Lupus
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Pancreatitis is a relatively rare complication in systemic lupus erythematosus (SLE). Here we present a case of SLE associated with autoimmune pancreatitis. A 37-year old woman was admitted to our hospital because of fever, skin rash, proteinuria and abdominal pain. ⋯ Systemic administration of glucocorticoid successfully induced remission of pancreatitis and nephritis along with the reduction of IgG4. Autoimmune pancreatitis is a newly recognized type of pancreatitis, in which IgG4 immune response is thought to participate pathophysiologically. Although the disease has been observed to develop in patients having various connective tissue diseases, our report is the first to describe IgG4-related autoimmune pancreatitis in a patient with SLE.
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Necrotizing fasciitis is an uncommon but life-threatening complication in immunocompromised hosts. We reported four patients with rheumatic diseases complicated by necrotizing fasciitis and reviewed 14 others from literature search. Most patients were on corticosteroid treatment. ⋯ Septic arthritis may also complicate the condition. Statistical analysis on the series showed the lack of major surgical debridement as the only risk factor associated with increased mortality (RR 7.5, 95% CI 2.1-27.3, P = 0.01). Timely debridement of necrotic tissue is important for reducing mortality.
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The number of publications in the field of pulmonary hypertension has increased exponentially over the past 10 years. As a consequence our understanding of the condition has advanced enormously. Pulmonary hypertension is now recognised as an uncommon and treatable disease, rather than a rare lethal disease. ⋯ Identifying patients with very early disease and analysing the drivers for disease progression, is the key to further progress. Early diagnosis is most readily achieved in patients with connective tissue disease associated pulmonary hypertension. This represents an opportunity for those with an interest in academic rheumatology.
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Autoimmune rheumatic diseases (AIRD) are not uncommon in the general population and up to one third of hospitalized patients with AIRD may need admission to intensive care unit (ICU). This paper describes the causes of admission, the clinical features and outcome of 24 AIRD patients admitted to a medical ICU from a third level hospital. Thirteen patients had systemic lupus erythematosus (54.2%), three rheumatoid arthritis (12.5%), three pulmonary renal syndrome (12.5%), two dermatopolymyositis (8.3%), two scleroderma (8.3%) and one antiphospholipid syndrome (4.2%). ⋯ Excluding shock requiring vasopressor support, no statistical difference was found between survivors and nonsurvivors; although there was a trend to higher test severity scores (APACHE II, ODIN) in nonsurvivors. Our results reveal a lower mortality rate in AIRD patients admitted to the ICU than reported previously. Severity scores such as APACHE II are predictors of mortality in patients with AIRD in the ICU.
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Randomized Controlled Trial Clinical Trial
Maintenance therapies for proliferative lupus nephritis: mycophenolate mofetil, azathioprine and intravenous cyclophosphamide.
For the treatment of proliferative lupus nephritis, long-term cyclophosphamide (CY) regimens are efficacious, however, at the expense of substantial toxicity. In the last decade, sequential regimens of short-term CY induction followed by either mycophenolate mofetil (MMF) or azathioprine (AZA) maintenance have shown to be efficacious and safe reducing the long-term exposure to CY. In a maintenance study including predominantly Hispanics and African-Americans, the patients who received MMF and AZA maintenance had a higher cumulative probability of remaining free of the composite of death or chronic renal failure (CRF) compared to quarterly intravenous CY (IVCY) maintenance (89% in MMF, 80%, in AZA and 45% in IVCY). ⋯ The incidence of permanent amenorrhea and infection were 8% and 33%, respectively. None of the Asian patients had an increase in serum creatinine level to double the baseline value. Maintenance therapies with MMF or AZA following short-term CY induction in a sequential regimen are efficacious and safe for the treatment of high-risk patients with proliferative lupus nephritis.