Nephritis is a common complication of systemic lupus erythematosus (SLE) for which current therapies often prove inadequate. Current lupus nephritis classification systems emphasize glomerular acuity and scarring. However, tubulointerstitial inflammation (TII) and scarring are much better predictors of progression to renal failure. It is now becoming clear that the immunological features, and probable underlying mechanisms, are very different in lupus glomerulonephritis (GN) and TII at time of biopsy. While GN is a manifestation of systemic autoimmunity, TII is associated with local, in situ adaptive immune cell networks predicted to amplify local inflammation and tissue damage. In addition, poorly defined networks of innate immune cells and effectors likely contribute to the severity of local inflammation. Understanding these in situ immune mechanisms should lead to a better understanding of prognostically meaningful lupus nephritis subsets and reveal novel therapeutic opportunities.
Purpose of review Despite recent developments and treatment successes, the outcome and prognosis of patients with lupus nephritis have not greatly changed since the 1980s. This review covers the application of new concepts to the understanding of renal inflammation and the study of new pharmacologic agents to improve patient outcomes. Recent findings Studies have shown that the presence of anti-vimentin antibodies and T follicular helper cells in patient biopsies are associated with more severe interstitial inflammation, which has been tied to faster disease progression and onset of end stage renal disease. Additionally, data regarding the role of serum IgE anti-dsDNA antibodies in lupus nephritis by means of mediating type I inferferon production by plasmacytoid dendritic cells is highlighted. Finally, a thorough review of completed and currently open clinical trials of therapeutic agents is provided. Summary Current management of lupus nephritis is guided almost exclusively by glomerular involvement. Based on the data provided in this review, we argue that renal tubulointerstitial inflammation is no less important and represents an overlooked feature in the current clinical approach to patients. Tubulointerstitial inflammation is driven by both adaptive and innate immune mechanisms that are still poorly understood. Studying these pathogenic processes promises to reveal new therapeutic opportunities for those lupus nephritis patients with the worse prognosis.
Background Systemic lupus erythematosus is an autoimmune disease that can have cutaneous and systemic manifestations. Lupus panniculitis, also known as lupus mastitis, is a subset of chronic cutaneous lupus erythematosus that involves inflammation of the subcutaneous fat. The pathogenesis of lupus mastitis is not fully understood. Diagnosis involves a combination of skin manifestations, imaging, and pathologic confirmation. Treatment typically includes steroids and antimalarials, with more severe disease requiring additional immunosuppressive medications. This report highlights a case of lupus mastitis treated with rituximab and a possible relationship between this disease process and thrombotic disease. Case presentation A 48-year-old African American female with systemic lupus erythematosus and antiphospholipid syndrome presented with new breast lesion. Mammography revealed calcifications and increased density with coarse trabecular pattern. Breast biopsy showed features of cutaneous lupus and occlusive vasculopathy. The patient was diagnosed with lupus mastitis and treated with anticoagulation, rituximab, mycophenolate mofetil, and quinacrine with resolution of her symptoms. Conclusion This patient experienced improvement in her breast symptoms with combination therapy including rituximab. There are only two other cases reported in literature of patients with lupus mastitis responding to rituximab, highlighting the possible role of B cell depleting therapy for those who have contraindications to standard treatments for lupus mastitis. While the pathophysiology of lupus mastitis is thought to be immune driven, some literature suggests that associated thrombosis commonly seen may be due to a physiologic overlap similar to antiphospholipid syndrome. The possible relationship between antiphospholipid syndrome and lupus mastitis and the use of antiplatelet and anticoagulation therapy is discussed and may warrant further investigation.
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