A Rare Presentation of Myositis and Diffuse Alveolar Hemorrhage Associated With Disseminated Cryptococcus neoformans Infection.

Cryptococcosis is a fungal infection caused by species of the Cryptococcus genus which are commonly found in soil contaminated with bird feces, decaying wood, and tree hollows. It is usually seen in immunocompromised patients such as those with AIDS, with hematological malignancy, on immunosuppressive therapy, or after organ transplantation, and rare in immunocompetent hosts. The primary site of infection is usually the lung and the infection starts after inhalation of the pathogen and depending upon the host's immune response shows a different pattern of infection. Here we present a case report of a female in her late forties, who presented with two weeks of rash in her bilateral upper extremity, lower extremity, chest, and back along with arthralgia, myalgia, and proximal lower extremity weakness. Initial laboratory workup showed leukocytosis, elevated erythrocyte sedimentation rate, C-reactive protein, serum ferritin, and serum aldolase level with normal creatinine kinase. Rheumatological workups including ANA, ANCA, RF, C3, and C4 were normal. Magnetic resonance imaging of the right femur showed hyperintensity of the thigh and proximal calf musculature suggestive of muscle edema. A punch biopsy from the rash showed dyskeratosis with mild perivascular neutrophilic infiltrate. Steroid therapy and rituximab were started with some improvement. However, the patient developed respiratory distress and diffuse alveolar hemorrhage. Bronchoscopy was done and bronchoalveolar lavage fluid grew Serratia and Candida. The patient improved with antibiotic and antifungal therapy. However, the patient again developed respiratory distress and a new diffuse alveolar hemorrhage. A repeat bronchoscopy was done and the new bronchoalveolar lavage grew Cryptococcus neoformans. Blood cultures also grew Cryptococcus neoformans. The patient was started on amphotericin B and flucytosine. The patient initially improved and was transferred to the rehabilitation unit but ultimately her course was complicated by multiple infections and intubations and she unfortunately passed away.

Does higher quality of care in systemic lupus erythematosus translate to better patient outcomes?

PURPOSE: To assess if high quality of care (QOC) in SLE results in improved outcomes of quality of life (QOL) and non-routine health care utilization (HCU). METHODS: One hundred and forty consecutive SLE patients were recruited from the Rheumatology clinic at an academic center. Data on QOC and QOL were collected along with demographics, socio-economic, and disease characteristics at baseline. LupusPRO assessing health-related (HR) QOL and non (N)HRQOL was utilized. Follow up QOL and HCU were collected prospectively at 6 months. High QOC was defined as those meeting ≥80% of the eligible quality indicators. Univariate and multivariate regression analyses were performed with QOC and high QOC as independent variables and HRQOL and NHRQOL as dependent variables at baseline and follow up. Multivariable models were adjusted for demographics and disease characteristics. Secondary outcomes included non-routine HCU and disease activity at follow up. RESULTS: Baseline and follow up data on 140 and 94 patients, respectively, were analyzed. Mean (SD) performance rate (QOC) was 78.6 (13.4) with 52% patients in the high QOC group. QOC was associated with better NHRQOL at baseline and follow up but not with HRQOL. Of all the NHRQOL domains, QOC was positively associated with treatment satisfaction. QOC or high QOC were not associated with non-routine HCU and were instead associated with higher disease activity at follow up. CONCLUSION: Higher QOC predicted better NHRQOL by directly impacting treatment satisfaction in SLE patients in this cohort. Higher QOC, however, was not associated with HRQOL, HCU, or improvement in disease activity at follow up.

Does Higher Quality of Care in Systemic Lupus Erythematosus Improve Quality of Life?

OBJECTIVE: To study the association between high quality of care (QOC) and quality of life (QOL) and nonroutine health care use (HCU) in systemic lupus erythematosus. METHODS: Data were derived from 814 participants from the Lupus Outcomes Study sample. Data on sociodemographic information, disease status, medications, and health care variables were collected through annual interviews. QOC was measured at baseline on 13 quality indices amenable to self-report. Follow-up QOL was measured using the Short Form 36 health survey (SF-36) 2 years later. Univariate and multivariate regression analyses assessed the relationship between QOC and SF-36 scores at baseline, and logistic regression analyses evaluated QOC at baseline as a predictor of minimal clinically important difference (MCID) improvements in SF-36 scores, emergency room (ER) visits, and hospitalizations at follow-up. RESULTS: Higher QOC was associated with worse scores on SF-36 domains on univariate analysis at baseline, which was mediated by comorbidities and high disease activity. QOC and the number of years in high QOC were not predictive of MCID improvements in SF-36 scores at follow-up, which were driven by baseline SF-36 scores, disease activity, and nonroutine HCU. A similar pattern was noted for ER visits and hospitalizations, for which disease activity, damage, and glucocorticoid dose were significant predictors and not QOC. CONCLUSION: High QOC at baseline and the number of years with high QOC are not associated with MCID improvement in SF-36 scores and nonroutine HCU on follow-up. High QOC, as determined by currently defined criteria, serves as a surrogate of greater disease activity, morbidity, and nonroutine HCU.

Pneumococcal Vaccination Among Lupus Patients: Who Are the Recipients?

PURPOSE: Pneumococcal vaccination (PV) is indicated for the elderly (age ≥65 years) and those with chronic disease or who are immunosuppressed. We aimed to study the rate and predictors of recommendation/receipt of 23 valent pneumococcal polysaccharide vaccine (PPSV23) in immunosuppressed systemic lupus erythematosus (SLE) patients. METHODS: Data were obtained through self-report questionnaires and medical chart review of 150 SLE patients. Information on rheumatologist recommendation or receipt of PPSV23 in the preceding 5 years was collected if self-reported in a questionnaire or documented in the medical chart. Chart review was also done to collect data on patient demographics, physician characteristics (if patients had a primary care physician and rheumatologist's SLE patient volume), and the disease characteristics of SLE. Comparisons using χ2 or t tests and logistic regression analyses were conducted for predictors of recommendation/receipt of PV. RESULTS: The mean (SD) age was 47.4 (15.9) years; 90% were women. Sixty-five of 94 eligible patients for PV (based on immunosuppressive medications use or age) had been either recommended or administered PPSV23. On univariate logistic regression analysis, age, duration of disease, current use of hydroxychloroquine or mycophenolate, and rheumatologist's SLE patient volume were significant correlates of recommendation/receipt of PPSV23. However, on multivariate analysis, the only significant predictor was rheumatologist's SLE patient volume after adjusting for the above correlates such that with every 50 patients increase in SLE patient clinic volume, the odds of recommendation/receipt of PPSV23 increased by 2.37 times. CONCLUSIONS: The volume of lupus patients that rheumatologists see is strongly associated with the likelihood that their SLE patients will have PPSV23 recommended and delivered, suggesting a volume outcome relationship.

Sleep Disturbance and Depression Symptoms Mediate Relationship Between Pain and Cognitive Dysfunction in Lupus.

OBJECTIVE: To determine whether sleep disturbance and symptoms of depression mediate the relationship between pain and cognitive dysfunction (CD) in a sample of 115 patients with systemic lupus erythematosus (SLE). METHODS: A total of 115 patients with SLE completed questionnaires regarding pain, perceived stress, depression, sleep, and CD. Relationships among pain, sleep, depression, and CD were assessed using bootstrap mediation models, controlling for race/ethnicity, fibromyalgia diagnosis, current corticosteroid use, disease activity and damage, and perceived stress. RESULTS: Mediation analyses indicated that the effect of pain on CD was mediated by sleep disturbance (ß = 0.30) and depression symptoms (ß = 0.33). These effects were maintained even after controlling for the aforementioned covariates, of which only disease activity (ß = 0.20) and stress (ß = 0.22) remained significantly linked to CD (overall model R2 = 0.53; all P < 0.05). CONCLUSION: After controlling for disease activity and perceived stress, the relationship between pain and CD was explained by sleep disturbance and depression symptoms. Although these relationships need validation in longitudinal studies with additional measurement modalities, our findings may indicate promising, nonpharmacologic intervention avenues for SLE patients with pain and CD. Specifically, cognitive behavioral therapies for depression and sleep are known to reduce distress and enhance functioning across various psychosocial domains. Given the symptom burden of SLE, interventions that maximize potential benefits without the use of additional pharmacologic treatments may be of particular utility.

Does Systemic Lupus Erythematosus Care Provided in a Lupus Clinic Result in Higher Quality of Care Than That Provided in a General Rheumatology Clinic?

OBJECTIVE: To compare the quality of care received by patients with systemic lupus erythematosus (SLE) in 2 settings within the academic institution (a dedicated lupus clinic and a general rheumatology clinic) using validated SLE quality measures. METHODS: One hundred fifty consenting, consecutive SLE patients receiving longitudinal care at the Rush University general rheumatology clinic (n = 73) or the subspecialty lupus clinic (n = 77) were recruited. An updated quality measure survey and retrospective medical chart review were used to evaluate each quality measure (n = 20). The overall and individual quality measure performance was calculated and compared between the 2 groups. Data on the number of SLE patients seen by each rheumatologist were collected to assess the relationship between SLE patient volume and quality measures. RESULTS: Overall quality measure performance was significantly better in SLE patients receiving care at the lupus clinic (85.8% versus 70.2% of patients receiving care at the general rheumatology clinic; P = 0.001). Differences between the 2 groups were observed for sunscreen counseling (98.7% and 83.6%, respectively; P = 0.001), antiphospholipid antibody testing (71.4% and 37%, respectively; P < 0.001), pneumococcal vaccination (84.8% and 48.8%, respectively; P < 0.001), bone mineral density testing (94.2% and 54.5%, respectively; P < 0.001), drug counseling (92.2% and 80.8%, respectively; P = 0.04), use of a steroid-sparing agent (100% and 82%, respectively; P < 0.007), use of an angiotensin-converting enzyme inhibitor (94.4% and 58.3%, respectively; P = 0.03), and cardiovascular disease risk assessment (40.3% and 15.1%, respectively; P = 0.01). There was a moderate correlation between physician volume and quality measure performance (ρ = 0.48, P < 0.001). CONCLUSION: Compared with the general rheumatology clinic, the dedicated lupus clinic had better quality measure performance in this cross-sectional single-center study. In our health care system, we also observed indicators suggesting that rheumatologists with a higher volume of SLE patients provide higher quality of care.