A 55 year old female with rheumatoid arthritis who was repeatedly admitted to internal medicine for fever, shortness of breath and pleuritic chest pain. Laboratory work up showed normal WBC but elevated CRP and sedimentatation rate. Cultures were negative. Imaging studies revealed elevated diaphragms, bilateral atelectasis and pleural fluid but normal lung parenchyma. Lung function testing showed restriction. Anti-dsDNA and anti-Ro/SSA were elevated. A clinical diagnosis of anti-TNF-induced lupus secondary to infliximab and shrinking lung syndrome was made. The patient showed improvement on steroids but subsequent worsening when tapered. Rituximab was then initiated with good results.

Figure I Chest X-rays  (CxR).  a)  At outpatient clinic (T0 + 8 months): Atelectasis in the right lower lobe,  otherwise normal CxR. b) Admission #1 (10 days later): Shallow breathing. Bilateral pleural fluid and left lower lobe opacity. c) Admission #3 (T0 + 14 months): Shallow breathing, encapsulated fluid collection in right lung and a small amount of pleural fluid on the left. Images b) and c) show clearly how small the lungs have become compared to the first image. d) Follow-up (T0 + 31 months). Shallow breathing but improving. Less atelectases and no pleural fluid.

Figure II Chest CT early on during admission #1: Collapse of lower lobes and bilateral pleural fluid, 2 cm on the right side and 3 cm on the left side. No lymphadenopathy or nodules were seen and the lung parenchyma was otherwise normal.

Table I Laboratories; white blood cells, inflammatory markers and rheumatologic serologies. Abbreviations: WBC = white blood cell count. ESR = erythrocyte sedimentation rate. CRP = C reactive protein. RF = rheumatoid factor. Anti-CCP = anti-citrullinated protein antibody. ANA = anti-nuclear antibody. ENA = extractable nuclear antigen antibody. Anti-dsDNA = anti –double stranded DNA. CH50 = total complement measurement. C3 = complement component 3. C4 = complement component 4.

Table II Pulmonary function testing. Abbreviations: FVC = forced vital capacity. FEV1= forced expiratory volume in one second. TLC = total lung capacity. RV = residual volume.  DLCO = diffusing capacity for carbon monoxide

Table III Timeline of illness.