Association of discoid lupus erythematosus with other clinical manifestations among patients with systemic lupus erythematosus.

BACKGROUND: Cutaneous discoid lupus erythematosus (DLE) among patients with systemic lupus erythematosus (SLE) may be associated with less severe disease and with low frequency of nephritis and end-stage renal disease (ESRD). OBJECTIVE: We sought to investigate associations between confirmed DLE and other SLE manifestations, adjusting for confounders. METHODS: We identified patients with rheumatologist confirmation, according to 1997 American College of Rheumatology (ACR) SLE classification criteria, more than 2 visits, longer than 3 months of follow-up, and documented year of SLE diagnosis. DLE was confirmed by a dermatologist, supported by histopathology and images. SLE manifestations, medications, and serologies were collected. Multivariable-adjusted logistic regression analyses tested for associations between DLE and each of the ACR SLE criteria, and ESRD. RESULTS: A total of 1043 patients with SLE (117 with DLE and 926 without DLE) were included in the study. After multivariable adjustment, DLE in SLE was significantly associated with photosensitivity (odds ratio [OR] 1.63), leukopenia (OR 1.55), and anti-Smith antibodies (OR 2.41). DLE was significantly associated with reduced risks of arthritis (OR 0.49) and pleuritis (OR 0.56). We found no significant associations between DLE and nephritis or ESRD. LIMITATIONS: Cross-sectional data collection with risk of data not captured from visits outside system was a limitation. CONCLUSIONS: In our SLE cohort, DLE was confirmed by a dermatologist and we adjusted for possible confounding by medication use, in particular hydroxychloroquine. We found increased risks of photosensitivity, leukopenia, and anti-Smith antibodies and decreased risks of pleuritis and arthritis in patients with SLE and DLE. DLE was not related to anti-double-stranded DNA antibodies, lupus nephritis, or ESRD. These findings have implications for prognosis among patients with SLE.

Is chronic cutaneous discoid lupus protective against severe renal disease in patients with systemic lupus erythematosus?

OBJECTIVE: The aim was to assess the level of systemic involvement and character of renal disease in patients with chronic cutaneous lupus erythematosus of the discoid lupus variety (hereafter referred to as 'discoid lupus') and features of systemic lupus erythematosus (SLE). Clinical confusion with other types of cutaneous lupus erythematosus complicates interpretation of some previously reported studies. METHODS: Over three years, sixteen patients met the diagnostic criteria of discoid lupus, positive anti-nuclear-antibody, and at least one extracutaneous manifestation. RESULTS: Most patients (14/16) were female, between 26 to 66 years old. Arthritis was the most common extracutaneous manifestation followed by Raynaud's phenomenon. The anti-nuclear-antibody was speckled in ten patients with titers ranging from 1:40 to 1:1280 IU/mL. Elevated levels of double-stranded-DNA in low titers were found in four patients, anti-Smith-antibody in four; anti-Sjogren-syndrome-A-antibody in seven, and anti-ribonucleoprotein-antibody in seven. Renal function markers were transiently high in some patients but normalized over time. Hematuria and/or proteinuria were present at some time in seven patients. The highest BUN and creatinine levels were 42 mg/dL and 1.5 mg/dL, respectively. One patient had membranous glomerulonephropathy class 5; however, discoid lupus developed well after the onset of renal disease during a time when renal function had returned to normal. CONCLUSION: Our observational data supports previous reports suggesting that patients with active discoid lupus rarely have progressive renal insufficiency. The mechanism for the development of discoid lupus may involve an immunologic mechanism that differs from that which produces severe organ involvement, especially advanced immune-complex-mediated renal disease. Patients with discoid lupus rarely have sustained high levels of antibodies to double-stranded-DNA. Discoid lupus appears to be a marker for a more benign lupus course. This clinical observation lays the groundwork for a larger prospective, longitudinal cohort study for further validation.

Dendritic cells in the pathogenesis of sarcoidosis.

Sarcoidosis is a noncaseating granulomatous disease, likely of autoimmune etiology, that causes inflammation and tissue damage in multiple organs, most commonly the lung, but also skin, and lymph nodes. Reduced dendritic cell (DC) function in sarcoidosis peripheral blood compared with peripheral blood from control subjects suggests that blunted end organ cellular immunity may contribute to sarcoidosis pathogenesis. Successful treatment of sarcoidosis with tumor necrosis factor (TNF) inhibitors, which modulate DC maturation and migration, has also been reported. Together, these observations suggest that DCs may be important mediators of sarcoidosis immunology. This review focuses on the phenotype and function of DCs in the lung, skin, blood, and lymph node of patients with sarcoidosis. We conclude that DCs in end organs are phenotypically and functionally immature (anergic), while DCs in the lymph node are mature and polarize pathogenic Th1 T cells. The success of TNF inhibitors is thus likely secondary to inhibition of DC-mediated Th1 polarization in the lymph node.

Systemic lupus erythematosus with generalized chronic cutaneous (discoid) lupus erythematosus.

A 24-year-old pregnant African-American woman had a 3-4 year history of chronic, scarring, hyperpigmented plaques on her scalp, face, trunk, and extremities. She complained of joint pain and fatigue. Clinical presentation, laboratory data, and histopathologic features were consistent with systemic lupus erythematosus in a patient with generalized chronic discoid lupus erythematosus. This subtype is a distinct lupus erythematosus subset that rarely develops renal disease and has a relatively benign but chronic course.