LAUSR

To the Editor: A 43-year-old woman was referred to the Autoimmune Diseases Unit in 2012 because of anti-nuclear antibody (ANA) positivity detected on Hep2 cells (1:160, homogenous pattern). There was no family history of autoimmune disease, rather a strong family history of colorectal cancer and no parental consanguinity. She had asthma as a child and from age 15 she suffered from guttate psoriasis and hypothyroidism for which she did not receive any systemic immunosuppressive therapy. Vitiligo, in the axillae and thighs, had responded to topical therapy. An atrioventricular nodal re-entrant tachycardia was resolved by radiofrequency catheter ablation. At age 18, a pulmonary mycobacterial infection was refractory to standard therapy requiring right lower lobe resection 18months later. Histological analysis of the surgical specimen confirmed ongoing parenchymal mycobacterial infection (species unidentified) and she required further therapy. She was well until age 39 when she complained of fatigue, chronic productive cough, exertional dyspnea, and right-sided pleuritic chest pain. There was no relevant travel history, smoking, occupational, or animal/pet exposure. Further tests revealed reduced diffusing lung capacity, centrilobular micronodularity, and septal and subpleural reticulation with interlobular septal thickening. Bronchial biopsy demonstrated a chronic inflammatory process with erosion and epithelial reactive metaplasia; there were no signs of neoplasia or infection upon bronchial lavage. An open lung biopsy showed multiple bronchiolectasis with areas of suppuration, nonnecrotizing granulomas, fibroblast proliferation, pneumocyte hyperplasia, and a mixed inflammatory infiltrate. Acid-fast bacilli, fungal stains, and microorganism cultures obtained from lavage and biopsy specimens, HIV antibody testing, and avian precipitins were negative; serum concentration of alpha 1 antitrypsin, angiotensin-converting enzyme, and vitamin D were in the normal range. Overall findings led to a presumptive diagnosis of hypersensitivity pneumonia. At that time, thrombocytopenia was encountered followed by pancytopenia (Table 1). Of note, there was severe monocytopenia but previous counts were unavailable. Bone marrow aspirate and trephine biopsy were unremarkable. By the time she was referred to our Autoimmune Diseases Unit, she was followed in Pneumology, Hematology, Gastroenterology, Endocrinology, and Dermatology. Psoriasis was present in the form of erythematous papules (approximately 3 to 5mm) in the scalp, and extensor surfaces of the arms and thighs. Flow cytometry in whole blood revealed lymphopenia with a total of 695 cells/μL. More specifically, there was a reduced frequency and number of CD3CD4 T cells (28% and 197/μL, respectively; normal range 31–60% and 410– 1590 cells/μL), and normal proportion but reduced number of B cells (defined as CD19; 7% and 47/μL, respectively; normal range 6–25% and 90–660 cells/μL) as well as normal frequency but reduced number of NK cells (defined as CD3/ CD1656, 11% and 78/μL; normal range 5–27% and 90– 590 cells/μL). Immunoglobulin M, G, and A concentrations Electronic supplementary material The online version of this article (https://doi.org/10.1007/s10875-018-0576-x) contains supplementary material, which is available to authorized users.