Postgrad. med. J. (February 1968) 44, 178-194. CASE REPORTS Pulmonary aspergillosis in two families R. H. ELLIS M.D., M.R.C.P. The Gloucestershire Royal Hospital REPORTS of more than one case of pulmonary aspergillosis occurring in a family are rare. Hertzog, Smith & Goblin (1949) described a brother and sister aged 5 and 11 who died of pneumonia within a month of each other, nec- ropsy in one of them showing multiple granu- lomata from which A. fumigatus was isolated. More recently, Strelling et al. (1966) reported two fatal cases of the invasive type of aspergillo- sis in two sisters, aged 4 years and 14 months respectively. In both cases the lungs were full of nodular lesions tending to become confluent, and A. funmigatus was cultured from lungs, and tracheobronchial lymph glands, which were en- larged. Because familial incidence of the allergic eosinophilic infiltration type of aspergillosis has not previously been reported, four such cases, occurring in two families are described. Case reports Family A-Country dwellers and poultry keepers. Case 1 (Fig. 1). R.W., aged 51 years, suffered from recurrent bronchitis with sinusitis. Two years later he developed pneumonia in the left upper lobe associated with hilar adenitis, followed a year later by a fresh lesion in the right lower lobe, which failed to resolve completely. Broncho- graphy showed suppurative bronchitis rather than bronchiectasis. Two years later he suffered his third episode of pneumonia, in the right upper lobe, followed 18 months later by a fourth epi- sode, in the left upper lobe. He was investigated at Brompton Hospital, where aspergillosis of lungs and sinuses was diagnosed based on posi- tive prick skin tests, bronchial sensitivity tests, and the isolation of A. fumigatus from bronchial secretions and antral washouts, together with a blood eosinophilia. He has developed cor pul- monale. i·:··li:li :;·:: i"iS.i.I::·..· ··::·II iiii $lii:i:::·::··:··. iidililr:::::. L%i'"'.aa. 1IBlll[t.E.E.s.p.piti.i.3...e.e.i IE.'Ii Ri..BIIB....P:iPYiPiB Hiiii.ifii!:i;liil: j.iiIigijli8i4i.idiitiiil:pl alai.ii.nii.ij.ii.iitii.iliii is.aii.:ldcaas ETi xa Iili.i85il.Liii liZiZ:H:: :1 liiiiSiBiliiii.i3iiil%i:iil.l;iili8iib, 1:::::::·:;:9::··:··:··::;::i::I:3::...' ·I::::::::i:·::·:'·:.·::;:::::i::;:i?ii8 ICi:i:8iiiiZ:ii:ii%:l;.isii·iii·i i%'''.aiiii.aiiiiili'·il..iE5;8:ii': i.li:l:iiiliiiliI:iIi5iZ.iiiiiriZf ila.ieii!il!iilii.iiii$Ri8iiiiliHii. .iieil!il;iCEiiiiI!iliiiQiiaiiiiii ::·.:::'::'::'i.'i:' :::::·l:·':·':i::i·:igi8iI:f:l liiiii.liliai4i::i:':: gii.iiiii3.iEiiiii!lil:l:'i.ii::i.:idi 91. .......... ..;... .......:...:::·:.·i.:·.·;:·.:·::··.··I:· 8:·:·:w·lli:'i:z:iiiiil: li;i: i:·ii:·i:.i::i:'aki:li:I:lii'iii::irili :··· :··:·:··: ·:··::·::·:·:::;I::"9 a:n·;:i;::;··.:· .·..:..·. ·.·:: i.liliriiiiii:ii:iIii':i.ii':::·:··::·:: :::·· :' ::.:.:·i:: iiiil .i-:....s·:I::r::i:·:I:::·I:··:···:···· ·'·: :·: :': '· :· '·":":';:';::·:: .%i.i.;il:il;i:ii:;i:Zil:·::.I: il:::::·:'l:;i: ::·'I::i: i': i.i'::::l:;ii5j.i:ii::i:'iil:l:·:::::· ?:i:ji::i:::::.·:::::':":":":'·:' ···i· :·.·:·:: .. ·. ·:· .··:··:··I:·; "i'· ··'·: ·:::i·::I::I:li8i58 ........ :·..:;.·:::::· ,I::...:.::.·..:;.·..·..::::::·.·.·..·:.· ·.··.:·:··i6i. Llli··i·'i:ii·: ·ii::.::r::: :.. '· ·...:.··.·;::·:·:I iiiihii:i:::i:::::il:::il::':·":: D;1:a::1·:1··1··.:'··: :1··1::1:·::·:: :··'· ··: ··'··':I·i::·.:·.::il.:'::li·:::::l:II.: :· ··:l·::·:i;::·i· :.·.·.·: ·::·:·········:···::'···:·:···'''''·':''·' ...::I··I;······ :··.· ...i... :: Lli.i:lii::i::i: l·. ·..·. is ····· ·'·'·i·::·::.·:i';'';;' I.i$i.g::·i'''·'·' :':·:·::·'·'·.i··::·I:':':: ····:··i·li*i:. ::.:::i::l· ·:·i·:··:::':i:i··i ···::::·::·::.· ks·.ls··I :·:·::·,:·ii·.wail :'·i··:·:':I·:::·:·'·:::::.:.:':: :··:;;.;n; :·i··:··:i.:i·:::i ·····:··'::''": i:·:·:.·; ·'·'·· ·'·::·::;::::::.':I::r::: :':'' FIG. 1. Family A, case 1. Tuberculin negative. Infiltra- tion in left upper lobe with well-marked left hilar adenitis. Case 2 (Fig. 2). M.W., aged 8 years, had pneu- monia in the right upper lobe, which cleared, but was followed by fresh infiltration in the left upper lobe, and left lower lobe, at intervals of 2 and 4 years. Tomography after the last illness showed distended bronchi in the right upper lobe, and bronchoscopy an inflamed right upper lobe orifice. He was transferred to Brompton Hos- pital with his brother, where aspergillosis of lungs was confirmed by positive prick skin tests, bron- by copyright. on May 5, 2021 by guest. Protected http://pmj.bmj.com/ Postgrad Med J: first published as 10.1136/pgmj.44.508.178 on 1 February 1968. Downloaded from
Transcript
Postgrad. med. J. (February 1968) 44, 178-194.
CASE REPORTS
Pulmonary aspergillosis in two families
R. H. ELLISM.D., M.R.C.P.
The Gloucestershire Royal Hospital
REPORTS of more than one case of pulmonaryaspergillosis occurring in a family are rare.
Hertzog, Smith & Goblin (1949) described abrother and sister aged 5 and 11 who died ofpneumonia within a month of each other, nec-ropsy in one of them showing multiple granu-lomata from which A. fumigatus was isolated.More recently, Strelling et al. (1966) reported
two fatal cases of the invasive type of aspergillo-sis in two sisters, aged 4 years and 14 monthsrespectively. In both cases the lungs were fullof nodular lesions tending to become confluent,and A. funmigatus was cultured from lungs, andtracheobronchial lymph glands, which were en-larged.
Because familial incidence of the allergiceosinophilic infiltration type of aspergillosis hasnot previously been reported, four such cases,occurring in two families are described.
Case reports
Family A-Country dwellers and poultry keepers.Case 1 (Fig. 1). R.W., aged 51 years, suffered
from recurrent bronchitis with sinusitis. Two yearslater he developed pneumonia in the left upperlobe associated with hilar adenitis, followed ayear later by a fresh lesion in the right lowerlobe, which failed to resolve completely. Broncho-graphy showed suppurative bronchitis rather thanbronchiectasis. Two years later he suffered histhird episode of pneumonia, in the right upperlobe, followed 18 months later by a fourth epi-sode, in the left upper lobe. He was investigatedat Brompton Hospital, where aspergillosis oflungs and sinuses was diagnosed based on posi-tive prick skin tests, bronchial sensitivity tests,and the isolation of A. fumigatus from bronchialsecretions and antral washouts, together with ablood eosinophilia. He has developed cor pul-monale.
FIG. 1. Family A, case 1. Tuberculin negative. Infiltra-tion in left upper lobe with well-marked left hilaradenitis.
Case 2 (Fig. 2). M.W., aged 8 years, had pneu-monia in the right upper lobe, which cleared,but was followed by fresh infiltration in the leftupper lobe, and left lower lobe, at intervals of2 and 4 years. Tomography after the last illnessshowed distended bronchi in the right upper lobe,and bronchoscopy an inflamed right upper lobeorifice. He was transferred to Brompton Hos-pital with his brother, where aspergillosis of lungswas confirmed by positive prick skin tests, bron-
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FIG. 2. Family A, case 2. Right upper tomogram show-ing pus-filled segmental bronchi in right upper lobe.
chial sensitivity tests and the isolation of A.fumigatus from bronchial secretions. Purulentsinusitis was also found, but A. fumigatus wasnot cultured from antral washouts. He died ofcor pulmonale a year later.
Family B-Farmers.Case 3 (Fig. 3). H.E., farmer's wife, aged 60
years, had pneumonia in the right lower lobewith a blood eosinophilia which cleared rapidly.Eight years later she became suddenly breathlesswith thick sputum, and chest X-ray showed totalcollapse of the left lung. Aspergillosis was con-firmed by positive prick skin test, the presence ofserum precipitins and the isolation of A. fumi-gatus from bronchial aspirate, with a bloodeosinophilia. Eventual re-expansion of the lungwas achieved by bronchoscopic suction and in-tensive postural drainage and percussion. Shehas remained well since moving away from thefarm.
Case 4 (Fig. 4). E.E., farmer's son, aged 35years. Experienced breathlessness and chest tight-ness without wheeze. X-ray showed pneumonia
in left lower lobe, with blood eosinophilia, whichhad not completely cleared 4 months later whenhe developed fresh infiltration in the right dorsallobe. Aspergillosis was confirmed by positiveprick skin test, the finding of serum precipitins,and isolation of A. fumigatus from sputum. Hehas remained well since wearing a mask duringthreshing and grinding operations.
DiscussionThese cases show that when allergic asper-
gillosis occurs in more than one member of afamily, all the main radiological features mayoccur, including transient pulmonary exudations,pus-filled bronchi, and atelectasis of segments orof the whole lung.Although allergic aspergillosis is more com-
monly associated with asthma, there was no suchhistory in these families.The two boys in the first family (cases 1 and
2) both developed staphylococcal sinusitis neces-sitating repeated antral wash-outs from whichA. fumigatus was also cultured in one of them.It is known that the upper respiratory tract maybe the site of aspergillosis, as described by Mon-treuil (1955), Savetsky & Waltner (1961), Hora(1965) and others, but in none of their caseswere the lungs affected.
In case 1 of this series, I believe that thesinuses were secondarily infected from thebronchi, and that they should always be screenedfor aspergillosis in such cases.The presence of well-marked hilar adenitis in
one tuberculin-negative boy, during episodes ofpulmonary infiltration, is interesting, and is notseen in adult aspergillosis. Neither has it beendescribed as part of the radiological picture inchildren, though it concurs with the necropsyfindings of Strelling et al. (1966).
Neither boy had true bronchiectasis, thoughboth showed the typical bronchographic appear-ance of suppurative bronchitis. It is likely thatthey re-infected one another, and the asper-gillosis may have originated in the poultry meal.
Since the death of case 2 from cor pulmonalea year after aspergillosis was diagnosed, hisbrother has had no further infiltrations, but haslatterly developed cor pulmonale himself.
I conclude that the aspergillosis in these two
boys was a late event, secondarily imposed uponlong-standing upper and lower respiratory in-fection.The farming family show a different picture.
The mother (case 3), developed the unusual andserious complication of total collapse of thelung after previous infiltrations, and has beenreported in an earlier paper (Ellis, 1965). Nor-mally fit and well, both mother and son becameill following exposure to corn dust during thresh-ing and grinding which was probably the sourceof infection. This impression is unsupported bythe isolation of fungus from samples from thefarm, or by high spore counts inside the build-ings, but both have remained well since takingprecautions against dust exposure.The fact that other unrelated farmworkers were
equally exposed without developing aspergillosis,raises the question of hereditary predispositionto the disease in this family. This would be ren-dered more likely should the farmer himselfreveal evidence of aspergillosis during one of hisregular exacerbations of bronchitis.The demonstration of serum precipitins against
mouldy-hay antigens in the daughter-in-law anda cowman on this farm without symptoms andsigns of farmer's lung, illustrates the close asso-ciation between this disease and aspergillosis, andindicates that serological surveys in farming com-munities might yield information of epidemio-logical interest with regard to these two diseases.
AcknowledgmentI am grateful to Dr R. W. Riddell of the Brompton
Hospital, in whose department the precipitation tests weredone, for his advice, and encouragement to publish thesecases.
ReferencesELLIS, R.H. (1965) Total collapse of the lung in aspergillosis.
Thorax, 20, 119.HERTZOG, A.J., SMITH, T.S. & GOBLIN, M. (1949) Acutepulmonary aspergillosis. Report of a case. Paediatrics, 4,331.
HORA, J.F. (1965) Primary aspergillosis of the paranasalsinuses and associated areas. Laryngoscope, 75, 768.
MONTREUIL, F. (1955) Fungus infections of the antrum.J. Laryngol. Otol. 69, 559.
SAVETSKY, L. & WALTNER, S. (1961) Aspergillosis of themaxillary antrum. Arch. Otolaryngol. 74, 695.
STRELLING, M.K., RHANEY, K., SIMMONS, D.A.R. &THOMSON, J. (1966) Fatal acute pulmonary aspergillosisin two children of one family. Arch. Dis. Childh. 41, 34.
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