Management of recurrent lung infections in a case of … · 2019. 1. 6. · CASE REPORT Management...

CASE REPORT

Management of recurrent lung infections in a case of

hyperimmunoglobulinemia E (or Job's) syndrome

M ARSENAULT MD, V ECHAVE MD FRCSC, C F ISH MD FRCPC, J-P PRAUD MD PhD

Departments of Pediatrics, Surgery and Radiology, Centre hospitalier universitaire de Sherbrooke, Faculty of Medicine, Universite de Sherbrooke, Sherbrooke, Quebec

M ARSENAULT, V ECHAVE, C FISll,J-P PH.AUD. Management of recurrent lung infections in a case of hyperimmunoglobulinemia E (or Job's) syndrome. Can Respir .J 1995;2(1 ):77-80.

The diagnosis of hyperimmunoglobulinemia E (hyper lgE) or Job· s syndrome was made in a li ve-month-old girl with chronic staphylococcal mastitis. elevated serum IgE and abnormal neutrophil chemotaxis. Arte r mult ipl e hospitalizations for severe- skin in fcc tions. ri ght upper lobe bullae were found and treated by lobectomy when the patient was three years of age. Thereafter, the pat ient was repeatedly hospi tal ized for pneumonia while on cloxaci llin prophylaxis and rece iv ing regu lar chest physiotherapy. When she was 12 years old. pulmonary detcriorat ion (increased frequency or pulmonary in l'ct.: tions , hemoptysis. rad iological destructi on or the ri ght middle lobe) led 10 a righ t middle lobectomy. Si nce thi s intervent ion. the pat ient has had an improved quality nf life , takes part in regular sports activities. wit hout recurrence of severe pulmonary in fections. and has had ne,u- normal pulmon;uy funclion studies. A concerted mcdicosurgic,il therapeutic regi men can control severe pulmon.:uy cumplicatinns in patients with this rare syndrome.

Key Words: l J.11wri11111111110gloh 11/i11 ,'111i11 L srndnmu·. Joh·.,· .H11-

dru111e. L1111g uh.H't'SS

Prise en charge des infections pulmonaires recurrentes dans un cas d'hyperimmunoglobulinemie E (syndrome de Job)

RESUME : Le diagnostic d'hypcrimmunoglobulinemie E (hyper lgE) ou syndrome de Job a ele pose chez une fill ettc de 5 mois prcsen tant unc mas tile staphy lococcique chroni que. un taux e leve d' lgE ser iqu es ct un ch imiotacti smc des neut rophiles anormal. Apres de nombreuses hospital isati ons pour des infect ions cutanees graves. une bu llc a ete decelee dans le lobe supcrieur droi l et rraitee par lobectomie a !'age de trois ans. Par la suite. la patiente a etc hospitali sec plusicurs fois pour des pneu monies malgre une prophylax ie i1 la cloxac illine ct une physiotherapie pulmonaire reguliere. A r age de 12 ans. une deterioration pulmonai re (frequence accrue des infections pulmonaires, hemoplysies, signes radiologiques d'une destruction du lobe moyen ) a neccssitc une lobcctomie du lobe moycn. Depuis cc tte in tervention. la pat icnlc a vu sa quali te de vie s·ameliorer et parlicipe ~. des activites sporlives regulicres sans nouveaux episodes d'infections pulmonaires graves. Ses epreuve. de fonction pulmonaire son! proches cle la normalc. Un regi me thcrapeutique medicoch irurgical concene peut enrayer lcs L'Omp liL·ations pulmona ires graves cl1e1, les patients souffra nt de ce ra re syndrome.

Currespondence and repri11ts: /Jr Jca11 -P1111/ J'm11d. Departe111e111 d,- l'fdiatn,-. Farnlre de Medecine. U11~1·ersir,: de Sf,,,,-hrook,-.. WO/. North / 2th A ,·e11 11e. Sherbrooke. (}11 fhcc .1111 5N.J. Tcl, ·11hn11e (81 '/) 5/i.i-5555. e.rr ./851. Fax (8 /9) 564-52 t .,

Can Respir J Vol 2 No 1 Spring 1995 77

ARSENAULT ET AL

T l IE HYPERIMMUNOGLOB LINEM IA E (HYPER. !GE) OR.

Job ·s syndrome is a rare disease with e levated IgE and a Jefect in immu ne function ( l ,2) res ulting in repeated s inopu lrnonary tract infections in childhood which eventually

lead to serious pulmonary J estruction and respiratory compromise (3.4). Th is report describes a patient wi th hyper IgE syndrome and severe pul monary complications who now has acceptable pulmonary func tion and enjoys a good quality of Jile because of a complete medicosurgical therapeutic regimen including tailored pulmonary surgery, physiotherapy and bronchodi l::itor the rapy.

CASE REPORT T he five-month-old patient was diagnosed wit h hyper IgE

syndrome when she presented to the pediatric clinic with a history of chronic right breast discharge s ince birth. chronic seborrheic J ermatitis. perforated otitis media and le ft cervical aden itis. Family history was pos it ive for a maternal uncle with repeated cutaneous abscesses, pneumonia and multiple bouts of otitis media as a child. two maternal aunts with ch ildhood cutaneous abscesses. and several other maternal relati ves w ith chronic severe pustular lesions of the face and scalp. Physical examination revealed an active well looking afebrilc child wi th infected facial eczema. normal c hest,

cardiovascular and abdominal examination. le ft posterior cervical adenitis and a right breast abscess. Laboratory workup revea led an increaseJ scrum IgE level ( 113 U/mL. which later increased to 15,000 U/mL) and decreased chemo

taxis (assessed by the Boyden chamber technique 151). Leuko-LJ .

cyte count was 21.000x IO /L with 8'7r polymorphonuclear cells, 88'7' lymphocytes, l 7a monocytes and 2% eosinophils. Culture o r scalp. breast J ischarge and the cervical abscess were positi ve for Stap/irf oco< ·cus rwreus. Review of he r chart showed a positive S auffus culture of the umbilicus at two

days of age. By the age of three years. the patient had had three hospi

talizat ions, the first for right leg and perianal abscesses. the

second for conjunctivitis and an infraorbital abscess. and the thi rd for severe puru lent conjunctivi tis. All were cultureposit ive for S 011re11s. The episodes o,·curred while the patient was on oral clox::icillin prophylaxis and she responded well

to antistaphylococcal intravenous antibiotics. A lso, at 14 months o ld, she was fou nd to have congenital d islocati(lll uf the hip and later an associated genu valgum. which required three hospitalizations for surg i al correct ion.

At the age o f three years the patient began to develop pulmonary complications. W hile she was hospitaliLcd for a severe campylobacter gastroente ritis. routine rocntgenograms showed large bullac occupying the enti re right lung field. The patient was asymptomat ic and had normal blood gases. Perfusion lung scan showed almost complete loss of

perfusion to the ent ire right lung but normal left lung perfus ion. After four weeks of cloxacil lin the pat ient had slight dyspnea on effort. and repeat chest roentgenograms showed bul lae originating in the right upper lobe and compressing the right middle and lower lobes (Fi0 urc I). Two months later the patient underwent ;1 right superinr lobectomy, which was

78

0

Figure I) Posteroo111erior (top) w1d fureru/ (bottom) ch<'.1·1 mdiogru17/1.1· obtained al rhe age of 1/1ree yeurs de111011srrnre sei·ern/ lcirge p11eu111arocefes i11l'Oli·i11g rile e111ire rig/ti 11/'f'l'i" lobe a11d co111pressi11g 1!1e udjuce111 middle and fm,·er fohe.1

Can Respir J Vol 2 No 1 Spring 1995

Fi1,:urc 2) /'11.,1,.,-u,1111,·1111r d1cs1 w diog w1,l1 (top ) u11d co11111111ed 1u111t1gm 11!11· ' <'cl irHI 1/11m1gh rhl' c11 ri11a (bottom) nhtained 111 J/1('

age ()( 12 _1H1n· s/11111. lwsidc.1· righ1 111iper /ohec10111_,· changes. con w/itlut i()II mu/ 1·11 /111nc /11.1.1· of the reoriell!ed middle /ohe co11 -t<1 i11i11g 11111/J i11l,· 11ir)illt'tl, ,n·iti<'s. some o/ 1rhich repre.\'/:'11/ hro11 -c/1i,·,·tusi1

wl'll tokratl'd with a11 u11L' n ·ntf'u l postoperative course . At the time or "urgery the 111id<l le and lower lobes were noted to be normal. Pa tho logy examination showed multiple bullae wi th in chron ically inll amed pu lmonary ti ssue.

Over the ,uhsequen t eight years, the patient was hospital i, ed thrl'e to fou r times per year for pneumoccoccal 1)r


Pulmonary complications in hyper lgE syndrome

Figure 3) Posterow !lerior chcst radiugraph (top ) 011d cu111p11ted to111 ograp/1y section thro11gh th,· rnrina (bottom) ohwined ur thl' age of 14 yeurs sho11 · a 110r1110/ 11ostlubecto1m· chest appea1w1n·

Hue111nphiius i11/l11e11;.ae pneumon ia. loca li zed to the superi or portion of the right lung, whi le on oral cloxaei llin prophylaxis . The pati ent began to experience symptoms of chronic cough and di minished exerci se tolerance, and at 12 years or age she had occasional episoJes or mild hernoptysis.

While the patient was hospita li zed for another hout of pneumonia, chest roentgenogram and computerized 101110-

graphic (CT) scan demonst rated multiple air-filled cavi ties surrounded hy pulmonary opacit ies occupy ing the upper third of the right lung fi eld (Figure 2). Bronchoscopy showed

79

ARSENAULT ET AL

unremarkable scar tissue al the site of surgery, SO'?i stenosis of the right middle lobe bronchus, and abundant white secretions in the right and left main bronchi . Bronchial lavage rnlture was positi ve for S a11rcus and H i1ifluc11:::ae. Bronchography rcveall"d nu merous cavities unpenctrated by contrast medium, distortion of right midd le bronchus and no nidencc of bronchopleural fi stula. Pulmonary function studic., suggested a mild obstructive pattern with a forced expiratory volume in I s (FEY 1) of 7 1 % and a forced vital capacity (FVC) of 90% of predicted values. The patient responded well to intravenous antibiotic therapy, but over the next year had further frequent hospit~1 lizations for right upper lobe pne umonia; a repeat CT scan showed persistence of the cavities as well as atelectasis of the right middle lobe with occasional areas of bronchiectasis. A second perfusion lung scan showed diminished perfus ion of the upper right lung. Based on the laboratory evidence of damaged middle lobe parcnchyma and the patient' s c linical deterioration over oneand-a-half years, the patient un<lcrwcnt a right middle lobcctomy at the age o f 13. from which she made a rapid recovery with an unevent ful postoperative course.

In the foll owing two years. the patient has had :in improved quality of life, with normal daily activities including sports. and near normal pulmonary function studies showing normal lung volumes (greater than lJOCfo of predic ted values) anJ arterial blood gases, a mild obstructi ve spiromctry pattern (FEY 1 66<ii and FYC 83% or prc<licted values with a rise to FEY 1 76% and FYC 89% after inhalation of a beta2-agonist). She has also had only one three-day hospitalization for mi ld pneumonia during this time. She is still on oral cloxacillin prophylaxis. long term regular inhaled beta2-agonisl therapy and has daily chest physiotherapy. which h:1s helped to prevent any further complicat ions.

T he chest radiograph and CT scan obtained at the age or 14 showed a normal post lobectomy appearance (Figure 3 ).

DISCUSSION Job's syndrome. first reported by Davis cl al in 1966 ( l ).

Jerives its name fro m the biblical figure Job, who was inflicted with ' 'boils from the so le of his foot unto his crown'" (JOB ?.:7) by Satan. In 1972 Buckley e t a l (2) made a major advance in de lineating the syndrome. The disease is characterized by: repeated cutaneous, gang lionic. si nus and pulmonary in fec tions usually of staphyloccoca l, st reptococcal or If i11fl11e11;:,ae origin: cczcmatoicl dermatitis that is usua lly supcri nfccted: facial dysmorphism and coarse faci cs; retarded growth; and, rare ly, limb deformity (6). The syndrome shows characteristic hyper IgE. many hundred ti mes above normal, a mild chcrnotactic defect and eosinophilia (7,8 ).

Pulmonary involvement is usually important and can compromise the patient ' s pulmonary functi on to the point of severely reducing the quality of li fe and finally leading to death. Aggressive early antibiotic therapy together with chest physiotherapy must be instituted in order to keep pul monary damage to a mini mum. As seen in our patient, repeated pulmonary infections may lead lo lung destruction with bulla formation and bronchiectasis. A full pulmonary workup in-

80

eluding bronchoscopy with bacterial cultures. CT. ventilation scan and pulmonary funct ion studies is indicated in these patients whe n involvement is severe, in order lo optimize medico urgical therapy.

There are few treatment modalities that have proved to be bene ficial for patients with hyper lgE syndrome (7,8 ). Until the underlying pathogenesi s is more fully understood. a complete pulmonary therapeutic regime n including di.cussion of early tailored surgery is warranted for these patients. Anecdotal expe rience drnwn from our paticn1 and other previous case reports (3,4) suggests that removal o f the damaged lung parenchyma can reduce morbidity and improve pulmo nary function sign ificantly when conservative measures fail to control repeated respiratory infect ions. The two surgical procedures performed in our patient arc in accordance with pre vious reports in hyper lgE syndrome. Pneumatoccles have been reported to be frequent in hyper lgE syndrome patients; usual pcrsistance with secondary in fection is common (9). Also, current practice guide lines in thoracic surgery advise a lobectomy for localized bronchiectasis in the presence of symptomatic and recurrent pneumonia refractory to medica l treatment. recurrent he mophysis and adequate predicted postoperative lung function ( I 0). Surgery combined with an aggressive medical reg imen with frequent follow-up visits reduce hospitalizations, optimize pulmonary function, i111-prove the quality of life and allow a child with a chronic debilitat ing illness to live normally.

ACKNOWLEDGEMENTS: The authors thank Caruh.: Jacques l'or secretaria l as istance in the preparation o r the manuscript anJ Dr Marek Rola-Plcsl clynski for helpful cri tici. m.

REFERENCES I . Davis SD. Schaller S. WcJgcwooJ RJ. Job's syndro11w:

recun-ent ·cold' staphylococcal abscesses. Lancet 1966:i: IO 13-5 .

1 Buckley RH. W ray BB. Bclmakcr EZ. Extreme hyperimmunoglobulinaem ia E am.l undue susceptibility to in fect ion. Pediat rics 1972:49:59-70.

3. Lui RC. lnculct RI. fob 's synurome: a rare cau$,' of rcn,rr,·nt lung abscess in ch iluhood. /\nn Thorae Surg I 99():.'.)():992-4.

4. L' Huillier J-P. Th rcux P-H. Oelaval P, c t al. The hypcrimmunoglobulinacmia E and rccurrcnt ink:ct il1ns synJrome in an adult. Thorax 1990:45:707-8 .

.'.) , BoyJcn S . The chcmotactic effect o r mixtures of ,llltibody and antigen on p,1lymorphonuclcar leukocytes. J Exp Med 1962: 11 5:453-66.

h . Lebranchu Y. BlancharJ P, Lorette G. cl al. Hypcrimmunoglnbul in E syndrome with rect11Tcnt infections: associatio n with gcnu varum. Ann Pcdiatr 1986:33: I 09- 12.

7. Donabcdian H. G all in JI. The hyperi1111111111og lobulin E recurrent- infec tion (Job's) synd rome: a review of the NII I cxpcrience and the literaturc. Medic ine 1983:62: 195-208.

8. T rung PH, Oury C, Daumli ng S. Buriot D, BelohraJsky BH Syndrome de su$cCptihilitc aux in fec tions avcc hyper IgE. A propos de 19 nouvellcs observat ions. Arch Fr Pcdiatr I 982;39:353-8.

<) Shambcrger RC. Wohl ME, Pcrez-AtayJe ;\, Hendren W E. Pncumatocclc eomplicating hypcrimmunoglohulin E syndro111c (Job's synuromc). Ann Thorac Surg 1992:54: 1206-8.

l ll. Kaiser GC. Practice guiJe lincs in cardio1horaci,· ~urgcry. A11n Thorac S urg 1993:56: 1203- 1.1.


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