resection and severity and duration of symptoms of myasthenia gravis. New methods of thymectomy are minimally invasive and with rapid recovery, and most patients show remission of the disease after removal of thymus.

Svrha ovog preglednog rada je prikaz timektomije kao oblika liječenja u osoba oboljelih od miastenije gravis. Miastenija gravis je kronična, autoimuna, neuromuskularna bolest karakterizirana slabošću i zamaranjem skeletnih , poprečnoprugastih, mišića. U 80% oboljelih od miastenije gravis nalazi se abnormalnost timusa od čega 10-20% ima timom, a preostali dio hiperplaziju timusa. Timus je parenhimatozni organ sivocrvenkaste boje smješten u prednjem gornjem dijelu medijastinuma, iza prsne kosti. To je središnji organ limfatičnog sustava čija je glavna uloga prethodna obrada limfocita T. Poremećaji u obradi limfocita T imaju ulogu u patogenezi miastenije gravis. Liječenje miastenije gravis je simptomatsko, a cilj liječenja je potpuni nestanak simptoma. Timektomija, uz farmakološko liječenje, predstavlja oblik terapije za oboljele od miastenije gravis. Timektomijom se želi postići značajno poboljšanje simptoma kod bolesnika, prvenstveno smanjiti slabost mišića, zatim u najvećoj mogućoj mjeri reducirati farmakološku terapiju te u idealnom slučaju postići potpunu remisiju bolesti. Više različitih čimbenika, kao što su dob i spol bolesnika, težina simptoma, prisutnost timoma, prisutnost protutijela na AChR ili MuSK receptore, oblik bolesti, pa čak i trudnoća utječu na odluku o tome da li će osoba biti podvrgnuta timektomiji ili ne.Razlikujemo nekoliko glavnih kirurških pristupa: transcervikalnu, transsternalnu i VATS timektomiju. Nije potvrđeno da VATS timektomija ima veću učinkovitost u remisiji bolesti od transsternalne timektomije, ali minimalno invazivni postupci imaju niže stope morbiditeta i mortaliteta, manji broj komplikacija te je kraće vrijeme oporavka bolesnika., The purpose of this thesis is to present timectomy as a form of treatment in people suffering from myasthenia gravis. Myasthenia gravis is a chronic, autoimmune, neuromuscular disease characterized by weakness and fatigue of skeletal muscles. In 80% of patients with myasthenia gravis there is an abnormality of thymus, of which 10-20% has a thymoma, and the rest of them the thymus hyperplasia. Timus is a parenchymal organ of reddish-brown color placed in the front upper part of the mediastinum behind the chest bone. It is the central organ of the lymphatic system whose main role is the prior processing of lymphocytes T. Thymus plays a role in the pathogenesis of myasthenia gravis. The treatment of myasthenia gravis is symptomatic, and the goal of treatment is complete disappearance of symptoms. Timectomy, along with pharmacological treatment, is a form of therapy for people suffering from myasthenia gravis. Timectomy seeks to significantly improve clinical symptoms of the patient, primarily to reduce the muscle weakness, then to reduce the pharmacological therapy and ideally achieve complete remission of the disease. Multiple factors, such as age and sex, symptom weight, thymoma presence, presence of antibodies to AChR or MuSK receptors, the form of the disease, and even the pregnancy, influence the decision whether or not the subject will be operated. Major surgical approaches are: transcervical, transcernal, and VATS timectomy. It has not been shown that VATS timectomy has greater efficacy in remission of myasthenia gravis, but minimal invasive procedures have lower rates of morbidity and mortality, fewer complications, and shorter recovery time of the patient.

Myasthenia gravis is associated in 10 to 15 percent of patients with thymic tumors, rarely with aplastic anemia. We report a 45-year-old male diagnosed with myasthenia gravis ­associated with thymoma. We started treatment with pyridostigmine. After thymectomy, the patient ­received 30 irradiation sessions. In the postoperative course, he had mild worsening of myasthenia gravis, which improved with prednisone. Five months later, he developed severe aplastic anemia. He was dependent on blood supplement. After allogeneic transplantation of bone marrow, he improved but later he ­developed graft versus host disease. Myasthenia gravis was under good control with 480 mg of ­pyridostigmine per day., Miastenija gravis (MG) je u 10% do 15% bolesnika udružena s tumorima timusa, rijetko s aplastičnom anemijom. Prikazujemo 45-godišnjeg bolesnika s MG udruženom s timomom. Liječenje je započeto piridostigminom. Nakon timektomije je provedeno 30 zračenja. Poslijeoperacijski je imao blago pogoršanje MG koje se povuklo uz terapiju prednizonom. Pet mjeseci kasnije je razvio tešku aplastičnu anemiju. Postao je ovisan u krvnim derivatima. Nakon alogenične transplantacije koštane srži došlo je do poboljšanja, ali je kasnije razvio reakciju transplantata protiv primatelja. MG je bila dobro kontrolirana uz 480 mg piridostigmina na dan.