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Abstract
Interstitial pneumonia occurs in approximately 25% of patients with primary Sjögren's syndrome. Interstitial pneumonia combined with primary Sjögren's syndrome usually responds well to systemic steroids, and fatal cases are rare. Lymphocytic interstitial pneumonia shows diffuse infiltration of polyclonal B and T cells. Autologous stem cell transplantation is performed in cases of primary Sjögren's syndrome as an optional treatment when the condition responds poorly to conventional treatment. The hypothesis that primary Sjögren's syndrome improves after transplantation relies on the role of B-cell abnormalities in pathogenesis or the strong effects of immunosuppressive therapy. We experienced the case of a patient diagnosed with primary Sjögren's syndrome and lymphocytic interstitial pneumonia progression refractory to conventional treatment (steroid and immunosuppressive drugs) and cyclophosphamide pulse therapy. Our patient demonstrated improvement of lung manifestations and autoimmune disease activity after autologous stem cell transplantation.
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Figure 1.
(A) Multiple cysts are found in various sizes on both upper and middle lobes of lung accompanied by multiple lymphadenopathy. (B) Small sized centrilobular nodules are found mainly on both lower lobes of lung. (C) HRCT taken 6 months after auto SCT. Inflammation was improved on both lung field, size and number of lymphadenopathy were all decreased. (D) Both size and number of centrilobular nodules presented on both lung fields were all decreased.
Figure 2.
(A) 2×100 (H&E stain), (B) 4×400 (H&E stain). The section shows diffuse interstitial infiltration of lymphoid cells and plasma cells. Scattered small lymphoid follicles are present.
Figure 3.
Presented the flow of CRP (mg/L) and ESR (mm/h) during observation periods of the patient. Performed cytoxan pulse therapy from March, 2007 to August, 2007. Performed VAD induction chemotherapy from March, 2009 to June, 2009. Performed autologous SCT in December, 2009.
Table 1.
Outcome after autologous SCT of Sjögren's syndrome combined with or without hematologic malignancy (6,9-11)
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