RESUMEN
Idiopathic inflammatory myopathies (IIM) are rare disorders characterised by the presence of skeletal muscle inflammation, with interstitial lung disease (ILD) being the most frequent pulmonary manifestation. The spectrum of clinical presentations of myositis related ILD (M-ILD) encompasses a chronic process to a rapidly progressive ILD (RP-ILD); which is associated with a high mortality rate. The most effective treatments remain controversial and poses a unique challenge to both rheumatologists and respiratory physicians to manage. Given the rare heterogenous nature of M-ILD, there is a paucity of data to guide treatment. The cornerstone of existing treatments encompasses combinations of immunosuppressive therapies, as well as non-pharmacological therapies. In this review, we aim to summarize the current pharmacological therapies (including its dosing regimens and side effects profiles) and non-pharmacological therapies. Based on the existing literature to date, we propose a treatment algorithm for both chronic M-ILD and RP-ILD.
Asunto(s)
Enfermedades Pulmonares Intersticiales , Miositis , Humanos , Miositis/terapia , Miositis/tratamiento farmacológico , Enfermedades Pulmonares Intersticiales/complicaciones , Enfermedades Pulmonares Intersticiales/diagnóstico , Pulmón , Inflamación/complicaciones , Resultado del Tratamiento , Autoanticuerpos , Estudios RetrospectivosAsunto(s)
Bronquios , Bronquiectasia , Bronquios/diagnóstico por imagen , Bronquiectasia/diagnóstico , Broncoscopía , HumanosAsunto(s)
Factor Estimulante de Colonias de Granulocitos y Macrófagos , Inmunohistoquímica , Proteinosis Alveolar Pulmonar , Tos/etiología , Disnea/etiología , Humanos , Masculino , Persona de Mediana Edad , Proteinosis Alveolar Pulmonar/diagnóstico por imagen , Proteinosis Alveolar Pulmonar/patología , Enfermedades Raras , Tomografía Computarizada por Rayos XRESUMEN
Hypercalcaemia occurs in many granulomatous diseases. Among them, sarcoidosis and tuberculosis are the most common causes. Other causes include berylliosis, coccidioidomycosis, histoplasmosis, Crohn's disease, silicone-induced granulomas, cat-scratch disease, Wegener's granulomatosis and Pneumocystis carinii pneumonia. Hypercalcaemia in granulomatous disease occurs as a consequence of dysregulated production of 1,25-(OH)2 D3 (calcitriol) by activated macrophages in granulomas. Hypercalcaemia in patients with Mycobacterium tuberculosis infection has been reported in 0%-28% of cases. Uncultured bronchoalveolar lavage cells from patients with M. tuberculosis produce greater amounts of calcitriol compared with controls. Although Nayar et al described hypercalcaemia in a case of sepsis associated with intravesical Bacille Calmette Guerin therapy, there are no published reports describing hypercalcaemia in patients with pulmonary M. bovis infection. We describe a patient with M. bovis cavitary pulmonary infection with sustained hypercalcaemia that fluctuated and recurred repeatedly over the course of therapy, ultimately culminating in normalisation of serum calcium when therapy had led to cure. Treatment consisted of antituberculous therapy, oral corticosteroids and intravenous bisphosphonates with a favourable outcome.
Asunto(s)
Calcitriol/sangre , Hipercalcemia/microbiología , Mycobacterium bovis , Tuberculosis Pulmonar/complicaciones , Antituberculosos/uso terapéutico , Humanos , Masculino , Persona de Mediana Edad , Recurrencia , Tuberculosis Pulmonar/sangre , Tuberculosis Pulmonar/microbiologíaRESUMEN
OBJECTIVES: We designed a prospective study to investigate the in-vivo relationship between abdominal body composition and radiation exposure to determine the strongest body composition predictor of dose length product (DLP) at CT. METHODS: Following institutional review board approval, quantitative analysis was performed prospectively on 239 consecutive patients who underwent abdominopelvic CT. DLP, BMI, volumes of abdominal adipose tissue, muscle, bone and solid organs were recorded. RESULTS: All measured body composition parameters correlated positively with DLP. Linear regression (R2 = 0.77) revealed that total adipose volume was the strongest predictor of radiation exposure [B (95% CI) = 0.027(0.024-0.030), t=23.068, p < 0.001]. Stepwise linear regression using DLP as the dependent and BMI and total adipose tissue as independent variables demonstrated that total adipose tissue is more predictive of DLP than BMI [B (95% CI) = 16.045 (11.337-20.752), t=6.681, p < 0.001]. CONCLUSIONS: The volume of adipose tissue was the strongest predictor of radiation exposure in our cohort. MAIN MESSAGE: ⢠Individual body composition variables correlate with DLP at abdominopelvic CT. ⢠Total abdominal adipose tissue is the strongest predictor of radiation exposure. ⢠Muscle volume is also a significant but weaker predictor of DLP.
RESUMEN
A 76-year-old male non-smoker presented to our institution with cough and haemoptysis. He had been treated for cavitatory pulmonary Mycobacterium tuberculosis of the right upper lobe 10 years previously. Chest radiograph and subsequent computed tomography (CT) of the chest demonstrated a right upper cavity containing a mass suspicious for mycetoma. Flexible bronchoscopy under conscious sedation demonstrated a mass obstructing the anterior segment of the right upper lobe. The abnormality was subsequently removed using a flexible endobronchial cryoprobe. Histopathological analysis demonstrated abundant fungal organisms morphologically consistent with Aspergillus species. Microbiological culture of the bronchoalveolar lavage (BAL) from the cavity isolated both Aspergillus fumigatus and Staphylococcus aureus. The patient was commenced on the anti-fungal drug posaconazole and received a course of flucloxacillin. Three months later, there was no endobronchial obstruction and lavage of the affected cavity again isolated Staphylococcus aureus without Aspergillus species. Repeat thoracic CT and flexible bronchoscopy demonstrated no further re-occurrence of the mycetoma at 3 months.