Aspirin-sensitive asthma is a common and often underdiagnosed disease affecting up to 20% of the adult asthmatic population. It is associated with more severe asthma, requires increased use of inhaled and oral corticosteroids, more presentations to hospital and a risk of life-threatening reactions with aspirin/non-steroid anti-inflammatory drug (NSAID) ingestion. Aspirin-sensitive asthma is often accompanied by severe rhinosinusitis and recurrent nasal polyposis, causing significant impairment of patients' quality of life. The pathogenesis of aspirin-sensitive asthma is complex and involves chronic eosinophilic inflammatory changes, with evidence of increased mast cell activation. The cyclo-oxygenase pathways play a major role in the respiratory reactions that develop after aspirin ingestion. The cysteinyl-leukotrienes have also been shown to play a role in the pathogenesis of aspirin-sensitive asthma. The clinical management of aspirin-sensitive asthma is complicated by the lack of diagnostic testing, other than challenge procedures. Other aspects of management include management of the underlying asthma and avoidance of NSAID in the majority of patients. Other considerations in the management of patients with aspirin-sensitive asthma include the role of leukotriene modifying agents, aspirin desensitization, and the use of other agents, such as roxithromycin. The management of nasal polyposis in patients with aspirin-sensitive asthma often needs to be considered as a separate issue, and requires a team approach.
Granulomatous lymphocytic interstitial lung disease (GLILD) is characterized by lymphocytic and granulomatous pulmonary infiltration occurring in common variable immunodeficiency (CVID). It is associated with increased mortality compared with CVID patients without GLILD. There are no treatment guidelines due to the low prevalence and the heterogeneity of the condition. A case review of three patients diagnosed with GLILD was performed from a single Australian centre. Patients met the European Society of Immunodeficiency criteria for CVID and a diagnosis of GLILD was confirmed by a multidisciplinary team. Patients were managed with immunoglobulin (Ig) replacement and immunosuppressive agents if required: the decision for immunosuppression was made on the basis of symptoms and declining pulmonary function. All patients clinically improved. One patient had immunosuppressive treatment ceased. GLILD responds to varying immunosuppressive regimes when IgG monotherapy fails. Immunosuppressive therapy can be discontinued following improvement, but patients require close observation. This series helps inform future GLILD treatment guidelines.
Aim
To evaluate myositis line immunoassay (LIA) for diagnosis and sub‐classification of suspected idiopathic inflammatory myopathy (IIM). To investigate if test performance is improved by increasing signal strength cut‐off for myositis‐specific antibody (MSA) or combining MSA with indirect immunofluorescence (IIF).
Methods
A retrospective, consecutive case series of patients investigated for MSAs from June 2013 to June 2020 for suspected IIM. Specificity, sensitivity, positive predictive value, and negative predictive value were calculated with 95% confidence intervals for diagnosis of IIM. Association of IIM diagnosis with increased signal strength and presence of an expected IIF pattern on Hep‐2 cells was assessed by Fisher’s exact test in MSA‐positive patients.
Results
A total of 195 patients were evaluated. IIM was diagnosed in 32/195 (16.4%) patients. MSAs were detected in 41/195 (21%) patients, 18/41 (43.9%) patients with an MSA had a diagnosis of IIM. The probability of an IIM diagnosis was increased in MSA‐positive patients with high compared with low signal strength (83.3% vs 43.5%; P = 0.01) and an expected compared with unexpected IIF pattern (61.5% vs 23.8%; P = 0.04). Specificity for IIM was not significantly improved by increasing signal strength cut‐off (85.9% vs 93.8%). Positive predictive value of myositis LIA was only modest and not significantly improved by either increasing signal strength cut‐off or requiring an expected IIF pattern for determination of MSA positivity (43.9% vs 60% vs 61.5%). Sub‐classification of IIM correlated closely for respective MSAs (88.9%).
Conclusion
Increased MSA signal strength on myositis LIA and the presence of an expected IIF pattern were associated with IIM diagnosis. Test performance was non‐significantly improved by these methods. Prevalence of IIM in this patient cohort was low; it is not excluded that LIA performance could be improved by these methods in a higher prevalence cohort.
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