A chronic pulmonary syndrome associated with graft-versus-host disease after allogeneic marrow transplantation.
Of 143 consecutive patients who survived at least 6 months after bone marrow transplantation (allogeneic [n = 131]; syngeneic [n = 5]; or autologous [n = 7]) and whose pulmonary function was evaluated before and on at least 2 occasions after BMT, 29 (20%) developed a chronic pulmonary syndrome without evidence for an infectious etiology. Twenty-eight (97%) presented with cough and 22 (76%) with dyspnea; abnormal chest signs were crackles in 23 (79%) and wheeze in 22 (76%). Chest roentgenogram showed pulmonary infiltrates in 15 (52%) cases but was normal in 14 (48%). All patients had major reductions in lung volumes (forced expiratory volume in 1 sec [FEV1]; relaxed vital capacity [VC]; and alveolar volume [VA]), and/or diffusing capacity (pulmonary diffusing capacity [TLCO] and single-breath carbon monoxide coefficient [KCO]). The obstructive component varied with only 18 (62%) patients developing overt airways obstruction (FEV1/VC < 75%), and in 6 of this group the fall in lung volumes preceded the onset of airways obstruction. Open lung biopsy (n = 4) showed both bronchiolitis obliterans and chronic patchy interstitial pneumonitis. The development of this syndrome was associated with acute (P < 0.001) and chronic (P < 0.0001) graft-versus-host disease of other organ systems. Twenty-four (83%) patients had a partial or complete response to immunosuppressive agents. Six (21%) have died, five (17%) of pulmonary complications. We suggest that this syndrome may be a manifestation of chronic GVHD involvement of the lung.
Schwarer
A P
AP
MRC/LRF Leukaemia Unit, Royal Postgraduate Medical School, London, United Kingdom.
Hughes
J M
JM
Trotman-Dickenson
B
B
Krausz
T
T
Goldman
J M
JM
D000293:Adolescent; D000328:Adult; D000402:Airway Obstruction / Q000150:complications / Q000000981:diagnostic imaging / Q000382:microbiology; D001706:Biopsy; D016026:Bone Marrow Transplantation / Q000009:adverse effects*; D002648:Child; D002908:Chronic Disease; D005260:Female; D006086:Graft vs Host Disease / Q000150:complications* / Q000209:etiology*; D006801:Humans; D008168:Lung / Q000473:pathology; D008171:Lung Diseases / Q000150:complications* / Q000453:epidemiology / Q000503:physiopathology; D008297:Male; D008875:Middle Aged; D011658:Pulmonary Fibrosis / Q000150:complications / Q000000981:diagnostic imaging / Q000382:microbiology; D011859:Radiography; D012129:Respiratory Function Tests; D012307:Risk Factors; D013577:Syndrome; D014184:Transplantation, Homologous