Langvinn eósínófíl lungnabólga á Íslandi : faraldsfræði, klínísk einkenni og yfirlit

Abstract

OBJECTIVE: The objective of the study was to describe the incidence and clinical features of chronic eosinophilic pneumonia (CEP) in Iceland and review recent literature. MATERIAL AND METHODS: Retrospective study where information was obtained from clinical charts from 1990-2004. Records, imaging studies and histopathology were evaluated. RESULTS: During the study period 10 individuals were diagnosed with CEP, 7 males and 3 females. Mean age was 58 years. None of the patients was a current smoker. The incidence of CEP during the study period was 0.23 per 100,000/year but increased to 0.54 per 100,000/year during the last 5 years of the study period. Clinical symptoms were malaise, cough, dyspnea, sweating and weight loss. Sedimentation rate was 72 mm/h and C-reactive protein (CRP) 125 mg/L. Eight of the ten patients had increase in blood eosinophils. On chest auscultation crackles were heard in seven patients and wheezing in three. Forced vital capacity (FVC) was 75% of predicted value and forced expiratory volume in one second (FEV1) was 73% of predicted. Mean PO2 was 68 mmHg. All the patients had classic diffuse bilateral opacities on chest radiograph that most commonly were peripheral. All patients were treated with corticosteroids and responded well. The average initial dose of Prednisolone was 42.5 mg per day. Seven of the patients relapsed but they all responded well to repeated treatment. CONCLUSIONS: Chronic eosinophilic pneumonia is a rare disorder but it has specific radiologic and histologic features. It is important to think of the disease in patients with diffuse infiltrates that are resistant to antibiotics. CEP responds well to corticosteroids but there is a high relapse rate, which also responds to treatment.

Inngangur: Tilgangur rannsóknarinnar var að kanna nýgengi langvinnrar eósínófíl lungnabólgu á Íslandi, lýsa klínískum einkennum og veita yfirlit yfir sjúkdóminn. Efniviður og aðferðir: Aftursæ rannsókn þar sem upplýsingar voru fengnar úr sjúkraskrám árin 1990-2004. Sjúkraskrár voru yfirfarnar, kannaðar voru myndgreiningarrannsóknir og vefjafræðilegar greiningar endurskoðaðar. Niðurstöður: Á tímabilinu greindust 10 manns, sjö karlar og þrjár konur. Meðalaldur var 58 ár. Enginn sjúklinganna reykti. Nýgengi sjúkdómsins á öllu tímabilinu var 0,23 á 100.000/ári en jókst síðustu fimm árin í 0,54 á 100.000/ári. Einkenni voru slappleiki, þreyta, hósti, mæði, og megrun. Sökk var 72 mm/klst og C-reactive protein (CRP) 125 mg/L. Átta af 10 voru með aukningu eósínófíla í blóði. Við lungnahlustun heyrðist brak hjá sjö en önghljóð hjá þremur. Forced vital capacity (FVC) var 75% af áætluðu gildi og forced expiratory volume in one second (FEV1) var 73% af áætluðu gildi. Meðalhlutþrýstingur súrefnis (pO2) var 68 mmHg. Sjúklingarnir höfðu allir dæmigerðar dreifðar íferðir beggja vegna sem voru yfirleitt útlægar. Allir fengu sterameðferð og svöruðu meðferðinni fljótt og vel. Meðaltalsskammtur af prednisólon í upphafi meðferðar var 42,5 mg. Hjá sjö kom sjúkdómurinn aftur en allir svöruðu endurtekinni sterameðferð. Ályktanir: Langvinn eósínófíl lungnabólga er sjaldgæfur sjúkdómur en þó með einkennandi myndrænt og vefjafræðilegt útlit. Mikilvægt er að hafa hann í huga hjá sjúklingum með dreifðar íferðir sem svara ekki hefðbundinni sýklalyfjameðferð. Sjúkdómurinn svarar vel meðferð með barksterum sem getur þó þurft að endurtaka.