Shruthi Konda1, Betty Cheung2, Lola
Loewenthal1 and Raminder Aul1
1Respiratory Medicine, St. George's
University Hospital, London,
United Kingdom,?2Haematology, Croydon University Hospital,
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
present a case of a 38-year-old lady with Pure Red Cell Aplasia (PRCA)
and refractory OP.
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
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.
1. Asano et al,SpringerPlus 2014,3:3. 2.Yang L et al,Oncoimmunology