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The full British Thoracic Society (BTS) guideline for the use of long-term macrolides in adults with respiratory disease is published in Thorax. The following is a summary of the recommendations and good practice points. The sections referred to in the summary refer to the full guideline. The appendices are available in the full guideline and online appendices are available on the BTS website. This is the first BTS guideline to use the Grading of Recommendations, Assessment, Development and Evaluations (GRADE) approach as part of the process of guideline development and the guideline was used to pilot the new methodology.

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BackgroundNon-Tuberculous Mycobacterial–pulmonary disease (NTM-PD) is increasing in incidence and prevalence. Mycobacterium abscessus (M.abscessus) is a rapid growing multi-resistant NTM associated with severe NTM-PD requiring prolonged antibiotic therapy. Complications of therapy are common but reports on direct complications of active NTM-PD are rare. Vasculitis has been described as a rare complication of NTM-PD, most often in individuals with inherited immune defects. This case is the first to describe an ANCA positive vasculitide (Microscopic Polyangiitis) secondary to M.abscessus pulmonary disease.Case presentationA 70 year old female with bronchiectasis underwent a clinical decline associated with the growth of M.abscessus and was diagnosed with NTM-PD. Before treatment could be initiated she developed small joint arthralgia and a glove and stocking axonal loss sensorimotor neuropathy. Positive Perinuclear Anti-Neutrophil Cytoplasmic Antibodies (P-ANCA) and Myeloperoxidase-ANCA (MPO-ANCA) titres led to a diagnosis of microscopic polyangiitis. Further investigation revealed reduced interferon-gamma production but no other significant immune dysfunction.Dual treatment with immunosuppressive therapy (Corticosteroids/Cyclophosphamide) for vasculitis and antimicrobial therapy for M.abscessus NTM-PD was initiated. Clinical stability was difficult to achieve with reductions in immunosuppression triggering vasculitic flares. One flare led to retinal vein occlusion with impending visual loss requiring escalation in immunosuppression to Rituximab infusions. An increase in immunosuppression led to a deterioration in NTM-PD necessitating alterations to antibiotic regimes. Adverse effects including alopecia and Achilles tendonitis have further limited antibiotic choices resulting in a strategy of pulsed intra-venous therapy to stabilise NTM-PD.ConclusionsThis is the first reported case of an ANCA positive vasculitis secondary to M.abscessus pulmonary disease. This rare but important complication had a significant impact on the patient adding to the complexity of an already significant disease and treatment burden. The potential role of reduced interferon-gamma production in this case highlights the importance of investigating immune function in those with mycobacterial infection and the intricate relationship between mycobacterial infection and immune dysfunction. Immune dysfunction caused by genetic defects or immunosuppressive therapy is a known risk factor for NTM-PD. Balancing immunosuppressive therapy with prolonged antimicrobial treatment is challenging and likely to become more common as the number of individuals being treated with biologics and immunosuppressive agents increases.

6. Xu X, Li B, Wang L. Gender difference in smoking effects on adult pulmonary function. Eur Respir J 1994;7:477e83. 7

The microbial landscape of CF is changing, reflecting advances in microbial detection, CF therapies and an increasingly heterogeneous and ageing population. Gram negative organisms are important to clinical trajectory, however, some have unknown implications on disease course. Areas covered This review covers the evolving landscape of the microbial ecosystem of the CF lung and provides an update on current diagnostic and therapeutic options for management of Gram negative bacteria. Evidence for prevention of acquisition of new organisms, and eradication of Gram negative pathogens is reviewed. There is an increasing range of inhaled antibiotic therapies for chronic suppressive antimicrobial therapy, with an urgent need for research into the efficacy of specific combinations. Intravenous therapy for pulmonary exacerbations requires optimisation, focusing on greater precision, improved clinical outcomes, whilst reducing anti-microbial resistance and longterm side effects. The future role of CFTR modulators, anti-inflammatory agents and novel antiinfectives is also outlined. Expert opinion Antimicrobial therapy must evolve to reflect the evolving microbial landscape and the needs of current and future CF populations. With an increasing number of Gram negative organisms, detection methods and therapeutic options, it is critical therapy is targeted appropriately to the organism and the individual.