Reliability of C-reactive protein as an inflammatory marker in patients with immune-mediated inflammatory diseases and liver dysfunction.

Objectives: CRP is an acute-phase reactant widely used clinically as a marker of inflammation. CRP is a protein synthesized by hepatocytes. Previous studies have shown lower CRP levels in response to infections in patients with chronic liver disease. We hypothesized that CRP levels would also be lower during active immune-mediated inflammatory diseases (IMIDs) in patients with liver dysfunction. Methods: This retrospective cohort study used Slicer Dicer in Epic, our electronic medical record system, to search for patients with IMIDs both with and without concomitant liver disease. Patients with liver disease were excluded if there was no clear documentation of liver disease staging. Patients were also excluded if a CRP level was not available during disease flare or active disease. Arbitrarily, we considered normal CRP as ≤0.7 mg/dl, mild elevation of CRP as ≥0.8 and <3mg/dl, and elevated CRP as ≥3mg/dl. Results: We identified 68 patients with both liver disease and IMIDs (RA, PsA and PMR) and 296 patients with autoimmune disease and without liver disease. Presence of liver disease had the lowest odds ratio (odds ratio = 0.25, P < 0.0001) of having an elevated CRP during flare. Each specific IMID, except SLE and IBD, had higher median CRP levels during active disease episodes in patients without liver disease than in those with liver disease. Discussion: Overall, IMID patients with liver disease had lower serum CRP levels during active disease than their counterparts without liver dysfunction. This observation has implications for clinical use of CRP level as a reliable marker of disease activity in patients with IMIDs and liver dysfunction.

Association of hidradenitis suppurativa with autoimmune disease and autoantibodies.

Objective: There is thought to be an association between hidradenitis suppurativa (HS) and autoimmune diseases. This retrospective longitudinal cohort study looked to identify whether certain autoimmune diseases or autoantibody specificities are more closely associated with HS than others and whether such associations are related to the severity of HS. Methods: Patients were identified using the SlicerDicer search tool in Epic from 1 January 2010 to 15 August 2020. Search criteria included HS diagnosis by ICD-10 code (L73.2) and at least one visit to the dermatology department. Charts were reviewed to determine HS disease severity, treatment modalities, presence of autoimmune disease and autoantibody positivity. Results: Six hundred and twenty-seven patients were identified. Most patients were female (75.3%) and had obese BMIs (71.1%), but there were no significant demographic differences between HS patients with and without autoimmune diseases. One hundred and one (16.1%) patients in the total cohort had at least one autoimmune disease, most commonly thyroid disease, lupus, psoriasis and IBD. Two hundred and twelve patients were also tested for the presence of autoantibodies. The most common positive autoantibody, found in 54 patients (28.4%), was ANA. Fifty-four patients with more severe HS disease manifestations required biologic medications to treat their HS. Neither HS severity nor biologic treatment was associated with presence of autoimmune disease or positive autoantibodies. Conclusion: In a large cohort of patients with HS followed longitudinally, autoimmune disorders (especially lupus, psoriasis and IBD) and presence of autoantibodies were more commonly observed than expected in the normal population.

Incidence of blindness in a population of rheumatic patients treated with hydroxychloroquine.

OBJECTIVE: Long-term HCQ use for the treatment of rheumatic diseases has been associated with retinopathy in a daily and cumulative dose-dependent manner by weight. We examined the incidence of ocular toxicity in a large population of patients treated with HCQ for inflammatory arthritis and SLE and followed long term in a tertiary centre. METHODS: Our retrospective longitudinal review identified 2867 rheumatic patients from 1999 to August 2017 who had a prescription written for HCQ. Thirty-one patients were identified as having a diagnosis of blindness or toxic maculopathy in their electronic medical record, and we carried out an extensive chart review. RESULTS: Of our 31 patients with a diagnosis of blindness or toxic maculopathy, 11 had documented blindness, in all cases attributed to a cause other than HCQ-related ocular toxicity: stroke (27%), pre-existing macular disease (18%), diabetic retinopathy (18%), hypertensive retinopathy (9%) and cataracts (9%). Seventeen of 31 patients had visual impairment that was multifactorial and unrelated to HCQ. We identified two patients with bull's eye maculopathy [person-time incidence rate, 0.12 cases per 1000 person-years (95% CI: 0.01, 0.43)] and one with early HCQ toxic maculopathy [person-time incidence rate, 0.06 cases per 1000 person-years (95% CI: 0.002, 0.33)]. All three patients received HCQ for >18 years, and none had functional vision loss at diagnosis. CONCLUSION: HCQ-induced macular toxicity is rare in routine clinical practice, seems to require prolonged HCQ therapy (>18 years) and is not necessarily associated with functional visual loss. Our findings suggest that co-morbid conditions that are common in RA and SLE contribute substantially to vision loss and should not be ignored.

Triple Valvulopathy and Jaccoud's Arthropathy: A Case Report and Literature Review.

Cardiac involvement is fairly common in patients with systemic lupus erythematosus (SLE). It may involve all layers of the heart and coronary arteries as well as the heart valves. We report an extremely rare presentation of valvulitis and valvular dysfunction associated with systemic lupus erythematosus. This is the first case of lupus valvulitis which required three mechanical prosthetic valve replacements with disease recurrence leading to a fatal outcome. This is, in our point of view, the consequence of aggressive natural history of the disease and perhaps late diagnosis and treatment of underlying SLE which was unsuccessful.

Red cell distribution width: a measure of cardiovascular risk in rheumatoid arthritis patients?

This study aims to evaluate if myocardial infarction (MI) is more frequent in rheumatoid arthritis (RA) patients with elevated levels of red cell distribution width (RDW). Utilizing a secure cloud based platform, Explorys, we searched de-identified US patient data between 1999 and 2014. RA patients were identified by serologic positivity and ICD9 diagnosis code. Patients were stratified into high (≥15.6 %) RDW and low (<13.5 %) RDW groups (and excluding any patient with prior episode of RDW >15.6 %). The proportion of patients with diagnosis of MI in each RDW group was collected. For comparison, patients were divided into high and low CRP groups (≥2.5 and ≤0.8 mg/dL) and high and low ESR groups (≥50 and ≤30 mm/h), and MI data were collected. Statistical comparison between high and low laboratory test groups was performed with chi-square test, and odds ratios were calculated. The patient population included 20,810 patients with RA. The proportion of RA patients with MI was significantly increased in the high compared to low RDW, ESR, and CRP groups (p < 0.001 for each). The odds ratios of MI were greater in the high than in the low group for each parameter: RDW (OR1.5, 95 % CI 1.3 to 1.6); ESR (OR2.0, 95 % CI 1.8-2.3); and CRP (OR1.9, 95 % CI 1.7 to 2.2). These data from a large unselected population suggest that elevated RDW levels in RA patients should prompt physicians to aggressively screen and treat their patients for modifiable cardiovascular (CVS) risk factors, in addition to treating RA inflammation.

The effect of antiestrogen agents on risk of autoimmune disorders in patients with breast cancer.

OBJECTIVE: To investigate the relationship between antiestrogen therapy in women with breast cancer and risk of autoimmune disease. METHODS: We used a national database to assess the incidence of systemic lupus erythematosus (SLE) and rheumatoid arthritis (RA) following treatment with selective estrogen receptor modulators (SERM) or aromatase inhibitors (AI) in women with breast cancer. The total number of patients in our study was 190,620. RESULTS: We observed highly significant, cumulative dose-dependent increased OR of incidence of both SLE and RA following treatment with SERM (p < 0.0001). The odds of developing RA were also increased following AI therapy (p < 0.001), but there was a trend for reduced odds of SLE, though this trend did not attain statistical significance (p = 0.070 for 2-11 months of treatment and p = 0.254 for 12+ months of treatment). CONCLUSION: Antiestrogen agents may have an important effect on risk of autoimmune disease.

The effectiveness and safety of mycophenolate mofetil in lupus nephritis.

The purpose of this study is to evaluate the effectiveness and safety of mycophenolate mofetil (MMF) for inducing and/or maintaining remission of lupus nephritis (LN). This is a retrospective study of 25 LN patients consecutively treated with MMF. The primary outcome was complete renal remission (CR) defined by urine protein/creatinine ratio < or =0.5 g/g and inactive urine sediment and serum creatinine within <15% above baseline. For induction, 21 episodes of active, moderate to severe LN were treated with MMF. Twelve cases (57%) achieved CR over a median of 8.5 months. Of 13 patients who had LN for <12 months and took > or =2 g/day of MMF, 11 achieved CR, compared to one out of the eight patients who did not meet both criteria (p = 0.0022). For maintenance therapy, 15 patients received MMF for a median of 20 months (range 5-55 months). Two patients (13%) experienced renal flares while taking MMF. Most adverse events were transient and did not require change in therapy. This study suggests that MMF is an effective treatment for both induction and maintenance of remission of moderate to severe LN with a relatively favorable safety profile. Early treatment and a dose > or =2 g/day are essential for optimal outcome. CR may take >6 months.

Cardiovascular disease risk awareness in systemic lupus erythematosus patients.

OBJECTIVE: Cardiovascular disease (CVD) is a major cause of morbidity and mortality in systemic lupus erythematosus (SLE) patients. The aim of this study was to identify factors associated with patients' recognition of SLE as an independent risk factor for CVD and their perception of their personal CVD risk. METHODS: SLE patients were sent questionnaires that assessed demographic characteristics, any CVD risk factors, and information regarding the CVD counseling they had received from their physicians. Variables significantly associated with the outcomes were analyzed using logistic regression models. RESULTS: Information obtained from 226 questionnaires was analyzed. Fifty-eight percent of the respondents reported receiving no CVD counseling from a physician. Patients who recalled receiving counseling from a physician were 2.3 times more likely (P = 0.02) to recognize SLE as a CVD risk factor and 3.2 times more likely (P = 0.02) to perceive themselves as being at risk of CVD compared with those patients who did not receive physician counseling. Receiving physician CVD counseling was the strongest predictor of a patient's self-perception of CVD risk. Those patients at intermediate to high risk (n = 167) who reported having received counseling were 5.3 times as likely (P = 0.007) to perceive themselves to be at higher risk of CVD compared with similar patients who did not receive counseling. Younger patients were 4.2 times as likely (P = 0.002) as older patients to recognize SLE as a CVD risk factor. Other variables associated with patients' self-perceptions of CVD risk included family history of CVD and hypertension. CONCLUSION: Physician counseling regarding CVD in SLE patients has an important impact on patients' awareness of SLE as a CVD risk factor and their self-perception of CVD risk.