Targeted therapies in interstitial lung disease secondary to systemic autoimmune rheumatic disease. Current status and future development.

Autoimmune rheumatic diseases (ARD) are characterized by systemic manifestations and multiple organ involvement, including the lung. Interstitial Lung Disease (ILD) is a cardinal manifestation of lung involvement in patients with ARD and is associated with significant morbidity and mortality. Corticosteroids and immunosuppressive drugs are used as first -line treatment. Targeted therapies, such as biological disease modifying antirheumatic drugs (DMARDS) and anti- fibrotic agents are new treatment options. In this review we discuss the role of targeted therapies in patients with ILD secondary to ARD.

Acute pulmonary exacerbations of sarcoidosis.

Exacerbations of sarcoidosis are common. In particular, exacerbations of pulmonary sarcoidosis are reported in more than one-third of patients. Despite their frequent occurrence, there is little medical evidence concerning the definition, diagnosis, and treatment of pulmonary exacerbations of sarcoidosis. In this article, we propose a definition of acute pulmonary exacerbations of sarcoidosis (APES). We review the meager medical literature concerning the risk factors, diagnosis, and treatment of this condition. Given the limited information concerning APES, we acknowledge that this article is not a definitive resource but, rather, a position paper that will encourage greater consideration of the pathogenesis, diagnostic challenges, and treatment approaches to this condition. We believe that further focus on APES will improve the quality of care of patients with pulmonary sarcoidosis.

Clinical manifestations, radiographic findings, treatment options, and outcome in sarcoidosis patients with upper respiratory tract involvement.

BACKGROUND: Sarcoidosis of the upper respiratory tract (SURT) is an underappreciated manifestation of sarcoidosis. We described the clinicoradiological findings, methods of treatment, and outcomes of 68 patients with SURT seen in our clinic over a 7-year period. METHODS: This was a retrospective study. A Case Control Etiology of Sarcoidosis Study (ACCESS) instrument of organ involvement was used to classify patients with SURT. Patients with a definite or probable diagnosis of SURT underwent chart review for symptoms, radiological features, treatment, and outcome. RESULTS: There were 6.8% (68/998) of patients in the sarcoidosis clinic who had SURT. The most common presenting symptom was nasal congestion (61%, 34/56). In patients who had SURT diagnosed after the initial presentation of sarcoidosis, the mean prednisone dose more than quadrupled (4 + or - 6.5 vs 18.6 + or - 15.9, P = 0.002) between the diagnosis of SURT and the end of follow up. By the end of follow up, only 40% of our cohort had significant improvement of SURT. CONCLUSION: SURT is a form of sarcoidosis that is not uncommon. Relatively high doses of corticosteroids are required to control the disease, and improvement occurs in a minority of the patients although the disease usually can be stabilized. Surgery should be reserved for life-threatening complications as surgical outcomes are relatively poor.

Clinical outcomes in sarcoidosis after cessation of infliximab treatment.

BACKGROUND AND OBJECTIVE: Infliximab appears to be efficacious for the treatment of recalcitrant forms of sarcoidosis. However, there are minimal data concerning the course of sarcoidosis once infliximab is discontinued. METHODS: Clinical outcomes in patients who had received infliximab and had discontinued it for at least 2 months were analysed retrospectively. The severity of involvement of the index organ from the time of discontinuation of infliximab was compared with that at the end of the follow-up period. RESULTS: Fourteen patients with sarcoidosis who had been treated with infliximab and had discontinued this therapy were identified. Before discontinuation of infliximab, 9 of the 14 patients (64%) responded to infliximab treatment and only one (7%) deteriorated. Patients who discontinued infliximab were followed for a mean of 12 months. At the end of the follow-up period, 12 of the 14 patients (86%) had deteriorated as compared with their status at the time of discontinuation of infliximab and two (14%) had remained stable. Kaplan-Meier analysis of time to clinical deterioration showed that half the patients deteriorated within 3 months of discontinuing infliximab. Patients who had discontinued infliximab appeared to be more likely to have their dose of prednisone increased. CONCLUSION: Patients with recalcitrant sarcoidosis who receive infliximab appear likely to deteriorate after discontinuation of this medication.

Staple metalloptysis after lung resection for mycetoma: a case report and a review of the literature.

The expectoration of metal fragments (metalloptysis) is a rare complication after thoracic surgery. It usually occurs in patients with severe emphysema. We present a case of metalloptysis of surgical staples that is unique in several aspects: it occurred longer after surgery than any previously reported case, it occurred after lobar resection surgery, and it most likely developed as a result of chronic necrotizing aspergillosis. Although metalloptysis usually requires no therapy, it may indicate an active infectious process that requires appropriate therapy.