Perhimpunan Dokter Paru Indonesia - Rare association of organising pneumonia with pure red cell aplasia
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Rare association of organising pneumonia with pure red cell aplasia




Shruthi Konda1, Betty Cheung2, Lola Loewenthal1 and Raminder Aul1
1Respiratory Medicine, St. George's University Hospital, London, United Kingdom,?2Haematology, Croydon University Hospital, London, United Kingdom

Introduction: Organising pneumonia (OP) is commonly associated with connective tissue diseases (CTD)(1). Haematological disorders e.g.myelodysplastic syndromes (MDS) are associated with IPF in patients with telomerase mutations (2).

Aim: We present a case of a 38-year-old lady with Pure Red Cell Aplasia (PRCA) and refractory OP.

Case: The patient presented with breathlessness in July 2013. She was unable to perform spirometry (poor technique). HRCT chest showed classical OP. FBC, CK, CRP, ANA, ENA, anti Jo-1 and myositis panel were normal. There were no features of CTD. She was known to have schizophrenia, and was on clozapine, then aripiprazole, which was discontinued.

After 1 year she developed Pure Red Cell Aplasia. CT abdomen revealed an incidental unrelated angiomyolipoma. She then developed type 1 respiratory failure (T1RF), and a CT chest showed worsening OP, and no features of LAM. BAL showed no growth, with 96% macrophages, 2% lymphocytes and 1% neutrophils. She was pulsed with methylprednisolone, with no improvement. She was commenced on oral cyclophosphamide, with haematology input, which improved the T1RF. She remained transfusion dependent for her PRCA. She has lately been switched to oral cyclosporine, and is awaiting genetic testing for telomerase mutations.

Discussion: Fibrotic ILDs can occur with MDS, which can be difficult to manage. They usually respond to treatment of the haematological disorder, indicating a common immuno-genetic link. This is the first reported case of OP in association with PRCA. Increased clinician awareness of this association and further exploratory studies are needed.

References: 1. Asano et al,SpringerPlus 2014,3:3. 2.Yang L et al,Oncoimmunology 2013;2(11):e26329.

http://erj.ersjournals.com/content/48/suppl_60/PA3920



PDPI Malang. 11/04/17.



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