Bronchopulmonary Bacillary Angiomatosis

TCI-induced lung disease may vary from a relatively mild and completely reversible interstitial lung disease (case 1) to a severe form of bronchiolitis obliterans (BO) causing prolonged and complicated respiratory failure and leaving the patient severely disabled (case 2). This varied clinical course is most probably related primarily to the concentration of inspired toxic fume and to the length of the exposure period." Bronchiolitis obliterans is an uncommon disease characterized pathologically by an obstruction of the airway lumen beyond the terminal bronchioles by a cellular infiltrate or fibrosis and is associated with varied etiologic factors.>" Toxic-fume inhalation is a well-recognized cause of BO, and reports of SO. or CHI-related BO have been published.....However, the development of BO following TCI exposure has not been reported previously, to our knowledge, although Ducatman et ai' describe one patient who died of fulminant pulmonary edema after exposure to TCI. The triphasic evolution of the BO noted in case 2 is typical of toxic fume inhalation in both experimental and clinical settings." " Our patient was almost asymptomatic immediately after the injury, although some patients can present with pulmonary edema. The acute phase was followed by a latent, clinically asymptomatic phase of over two weeks followed by a full-blown acute respiratory failure. The finding of wheeztng unresponsive to bronchodilators is another feature of bronchospasm induced by fume inhalation.... An initially normal chest radiograph can be encountered (as in this case)" and may be misleading since acute respiratory failure may still develop following a latent period . Subsequently, the patients hyperinflation of the lung is frequently seen in BO, and the lung function tests (showing a combined obstructive + restrictive pattern) and ABG values (showing mild hypoxia and hypocarbia before the complications described above) are also typical ." Another common finding in fume-induced BO is the very rapid evolution from the acute phase to chronic lung disease: and our patient had the same evolution. Bronchiectasis is a wellrecognized complication of SO. inhalation;' however, to our knowledge, the appearance of spontaneous pneumothorax, lung bullae, and BPF have not been reported previously in fume-induced BO. The prognosis of fume-induced BO is generally poor: although in some cases, lung function may slowly improve after two to three years. 12 The first of our patients apparently responded well to steroid therapy, but our second patient developed severe BO and remains a respiratory cripple although he was immediately treated with steroids to minimize lung damage. Generally, steroids are less useful in fume-induced BO (except for N02 inhalation) and steroid therapy should possibly be stopped if no improvement is seen during the first days because this treatment may increase the risk of lung infection in the presence of a denuded lung epithelium. 13-" In conclusion, our two cases demonstrate the spectrum of TCI-induced lung injury ranging from a relatively mild and reversible interstitial disease to progressive severe respiratory failure . To our knowledge, it is also the first report of BO induced by TCI, an occupational hazard that should be better recognized in the future .

REFERENCES 1 Ducatman AM, Ducatman BS, Barnes ]A . Lithium battery hazards: old-fashioned planning implications of new technology. J Occup Med 1988; 30 :309-11 2 Ezri T, Konichezky S, Schattner A, Eliraz A, Halperin D, Sorokez D . Bronchiolitis obliterans-a review. Submitted 3 Epler GR , Colby Tv. The spectrum of bronchiolitis obliterans [editorial). Chest 1983; 83 :161-62 4 Gosink BB, Fridman PJ, Liehow AA. Bronchiolitis obliteransroentgenological-pathological correlation. AJR 1973; 117:816-32 5 McLoud Te, Epler GR, Colby Tv, Gaensler EA, Carrington CB . Bronchiolitis obliterans. Radiology 1986; 159:1-8 6 Charan NB, Myers CG, Lakshminarayan S, Spencer FM . Pulmonary injuries associated with acute sulfur dioxide inhalation . Am Rev Respir Dis 1979; 119:555-60 7 Byatt CM . Long-term effects of exposure to SO•. Am Rev Respir Dis 1983; 128:890-93 8 Chester EH, Kamal PJ, Payne CB, et aI. Pulmonary injury following exposure to chlorine gas: possible beneficial effects of steroid treatment. Chest 1977; 72:247-50 9 Vai F, Fournier MF, Lafuma JC, Touaty I, Pariente R. SO. induced bronchopathy in the rat : abnormal permeability of the bronchial epithelium in vivo and in vitro after anatomic recovery. Am Rev Respir Dis 1980; 121:851-58 10 Horvath Ep, do Pico GA, Barbee RA, et aI. Nitrogen oxide induced pulmonary disease: five new cases and a review of the literature. J Occup Med 1978; 20 :103-10 11 Kizer KW Toxic inhalations. Emerg Med Clin North Am 1984; 2:649-66 12 Bordow RA, Moser KM. Manual of clinical problems in pulmonary medicine. 4th ed. Boston : Little-Brown, 1988; 322-33 13 Zikria BA, Budd DC, Floch F, Ferrer JM . What is clinical smoke poisoning? Ann Surg 1975; 181:151-56 14 Seggev JS, Mason UG III, Worthen S, Stanford RE , Fernandez E . Bronchiolitis obliterans - report of three cases with detailed physiologic studies. Chest 1983; 83:169-74 15 Skornik WA, Dressler DP. The effects of short-term steroid therapy on lung bacterial clearance and survival in rats. Ann Surg 1974; 179:415-21

Bronchopulmonary Bacillary Angiomatosis· Michael A. Foltzer; M.D.; William B. Guiney, Jr., M.D.; Gilbert C . "bger; M.D.; and Harlan D. Alpern, M.D.

A man with prior AIDS developed acute febrile interstitial pneumonitis, hilar and paratracheal adenopathy, and bronchial polyps. The polyps were histologically typical for bacillary angiomatosis and complete symptomatic and radiographic response to oral c1arithromycin was seen. The clinical presentation of bacillary angiomatosis includes pulmonary disease and in particular bronchial polyps; c1arithromycin is an effective oral antibiotic. (Chat 1993; 104:973-75)

I

BA

=bacillary angiomatosis

I

·From the Departments of Medicine and of Pathology, Mary Imogene Bassett Hospital, Cooperstown, NY, and Columbia University College of Physicians and Surgeons, New York. CHEST I 104 13 I SEPTEMBER. 1993

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S

ince tlH' first description in 19/;:3 of a novel subcutaneous infeetion in an AIDS patie-nt. tlu- clinical spectrum of diseasr- produced hy till' rickl'ttsial agf'nt of haeillary angiomatosis (RA). Rochal imae« lienselae , continue-s to e-xpand. Recent re-ports in immunocompromised patients include subcutaneous masses with contiguous erosiv« hone lesions.' Iwliosis lu-patis .' dissemination with visce-ral ahscf'ss formation and hone marrow infiltration , I intracr-re brnl diseas(' , ' and fphrile haetprt'lllia :' In thp immunocompetent patient. only fehrile haetprt'mia is descrilwd ." A patie-nt with AIDS had interstitia] lung disease, con comitant hilar adenopathy. and unusual hronchial polyps histologically demonstrating har-illary angiomatosis : 11(' responded to clurithromvcin . CASE REPORT

A -t2-\'f·ar -old . 11IIman immm1l"I,·fid..",·y virus (1IIV )-posili\".- . male homos--vnal with prior P"'-lIm".."st;s cllri"ii pm-umonia and e-xtensiv.. long-standing r-utam-ou« Kaposi 's surcotna. with an ahsoInll' ( :n-t count of Ii cells p.. r cuhi« millime-te-r, re-port .. d a I-monlh hislory of 1',.\"••1' a('('ompani..d hy r..('nrrt'nl shaking ('hills . p....fonnd nodnrnal sw,·aling. i-kg w"ighl loss. inlt'n,,·. nonpn"ln<'li\".. ('ongh lhal ,,,'('asional'" indn(·..d ,·m, ·sis. and posilional right-si(h·d dll'sl pain lhal was ..\ac,·rhall·d with lying snpi",': h.· d, ·ni.·d dysp,wa wilh ,·",rlion . lIis ph ysical ..,aminalion showl'd normal , ·ital sign s wilhonl r..spiralory disln·ss. II.· had s('all .. r..d I..sions compalihl.. wilh Kaposi's sar('oma 0""1' his fa(·.·. lrunk . and .-,In·mili..s. with a singl. ·I ..sion on his hard palal ... Th .. lnngs w"n' d"ar 10 ans('nltalion. Ih.. h,-arl IfIIll'S weI''' normal, a nd Ih.. re was no palpahl.· organom"ga'" on alxlominal ..,aminalion. Findings from Ilw n·maind,-,. of his physical .. xaminalion w.. n · nnn·markahl.- . Asid. · from a Iwmalo<:ril of :32.i p.. r<:,·nl and a la<'lall' d..hydrog,·na ,,· \"ah,.. of 1 . :~1.,) UtI, (npI)('r normal, filii), n 'snlts of Ilw S(T" I' ning lahoralory I',amination WI·.... normal. Clwsl radiograph was ohlaillt'd (Fig I) and shm\'f-d prominl'nl inlt 'rslilial markings and nll'diaslinal ad. ·nopalhy. Th.· ad"nopathy was I'lIlfirnlt'd h\" I·ompnl..d 1.lIImgraphie sean of lIlt' ('h..sl.

F, cl'lW I . l'romi'lf'nl inlt 'rslitial markings. hilah'ral hila,. ad.-Impa · thy. and righl paralradwal adl 'lmpall,,' is "-I 'n .

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Brunchoscopv was perform..d . and a solit arv plaqu.. of pre-sumptive- Kaposfs sarcoma was s.... n at Ih.. right upper loht· carina . Multipl« pal .. white friahl .. polypoid mucosa] I..sions m..asnring :3 10 5 mm in diameter w • s", 'n in 11ll' right low..r lollt' bronchus and 1111' anterior and la ral basilar " 'gITlt'nlal bronchi . Stains of bronchial washings w.. n · IT"gali"t' for mvcohact.. ria . fnngi. bact e rial pathog.. ns , a"d P cllri"ii . Two davs lal.. r, Ih.· pati .. nl h.. earn.. profonndly dvspneu- , with a r..slingowg.. n salnralion 01'''-') perr -.. nl. B"p"al ch"sl radiograph (not shown ) show..d no pneumothorax. slahl.. inl .. rstilial di,,-as,· . and ad ..nopathy with new bilateral small pl.. nral ..Airsions and a sm all righl lower lohl' inIHtral e. Tn-atm.. nl with e-lurtthromycin. 50() mg orally r-ve-ry 12 h. and e-thambutol. (.') mWkg orally 0""" daily. was Iwgnn JK·nding ...-sults of my('1hacll'ria hlood cultun-s and hronchial wash cultu...·s. Thr- polypoid I,-sions (Fig 2 ) showed histologie ft'ahlr..s typicnl of BA (nol shown). Cro"ps of prolif"raling c-apillarv-xi z..d \""ssl'ls with plump .. ndotlll'liall·I·lIs w .. re prese-nt hen ...u h a metaplustie squnmnu« mucosa . Th,·...• was a mixed inflammalory c.. 11 infiltrnt». indnding nenlrophik \Varlhin -Starry slain dernonstruted nnmerons hadllary strudnrt·s a sS( ,,·iah·d with tIll' tissllt· re action . On,' month lalt'r. Ill' had I·ompl..... rl'solntion of all symptoms. a normal ('h.-st radiograph. and room -air owg.. n satnration of 95 pprt·.. nl at ...·sl. II.. had no ;"h-.. r,,· .. If.....s from Ih,· clarithrnmyl'in. All ..lIltll,..-, "'..,... n,·galin- . and ..thamhlllol lh"rapy was di'I'lIltinlI..d : h,· I·mnpl ..t..d a l",o-m'lIllh COllrsl' of d arithromyl'in . Th.. r.. was lie. n 'g,nO ss ifUl fl tht'

( 'lllafU'cnl" K aposi 's

san"'()ma It-sinn",.

CO"tMF"IT

Interstitial lung dist'ase is a common dinical prohlem in tilt' H J\ '-inft'ded pati(·nt . Th e inff'l'tious difff'rf'ntial diagnosis is I'xtn'nwly hroad and includes P ('(/rinii. Cryptorocrl/s 'lI'o!orlll(/ns. and dimorphic fungi , including Histo1J!aslIIo rapsll!(/tl/lII and Co('ridioidf'S illllllifis. My('ohacteria sppcies, To:wp!(/sIIwl!.ondii. and . rarply, virust·s or pyogt'niC' hacteria . HiJar adl'nopathy gt>.wrally lim its thp inf('ctious ('onsidf'rations to fungal or mycohaeterial dis('ast's, with Kaposi s sareoma tht· prf'dominant nf'oplastic ('ausp . Becausp of this patit'nts f'xtf'nsi"e eutaneous disf'asf'. Wf' were suspicious that the hypoxia and radiographic findings Wprt· those of pulmonary Kapos(s sarcoma: the f('\'t'r was thought to hf' s('clllldary to disst'minated M!lroh(/cfPriIlIll (/vil/III complex, which initiated I'lllpiric therapy with clarithromycin and ethamhutol whilt' awaiting hlood culturf' and hronchoscopy n·sults. Histologic study demonstrating BA," the ahsf'nc(' of altf'r-

F, r;"IlF 2. Photomicrograph (I,,·matoxylin-I·o, in. original m:lgnifi,·alilln . x :;) d. 'mml\trating an 1'1 .." alf't1 hrnn..hialllll\(·osall,·sion . Bronchopulmonary Bacillary Angioma.osis (Foltter et a/)

native pathogens on bronchoalveolar lavage. as well as the complete clinical and radiographic response to directed antimicrobial therapy support the pathogenicity of R henselae in this patient. To our knowledge, bronchial polyps and interstitial lung disease with associated hilar adenopathy have not been associated previously with BA; however. this clinical picture has been reported with pulmonary Mycobacterium avium complex in AIOS patients." The agent of BA has been recently identified as R henselae, a rickettsial organism . Tick bites have been epi demiologically linked to transmission of infection. yet this association remains unproved ." Antibiotics reported effective for BA include erythromycin, doxycycline. riiampin, and chloramphenicol.'·- Because of the potential for intracellular localization of R henselae coupled with slow growth characteristics. protracted administration of antibiotics capable of intracellular penetration has been recommended ." Clarithromycin, a 6-o-methyl derivative of erythromycin that demonstrates unusually high tissue and polymorphonuclear cell penetration. may be a particularly active antibiotic in the treatment of BA. This case serves to expand the clinical presentation of BA as well as the differential diagnosis of interstitial lung disease with hilar adenopathy and bronchial polyps in HIV-infected patients. Effective antimicrobial regimens should include c1arithromycin. ACKNOWLEDGMENTS: We thank Dr. Philip E. LeBoit, University of California, San Francisco. for review of the hronchial biopsy specimen.

REFERENCES

2

3

4

5

6

7

II

Koehler JE. LeBoit PE . Egbert BM . Berger TG . Cutaneous vascular lesions and disseminated cat-scratch disease in patients with the acquired tmmunodefieiency syndrome (AIDS) and AIDSrelated complex . Ann Intern Med 1988; 109:449-55 Kemper CA. Lomhard CM, Deresinski SC. Tompkins LS. Visceral hacillary angiomatosis: possible manifestations of disseminated cat-scratch disease in the immunocompromised host: a report of two cases. Am J Med 1990; 119:216-22 Perkocha LA, Ceaghan SM. Benedict Yen TS . Nishimura SL. Chan Sf, Garcia-Kennedy R. et al. Clinical and pathologic features of bacillary peliosis hepatis in association with human immunodeficiency virus infection. N EnRI J Med 1990; 32.1:15111 -116 Spach DH. Panther LA. Thorning DR . Dunn JE. Plorde 11. Miller RA. Intracerebral bacillary angiomatosis in a patient infected with human immunodeficiency virus , Ann Intern Med 1992; 116:740-42 Slater LN. Welch DF. Hensel D . Coody DW A newly recognized fastidious Cram-negattve pathogen as a cause of fever and hacteremia. N Engl J Med 1990; 323 :15117-93 Lucey D . Dolan MI, Moss CWo Garcia M, Hollis DG. WeRner S. et al. Relapsing illness due to RochalifJllU'a henselae in immunocompetent hosts: implications for therapy and new epidemiologic associations. Clin Infect Dis 1992; 14:683-811 LeBoit PE. Berger TG . Egbert BM, Beckstead JH. Benedict Yen TS . Stoler MH. Bacillary angiomatosis: the histopathology and differential diagnosis of a pseudoneoplastic infection in patients with human immunodeficiency virus syndrome. Am J SUfRPathol 19119; 13:909-20 Packer SJ, Cesario T. Williams JH Jr. Mycobacterium avium complex infection presenting as endobronchial lesions in immunosuppressed patients. Ann Intern Med 1988; 109:389-93

Inclusion Body Myositis as a Cause of Respiratory Failure* Rubin Cohen. M.D .; Stanlry l ipper: M.D .; and David R . Dantzker; M .D .. F.C.C .P.

Inclusion body myositis (IBM) is a slowly progressive myopathy that has not been reported to affect respiratory muscles. It is often refractory to treatment and a muscle biopsy specimen is necessary for the diagnosis. This is a report of a patient with IBM who quickly progressed to respiratory muscle failure requiring intubation. (Chest 1993; 104:975-77) 118M

=inclusion body myositis

I

I

nclusion body myositis (IBM) is a form of inflammatory myopathy that is characterized by a protracted course. involvement of distal muscles. and unresponsiveness to steroid therapy. The most severely affected muscles are those of the limbs and. to our knowledge. there are no reports of respiratory muscle involvement. Extramuscular involvement of this disease has been described infrequently and usuallv involves the cardiovascular and the gastrointestinal systems, We describe a patient with respiratory failure secondary to IBM. CASE REPORT

A fi9-vear-old woman was ad mitted to the ICU with respiratory Iailure. Sht' had a 10-day history of progressive shortness ofhreath. cough . lethargy. and weakness, There was no history offe\'er, swe ats, or chills. The patient was hospitalized in 19117 with a similar presentation. At that time, she had complained of a 2-week history of progressive dyspnea and slowly worsening limh weakness for the preceeding five years . The electromyogram (EMG) at that time was compatible with a myopathy; however. the creatine kinase (CK) and liver function test results were normal. She was found to have an elevated partial thromboplastin time (P'IT) that was shown to he due to a lupus anticoagulant, Her antinuclear antibody (ANA) titer was I :320. Her platelet count was normal as were the results of her thyroid function tests. A Tensilon test was negative. The chest radiograph was normal. She required mechanical ventilation and was tr eated as havin~ mixed connective tissue disease and received lar~e doses of intravenous methylprednisolone sodium succinate (Solu-Medrol), Her hospital course was complicated hy multiple infections that were , in part. attributed to the large doses of steroids. As then' was no clinical improvement , the steroid therapy was disrontinued. The patient underwent a muscle hiopsy from the left quadricep that showed isolated myofiber degeneration, atrophic fibers. and no inRammatory infiltrates. She was discharged from the hospital 1 year later with a dtagnosts of idiopathic myopathy. She remained well until the present hospital admission without worsemng of the myopathy or further respiratory problems, The physical examination showed the patient to he in moderate respiratory distress. She had petechiae on both calves and poor air entry in the lungs , which were otherwise clear. The neurologic examination showed her to he alert and oriented. with distal muscle ·From the Long Island Jewish Medical Center (Drs. Cohen and Dantzker) New Hvde Park, NY; Alhert Einstein College of Medicine ·(D rs. Cohen and Dantzker), and Winthrop University Medical Center (Dr. Lipper), Mineola. NY. Reprint "quests: Dr. Cohen, Allmonnry and Critical Care , l.m1j!; Island Jewish Medical Center, New Hyde Fbrk, New York 11040 CHEST 1104 I 3 I SEPTEMBER, 1993

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