Two challenging cases of anti-MDA-5 dermatomyositis with rapidly progressive interstitial lung disease.

Anti-MDA-5 dermatomyositis (DM) is a subtype of idiopathic inflammatory myopathy, commonly presenting as clinically amyopathic dermatomyositis. It is associated with rapidly progressive interstitial lung disease and a poor prognosis. Here, we present two cases of anti-MDA-5 DM and discuss the challenges associated with timely diagnosis, and the importance of early and aggressive treatment.

Non-invasive ventilation (NIV) as an aid to rehabilitation in acute respiratory disease.

BACKGROUND: Non-invasive ventilation (NIV) can increase exercise tolerance, reduce exercise induced desaturation and improve the outcome of pulmonary rehabilitation in patients with chronic respiratory disease. It is not known whether it can be applied to increase exercise capacity in patients admitted with non-hypercapnic acute exacerbations of COPD (AECOPD). We investigated the acceptability and feasibility of using NIV for this purpose. METHODS: On a single occasion, patients admitted with an acute exacerbation of chronic respiratory disease who were unable to cycle for five minutes at 20 watts attempted to cycle using NIV and their endurance time (T(lim)) was recorded. To determine feasibility of this approach in clinical practice patients admitted with AECOPD were screened for participation in a trial of regular NIV assisted rehabilitation during their hospital admission. RESULTS: In 12 patients tested on a single occasion NIV increased T(lim) from 184(65) seconds to 331(229) seconds (p = 0.04) and patients desaturated less (median difference = 3.5%, p = 0.029). In the second study, 60 patients were admitted to hospital during a three month period of whom only 18(30)% were eligible to participate and of these patients, only four (7%) consented to participate. CONCLUSION: NIV improves exercise tolerance in patients with acute exacerbations of chronic respiratory disease but the applicability of this approach in routine clinical practice may be limited. TRIAL REGISTRATION: http://www.controlled-trials.com/ISRCTN35692743.

Chronic Pneumocystis jiroveci presenting as asymptomatic granulomatous pulmonary nodules in lymphoma.

We present a rare case of chronic Pneumocystis jiroveci infection presenting as multiple persistent granulomatous pulmonary nodules over a 12 month period in a patient with follicular lymphoma undergoing treatment with Rituximab, Cyclophosphamide, Vincristine, and Prednisolone chemotherapy. Remarkably during this period the patient remained asymptomatic. This is the first case to combine these atypical histological, radiological, and clinical manifestations of P. jiroveci infection highlighting unusual manifestations of the disease.