Shrinking lung syndrome in autoimmune inflammatory diseases: A case series and review of literature

Xiao-chan Chen, Shan He, Jing Xue

Table 1. Main clinical characteristics of the three patients in this study

Variables Case 1 Case 2 Case 3 Age, years 26 53 68 Sex Female Female Female Primary disease SLE SLE AOSD Duration of the primary disease, years 12.0 2.0 0.4 Treatment before the diagnosis of SLS Prednisone, HCQ, TAC Prednisone, HCQ Prednisone Presenting features of SLS Dyspnea, cough Dyspnea, chest pain Dyspnea Other clinical manifestations Mucocutaneous, arthritis, fever, nephritis Mucocutaneous, arthritis, nephritis, cardiac valvular dysfunction Fever, mucocutaneous Autoantibodies ANA, Smith, RNP, SSA, Ro-52, SSB, ARPA ANA, dsDNA, SSA, Ro-52, SSB, None Chest CT Aatelectasis, diaphragm elevation, pleural effusion, pleural thickening Aatelectasis, pleural effusion Atelectasis, diaphragm elevation SLEDAI 10 20 / Pulmonary function test Forced vital capacity, % 62.5 59.1 72.4 Total lung capacity, % 69.9 72.2 66.9 FEV1, % 67.3 65.0 74.8 DLCO, % 42.4 55.2 50.4 Treatment MP 60 mg/d+CYC+TAC MP 500 mg/d+CYC+IVIG MP 160 mg/d+CYC Follow-up time, months 36 8 6 Clinical outcome Improved Improved Improved PFT outcome Improved Improved Not improved HRCT outcome Improved Improved Not improved