The timing of pirfenidone treatment has been studied in animal models, and the effect of pirfenidone seems to be better when treatment is started prophylactically or early after bleomycin treatment [6]. The bleomycin lung fibrosis model in animals is widely used but has limitations. More than 200 drugs have been tried
in the bleomycin mouse model but only pirfenidone have qualified for clinical use. Bleomycin causes inflammation by overproduction of free radicals and induction of pro-inflammatory cytokines and resembles more an acute lung injury in the early phases. The subsequent fibrosis is developed after about 7 GSK-3 beta pathway days and is partly reversible. Thus, the slow and irreversible progressive of fibrosis seen in IPF is not reproduced in the bleomycin animal models, which may explain the disappointing success rate of translating results from the bleomycin model to IPF patients. It is also unclear whether the bleomycin model in rodents can be translated to human bleomycin-induced pneumonitis, although it may intuitively seem more plausible. Pirfenidone administered before or up to 7 days after bleomycin treatment will treat inflammation while pirfenidone administered more than 7 days after bleomycin targets the fibrotic pathways. Pirfenidone Selleckchem Venetoclax has shown beneficial
effects with both scenarios although preventive treatment was the most efficient [6] and [11]. Therefore, the lack of effect on BIP in our patients may be due to the initiation of therapy at a time when overt and fulminant BIP had already developed. Thus, studies of early or Thiamine-diphosphate kinase prophylactic pirfenidone
treatment to clarify the role of pirfenidone in bleomycin-induced pneumonitis and fibrosis in humans are strongly needed. 1. Substantial contributions to conception and design, acquisition of data, or analysis and interpretation of data: EB “
“A 28-year old woman presented to the respiratory clinic with a history of progressive cough and breathlessness over the preceding 2–3 years. Her exercise capacity on initial presentation was consistent with an MRC Dyspnoea score of 4. She also described associated wheeze, chest tightness and a nocturnal cough. The patient smoked 20 cigarettes per day and had a twelve year pack history. She is a college student with three children and pet dog and no relevant employment. She denied any illicit drug use including cannabis. Her other past medical history was that of Hypothyroidism. She was a term delivery at birth. There appeared to be a significant family history of early diagnosis COPD with her mother and maternal grandmother diagnosed with the condition in their thirties. Furthermore one of her sons is an asthmatic. A Chest X-ray (Fig.