Objective. To generate a core set of items to develop classification criteria for scleroderma renal crisis (SRC) using consensus methodology.Methods. An international, multidisciplinary panel of experts was invited to participate in a 3-round Delphi exercise developed using a survey based on items identified by a scoping review. In round 1, participants were asked to identify omissions and clarify ambiguities regarding the items in the survey. In round 2, participants were asked to rate the validity and feasibility of the items using Likert-type scales ranging from 1 to 9 (where 1 = very invalid/unfeasible, 5 = uncertain, and 9 = very valid/feasible). In round 3, participants reviewed the results and comments from round 2 and were asked to provide final ratings. Items rated as highly valid and feasible (median scores ≥7 for each) in round 3 were selected as the provisional core set of items. A consensus meeting using a nominal group technique was conducted to further reduce the core set of items.Results. Ninety-nine experts from 16 countries participated in the Delphi exercise. Of the 31 items in the survey, consensus was achieved on 13, in the categories hypertension, renal insufficiency, proteinuria, and hemolysis. Eleven experts took part in the nominal group technique discussion, where consensus was achieved in 5 domains: blood pressure, acute kidney injury, microangiopathic hemolytic anemia, target organ dysfunction, and renal histopathology.Conclusion. A core set of items that characterize SRC was identified using consensus methodology. This core set will be used in future data-driven phases of this project to develop classification criteria for SRC.
Wegener granulomatosis (WG) is a systemic disease of unknown etiology characterized by necrotizing granulomatous inflammation, tissue necrosis, and variable degrees of vasculitis in small and medium-sized blood vessels. The classic clinical pattern is a triad involving the upper airways, lungs and kidneys. Ninety percent of patients present with symptoms involving the upper and/or lower airways, and 80% will eventually develop renal disease. WG should be suspected in any patient with progressive or unresponsive sinus disease, glomerulonephritis, pulmonary hemorrhage, mononeuritis multiplex or unexplained multisystem disease. Before the routine use of glucocorticoids and cyclophosphamide, the one year mortality was 82%. However in 1973, Fauci and Wolf discovered that daily prednisone and cyclophosphamide induced complete remission in 75% of patients. The continued use of prednisone and cyclophosphamide for 1 year past remission leads to marked improvement in more than 90% of patients; however, is also associated with serious toxicities. Depending on the disease severity, current treatments employ induction with short-term cyclophosphamide followed by less toxic agents such as methotrexate to maintain disease remission. Although it is a rare disorder, it is pertinent to internists because it is a multisystem disease that presents in a variety of ways. We describe a 63-year-old white male with WG who presented with progressively worsening headaches, bilateral eye redness, epistaxis, hemoptysis and an unintentional 20 pound weight loss, and review the current treatment recommendations.
Pulmonary manifestations of adult-onset Still's disease (AOSD) include aseptic pneumonitis, pleural effusions, rarely acute respiratory distress syndrome, and restrictive lung disease. Pulmonary arterial hypertension (PAH) occurs with several rheumatologic diseases, however, has only been reported once in AOSD. We describe a 29-year-old woman with a 9-year history of AOSD, who developed PAH without any other obvious cause. Therefore, we conclude that this is likely a result of pulmonary vascular changes related to AOSD.
Primary cutaneous infection with Aspergillus species and Rhizopus species was observed in five patients between October 1986 and January 1988. All patients had an underlying hematological malignancy and were neutropenic. Four patients developed skin lesions with eschars at and around the site of intravenous catheter insertion. One patient had two lesions along the course of the intravenous line. The fifth patient developed the lesion on the left side of the lower part of the back subsequent to the appearance of a rash due to excessive perspiration. A diagnosis was made in each case by histopathological studies and cultures of skin biopsy specimens. The fungal organisms cultured were Aspergillus species in three patients and Rhizopus species in two patients. All patients were treated with amphotericin B. Three patients responded to antifungal therapy and local care without surgical débridgement. Two patients died of disseminated fungal infection. The development of cutaneous lesions due to opportunistic fungal pathogens seemed to have been related to the moist and humid conditions created by occlusive dressings or excessive perspiration.
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