Pulmonary Vein Vasculitis Presenting as Multiple …Tamai, et al: Pulmonary SLE vasculitis 323...
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323 Tamai, et al: Pulmonary SLE vasculitis Personal non-commercial use only. The Journal of Rheumatology Copyright © 2019. All rights reserved. Images in Rheumatology Pulmonary Vein Vasculitis Presenting as Multiple Pulmonary Nodules in a Patient with Systemic Lupus Erythematosus HIROYA TAMAI, MD; NAOSHI NISHINA, MD, PhD; TSUTOMU TAKEUCHI, MD, PhD, Keio University School of Medicine, Tokyo, Japan. Address correspondence to Dr. H. Tamai, 35 Shinano-machi, Shinjuku-ku, Tokyo, Japan, 160-8582. E-mail: [email protected]. Ethics board approval is not required because this is a single case report and no intervention had been made for research. The patient gave written informed consent to publish the material. J Rheumatol 2019;46:323–4; doi:10.3899/jrheum.180602 Pulmonary involvement in systemic lupus erythematosus (SLE) can take the form of pleuritis, interstitial lung disease, alveolar hemorrhage, or pulmonary hypertension, but rarely does it appear as pulmonary vein vasculitis 1 . A 19-year-old woman was diagnosed with SLE 6 months before presentation because of malar rash, alopecia, arthritis, leukocytopenia, low complement, and positive anti-DNA antibody, and 30 mg daily prednisolone (PSL) treatment was started. Fever, skin ulcers on the scalp, and dry cough appeared 2 months before presentation, while she was taking 15 mg of PSL. Thoracic computed tomography (CT) scan revealed bilateral multiple nodules distributed along pulmonary veins (Figure 1A). The patient had jaundice and discoid lesions with ulcers on the scalp on physical examination. Laboratory findings showed acute liver dysfunction, decreased complement titer, positive anti-DNA antibody, negative antiphospholipid antibody, and no kidney involvement. Lung biopsy revealed lymphocytes and foam cell infiltration and fibrosis around pulmonary veins (Figure 2C–E) with disrupted internal elastic lamina (Figure 2F). Methylprednisolone pulse therapy followed by 50 mg of daily PSL was started, and intravenous cyclophosphamide treatment was added. The discoid lesions resolved with scarring, and liver dysfunction improved soon after the initiation of therapy. One month later, thoracic CT scan showed that the pulmonary nodules were drastically reduced in size (Figure 1B). Our original view was that the patient’s pulmonary vein vasculitis was a manifestation of SLE because the SLE-specific discoid lesions were exacerbated at the same time. Although we sometimes see SLE with vasculitis, this case included the extremely unusual presentation of vasculitis limited to pulmonary veins 2 . This case highlights the possi- bility of vasculitis when nodules along pulmonary veins are found. ACKNOWLEDGMENT We thank Dr. H. Sugiura for his advice on radiological findings. We also thank Dr. A. Sasaki and Dr. K. Kameyama for useful comments on the pathology. REFERENCES 1. Mittoo S, Fell CD. Pulmonary manifestations of systemic lupus erythematosus. Semin Respir Crit Care Med 2014;35:249-54. 2. Ramos-Casals M, Nardi N, Lagrutta M, Brito-Zeron P, Bove A, Delgado G, et al. Vasculitis in systemic lupus erythematosus: Prevalence and clinical characteristics in 670 patients. Medicine 2006;85:95-104. Figure 1. CT manifestations. A. CT scan revealed nodules distributed along pulmonary veins. B. CT scan taken 1 month later showed that the pulmonary nodules were drastically reduced in size. CT: computed tomography. www.jrheum.org Downloaded on December 15, 2020 from
Transcript of Pulmonary Vein Vasculitis Presenting as Multiple …Tamai, et al: Pulmonary SLE vasculitis 323...
323Tamai, et al: Pulmonary SLE vasculitis
Personal non-commercial use only. The Journal of Rheumatology Copyright © 2019. All rights reserved.
Images in Rheumatology
Pulmonary Vein Vasculitis Presenting as MultiplePulmonary Nodules in a Patient with Systemic LupusErythematosusHIROYA TAMAI, MD; NAOSHI NISHINA, MD, PhD; TSUTOMU TAKEUCHI, MD, PhD, Keio University School of Medicine, Tokyo, Japan.Address correspondence to Dr. H. Tamai, 35 Shinano-machi, Shinjuku-ku, Tokyo, Japan, 160-8582. E-mail: [email protected]. Ethics board approval is notrequired because this is a single case report and no intervention had been made for research. The patient gave written informed consent to publish thematerial. J Rheumatol 2019;46:323–4; doi:10.3899/jrheum.180602
Pulmonary involvement in systemic lupus erythematosus(SLE) can take the form of pleuritis, interstitial lung disease,alveolar hemorrhage, or pulmonary hypertension, but rarelydoes it appear as pulmonary vein vasculitis1. A 19-year-old woman was diagnosed with SLE 6 monthsbefore presentation because of malar rash, alopecia, arthritis,leukocytopenia, low complement, and positive anti-DNAantibody, and 30 mg daily prednisolone (PSL) treatment wasstarted. Fever, skin ulcers on the scalp, and dry coughappeared 2 months before presentation, while she was taking15 mg of PSL. Thoracic computed tomography (CT) scanrevealed bilateral multiple nodules distributed alongpulmonary veins (Figure 1A). The patient had jaundice and discoid lesions with ulcerson the scalp on physical examination. Laboratory findingsshowed acute liver dysfunction, decreased complement titer,positive anti-DNA antibody, negative antiphospholipidantibody, and no kidney involvement. Lung biopsy revealedlymphocytes and foam cell infiltration and fibrosis aroundpulmonary veins (Figure 2C–E) with disrupted internalelastic lamina (Figure 2F). Methylprednisolone pulse therapyfollowed by 50 mg of daily PSL was started, and intravenouscyclophosphamide treatment was added. The discoid lesionsresolved with scarring, and liver dysfunction improved soon
after the initiation of therapy. One month later, thoracic CTscan showed that the pulmonary nodules were drasticallyreduced in size (Figure 1B). Our original view was that the patient’s pulmonary veinvasculitis was a manifestation of SLE because theSLE-specific discoid lesions were exacerbated at the sametime. Although we sometimes see SLE with vasculitis, thiscase included the extremely unusual presentation of vasculitislimited to pulmonary veins2. This case highlights the possi-bility of vasculitis when nodules along pulmonary veins arefound.
ACKNOWLEDGMENTWe thank Dr. H. Sugiura for his advice on radiological findings. We alsothank Dr. A. Sasaki and Dr. K. Kameyama for useful comments on thepathology.
REFERENCES 1. Mittoo S, Fell CD. Pulmonary manifestations of systemic lupus
erythematosus. Semin Respir Crit Care Med 2014;35:249-54. 2. Ramos-Casals M, Nardi N, Lagrutta M, Brito-Zeron P, Bove A,
Delgado G, et al. Vasculitis in systemic lupus erythematosus:Prevalence and clinical characteristics in 670 patients. Medicine2006;85:95-104.
Figure 1. CT manifestations. A. CT scan revealed nodules distributed along pulmonary veins. B. CT scan taken 1 month later showedthat the pulmonary nodules were drastically reduced in size. CT: computed tomography.
www.jrheum.orgDownloaded on December 15, 2020 from
324 The Journal of Rheumatology 2019; 46:3; doi:10.3899/jrheum.180602
Personal non-commercial use only. The Journal of Rheumatology Copyright © 2019. All rights reserved.
Figure 2. Histopathology of the lung tissue. Panels C–E show lung biopsy that revealed lymphocytes and foam cell infiltration and fibrosis aroundpulmonary veins, but without pulmonary artery lesions or thrombosis (H&E staining, magnification C: ×40, D: ×100, E: ×200). F. Disrupted internalelastic lamina were also seen (arrows, Elastica van Gieson staining, magnification ×100).
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