Supplementary Materials

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  • Supplementary Materials and Methods
  • Fig. S1. Enlarged mediastinal lymph nodes in patients with APECED pneumonitis.
  • Fig. S2. Association of autoantibody reactivity against cytokines and tissue-specific autoantigens and the time to development of pneumonitis in patients with APECED.
  • Fig. S3. Serum inflammatory markers in patients with APECED pneumonitis.
  • Fig. S4. Immunoglobulin concentrations in patients with APECED pneumonitis.
  • Fig. S5. Leukocyte subsets other than neutrophils do not exhibit increased accumulation in the airways of patients with APECED pneumonitis.
  • Fig. S6. Primary lymphoid follicle and germinal center formation in lung tissue of patients with APECED pneumonitis.
  • Fig. S7. Intraepithelial lymphocyte infiltration in Aire−/− mouse lung tissue.
  • Fig. S8. Schematic illustration of the lymphocyte-directed immunomodulation used in this study.
  • Fig. S9. Treatment response assessed by radiography in patient 1.
  • Fig. S10. Treatment response assessed by radiography in patient 2.
  • Fig. S11. Treatment response assessed by radiography in patient 3.
  • Fig. S12. Treatment response assessed by radiography in patient 4.
  • Fig. S13. Treatment response assessed by radiography in patient 5.
  • Fig. S14. Lymphocyte-directed immunomodulation causes rapid depletion of B, but not T, lymphocytes in peripheral blood of patients with APECED with pneumonitis.
  • Fig. S15. Lymphocyte-directed immunomodulation improves salivary production in patients with APECED with Sjogren’s-like syndrome.
  • Fig. S16. Lymphocyte-directed immunomodulation caused resolution of nail dystrophy in one of the three patients with APECED with this condition.
  • Table S1. Demographic and geographic origin characteristics of our patients with APECED.
  • Table S2. Demographic, clinical, and radiographic response characteristics of the five patients with APECED pneumonitis who received lymphocyte-directed immunomodulation.
  • Table S3. Patients with APECED pneumonitis do not carry serum autoantibodies against MDA5.
  • Table S4. Percent of lymphocyte subsets within corresponding lymphocytes in the peripheral blood of patients with APECED with or without pneumonitis.
  • Table S5. Absolute numbers of lymphocyte subsets in the peripheral blood of patients with APECED with or without pneumonitis.
  • Table S6. Standardized pulmonary clinical history questionnaire used for the evaluation of clinical features of APECED pneumonitis in our study.
  • Table S7. Clinical, radiographic, lung biopsy, and autoantibody features of pneumonitis in the 21 affected patients with APECED included in this study.
  • References (4858)

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