The online version of this article (doi:10.1186/1752-1947-6-191) contains supplementary material, which is available to authorized users.
The authors declare that they have no competing interests.
SS, YO and HK made substantial contributions to the histological examination and revised the manuscript. HN made substantial contributions to conception and design of the study, and analysis and interpretation of data. KT and YM were involved in revising the manuscript critically for important intellectual content. SM, TF and RE were involved in the care of our patient in the pulmonary department. TN and FT performed a thoracoscopic lung biopsy and revised the manuscript. All authors read and approved the final manuscript.
Granulomatous lesions are commonly encountered abnormalities in pulmonary pathology, and often pose a diagnostic challenge. We report an unusual case of granulomatous lung disease with uncommon characteristics, which developed following Epstein-Barr-virus-induced mononucleosis and new-onset systemic lupus erythematosus. We aim to highlight a diagnostic approach for the condition and to raise awareness of the possibility of it being related to the immunological reaction caused by Epstein-Barr virus infection.
A 36-year-old Japanese man, who had been diagnosed with Epstein-Barr-virus-induced infectious mononucleosis, new-onset systemic lupus erythematosus, and secondary Sjögren’s syndrome three weeks previously, presented to our facility with fever and diffuse pulmonary infiltrates. A computed tomography scan of the chest revealed multiple small nodules in both lungs. Fiberoptic bronchoscopy with bronchoalveolar lavage revealed lymphocytosis with predominance of T lymphocytes. A histological examination of a lung biopsy taken during video-assisted thoracic surgery showed randomly distributed tiny granulomatous lesions with infiltration of eosinophils. The differential diagnoses included hypersensitivity pneumonitis, sarcoidosis, and pulmonary involvement of Crohn’s disease, systemic lupus erythematosus, and Sjögren’s syndrome, but the clinical and pathological findings were not consistent with any of these. Our patient’s condition did not improve; therefore, prednisolone therapy was started because of the possibility of specific immunological reactions associated with Epstein-Barr virus infection. After steroid treatment, our patient showed radiological and clinical improvement.
To the best of our knowledge, this is the first case of a patient developing randomly distributed multiple granulomatous lung lesions with eosinophilic infiltrates after Epstein-Barr virus infection and systemic lupus erythematosus. On the basis of our data, we hypothesize that Epstein-Barr virus infection altered the immune response of our predisposed patient and contributed to the pathogenesis of the lung lesions. Our patient’s clinical response to steroid treatment was excellent.
Mukhopadhyay S, Gal AA: Granulomatous lung disease: an approach to the differential diagnosis. Arch Pathol Lab Med. 2010, 134: 667-690. PubMed
Barrios RJ: Hypersensitivity pneumonitis: histopathology. Arch Pathol Lab Med. 2008, 132: 199-203. PubMed
Longcope WT, Freiman DG: A study of sarcoidosis; based on a combined investigation of 160 cases including 30 autopsies from The Johns Hopkins Hospital and Massachusetts General Hospital. Medicine (Baltimore). 1952, 31: 1-132. 10.1097/00005792-195202000-00001. CrossRef
Chowers Y: Interaction of genetic, environmental and immune factors in the pathogenesis of inflammatory bowel diseases. Isr Med Assoc J. 2002, 4: 815-817. PubMed
Chaiamnuay S, Heck LW, Bell WC, Bastian HM: Acute granulomatous lupus pneumonitis: the first case report. Lupus. 2007, 16: 201-204. PubMed
Ramos-Casals M, Brito-Zerón P, García-Carrasco M, Font J: Sarcoidosis or Sjögren syndrome? Clues to defining mimicry or coexistence in 59 cases. Medicine (Baltimore). 2004, 83: 85-95. 10.1097/01.md.0000121237.98962.1e. CrossRef
Fairfax AJ, Haslam PL, Pavia D, Sheahan NF, Bateman JR, Agnew JE, Clarke SW, Turner-Warwick M: Pulmonary disorders associated with Sjögren’s syndrome. Q J Med. 1981, 50: 279-295. PubMed
- Multiple granulomatous lung lesions in a patient with Epstein-Barr-virus-induced mononucleosis and new-onset systemic lupus erythematosus: a case report
- BioMed Central