There are no specific recommendations about when to initiate corticosteroid treatment. Data suggest that oral steroids should be used in patients with stages II and III disease, with moderate to severe or progressive symptoms and chest radiograph changes. It is unclear if asymptomatic patients will ever need therapy, even if they have diffuse lung infiltration. Because there is no consensus on the optimal dosage or duration of therapy, the course is individualized for each patient. A recent joint statement of the American Thoracic Society, the European Respiratory Society, and the World Association of Sarcoidosis and Other Granulomatous Disorders 1 included the following guidelines: an initiation dose of prednisone of 20 to 40 mg per day or its equivalent is recommended. Every-otherday dosing may be considered. Patients should be evaluated after one to three months for response. Patients who fail treatment after three months usually will not respond to a more protracted course of treatment. In responders, the prednisone dosage should be tapered to 5 to 10 mg per day or to an every-otherday regimen, and therapy should continue for a minimum of 12 months. 1 There is no consensus guidance on treatment beyond two years. 2 Patients must be monitored after cessation of treatment for possible relapse; some patients will require long-term low-dose therapy to prevent recurrent disease. 1
Two small trials recruiting 23 participants met the inclusion criteria for the review . Participants may have been suffering from comorbid lung disease. No data on oral steroid consumption were reported. No significant differences were observed in the studies for FEV 1 , FVC, PaO 2 and symptoms. One study reported a statistically significant difference in SGaw, but the clinical importance of this is uncertain. Due to concerns over the small sample sizes and methodological shortcomings in terms of inadequate washout in one study , and methods used in outcome assessment for both studies, the findings of the studies are not generalisable to the issue of steroid tapering. An update search conducted in August 2010 did not identify any new studies for consideration in the review .
As with MCD, the hallmark of FSGS is the presence of proteinuria, which can vary in severity from only 1 to 2 g/day to more than 10 g/day. Unlike MCD, at presentation, patients with FSGS are more prone to have concomitant microscopic hematuria (up to 50% of the time), hypertension (33% of the time), and a depressed glomerular filtration rate leading to elevated serum creatinine levels (33% of the time). Typically, patients with heavy proteinuria present clinically with signs and symptoms of the nephrotic syndrome (see earlier). As with MCD, serum complement levels are normal.