Pulmonary necrotizing sarcoid granulomatosis

Osszefoglalo. A nekrotizalo sarcoid granulomatosis a granulomatosus pulmonalis angitisek koze tartozo, ritka korkep. Egyesek a sarcoidosis variansanak, masok primer pulmonalis vasculitisnek tartjak. A korkep klinikai es patologiai jellegzetessegeit ket eset bemutatasaval ismertetjuk. A 20 eves nőbeteg surgősseggel kerult pulmonologiai osztalyra szaraz kohoges, jobb oldali, mely belegzessel osszefuggő mellkasi fajdalom es laz miatt, a 63 eves ferfi beteget pedig pneumoniat kovető kontroll-mellkasrontgenfelvetelen latott elvaltozas kivizsgalasa soran eszleltek. Az autoimmun panel vizsgalata, a mikrobiologiai tesztek mindket betegnel negativnak bizonyultak, a legzesfunkcios vizsgalat es a bronchoszkopos vizsgalat nem talalt elterest. A mellkas-CT-felvetelen lagyresz-denzitasu nodulusok latszottak egyoldali dominanciaval, a folyamatot nem kiserte a hilusi nyirokcsomok szimmetrikus megnagyobbodasa. A nodulusok szovettani vizsgalata valt indokoltta, melyet videoasszisztalt torakoszkopos tudőreszekcios mintavetellel biztositottak. Mikroszkoposan a tudőparenchymaban gocos nekrozisokat, a kornyezetukben el nem sajtosodo epitheloid sejtes granulomatosus gocokat, az atfuto arteriakban pedig granulomatosus arteritist lattak; a klinikai adatok figyelembevetelevel a tudő nekrotizalo sarcoid granulomatosisa diagnozisat allitottak fel. A tudőbetegseg mindket betegnel egy ev alatt spontan regredialt. Az irodalom adatait es az eseteket osszegezve, a tudő nekrotizalo sarcoid granulomatosisaban mikrobiologiai vizsgalatokkal nem igazolhato tudőfertőzes, es az immunologiai kivizsgalas sem tar fel szisztemas autoimmun betegseget; a diagnozis a klinikai kep es a kepalkoto vizsgalatok alapjan indikalt szovettani vizsgalattal allithato fel. A betegseg szteroidkezelesre jol reagal, de előfordul spontan regresszio is, az utobbira lattunk peldat. Bar az entitas atmenetet kepez a nekrotizalo vasculitisek es a sarcoidosis kozott, egyre tobb erv szol amellett, hogy a sarcoidosis spektrumaba tartozik. Orv Hetil. 2021; 162(38): 1541-1547. Summary. Necrotizing sarcoid granulomatosis is a rare entity currently classified as a subtype of granulomatous pulmonary angiitis. It is considered to be either a variant of sarcoidosis or a primary pulmonary angiitis. Two cases are demonstrated to present its clinical and pathological features. A 20-year-old female patient was admitted to the department of pulmonology with dry cough, right-sided chest pain during hyperventilation and fever. A 63-year-old male patient was observed with a right-sided lesion on chest X-ray after pneumonia. In both cases, autoimmune panel examination, microbiology tests, spirometry function test and bronchoscopy were unremarkable. Chest CT scans have revealed nodules with soft-tissue density without bilateral hilar lymphadenopathy. In order to clarify the diagnosis, video-assisted thoracoscopic resection (biopsy) was performed. Microscopically, parenchymal focal necrosis with adjacent to non-caseating granulomas and granulomatous angiitis were detected. In both cases, spontaneous remission occurred within a year. Histological examination - integrated with clinical data and radiological tests' results - is the gold standard form of evaluation to confirm necrotizing sarcoid granulomatosis; furthermore, exclusion of pneumonia and autoimmune diseases are also required. The disease responds well to corticosteroids; moreover, spontaneous remission is often reported, as it happened in both cases. Necrotizing sarcoid granulomatosis is a transition between necrotizing vasculitides and sarcoidosis; although more and more evidence appears supporting the fact that necrotizing sarcoid granulomatosis may belong to the spectrum of sarcoidosis. Orv Hetil. 2021; 162(38): 1541-1547.