To be or not to be compliant? Hospitals' initial strategic responses to the federal price transparency rule.

OBJECTIVE: To understand US hospitals' initial strategic responses to the federal price transparency rule that took effect January 2021. DATA SOURCES AND STUDY SETTING: Primary interview data collected from 12 not-for-profit hospital organizations in six US metropolitan markets. All but one organization were multihospital systems; the 12 organizations represent a total of 81 hospitals. STUDY DESIGN: Exploratory, cross-sectional, qualitative interview study of a convenience sample of hospital organizations across six geographically and compliance diverse markets. DATA COLLECTION/EXTRACTION METHODS: In-depth, semi-structured, qualitative interviews with 16 key informants across sampled organizations between November 2021 and March 2022. Interviews solicited data about internal organizational factors and external market factors affecting strategic responses. Transcribed interviews were de-identified, coded, and analyzed using the constant comparative method. PRINCIPAL FINDINGS: Hospitals' strategic responses were influenced internally by the degree of the regulation's alignment with organizational values and goals, and task complexity vis-a-vis available resources. We found extensive variation in organizational capabilities to comply, and all but one organization relied on consultants and vendors to some degree. Key external factors driving strategic responses were hospitals' variable perceptions about how available price information would affect their competitive position, bottom line, and reputation. Organizations with more confidence in their interpretation of the environment, including how peers or purchasers would behave, and greater clarity in their own organization's position and goals, had more definitive initial strategic responses. In the first year, organizations' strategic responses skewed toward compliance, especially for the rule's consumer shopping requirements. CONCLUSIONS: A deeper understanding of the realities of operationalizing price transparency policy for hospitals is needed to improve its impact.

Clinical Associations of Degos-Like Lesions in Patients With Systemic Sclerosis.

Importance: Degos-like lesions are cutaneous manifestations of a small-vessel vasculopathy that appear as atrophic, porcelain-white papules with red, telangiectatic borders. No study has adequately examined Degos-like lesions in patients with systemic sclerosis (SSc). Objective: To characterize the serologic, cutaneous, and internal organ manifestations associated with Degos-like lesions in a large cohort of patients with SSc. Design, Settings, and Participants: This retrospective cohort study involved adult patients with SSc who were seen at Stanford Rheumatologic Dermatology Clinic between January 1, 1998, and December 31, 2018. Participants fulfilled the 2013 classification criteria for SSc. Data analysis was conducted from February 1 to June 1, 2019. Main Outcomes and Measures: Data on demographic characteristics; autoantibody status; clinical characteristics, including cutaneous and systemic manifestations of SSc; and presence of Degos-like lesions were collected. Results: The cohort comprised 506 patients with SSc (447 females [88.3%]; mean [SD] age at first non-Raynaud disease symptoms, 46.1 [15.2] years). Twenty-seven patients (5.3%) had Degos-like lesions, of whom 24 (89.0%) had lesions affecting the fingers. Patients with Degos-like lesions were more likely to have diffuse cutaneous SSc compared with patients without lesions (15 [55.6%] vs 181 [37.8%]; P = .04). Degos-like lesions were also associated with acro-osteolysis (10 [37.0%] vs 62 [12.9%]; P < .01), digital ulcers (15 [55.6%] vs 173 [36.1%]; P = .04), and calcinosis (15 [55.6%] vs 115 [24.0%]; P < .01). While Degos-like lesions were not associated with internal organ manifestations, such as scleroderma renal crisis, interstitial lung disease, or pulmonary arterial hypertension, there was P < .10 for the association with gastric antral vascular ectasia. Conclusions and Relevance: Results of this study suggest an association of Degos-like lesions with diffuse cutaneous SSc and other cutaneous manifestations of vasculopathy, including acro-osteolysis, calcinosis, and digital ulcers. A prospective longitudinal study is warranted to examine the onset of Degos-like lesions and to elucidate whether these lesions play a role in SSc.

The Extent Hospital Organizational Factors Influence Inpatient Care Delivery: A Case Study Looking at Knee and Hip Replacement Surgery.

There is a body of Implementation and Dissemination research describing the importance of "context"-the characteristics describing the setting where a process or innovation occurs-when evaluating delivery, outcomes and cost of health services. These contextual factors, which can occur at the system, organization, or provider level, may either facilitate or erect barriers to the utilization of evidence-based practices and the outcomes achieved. This paper examines the influence of organizational structure and operating environment characteristics of where inpatient health care is delivered, controlling for patient and provider characteristics, on health services delivery and outcomes achieved. We used inpatient cost-of-care to represent the bundle of services provided to patients receiving primary knee and hip replacement procedures. Data includes patient level data from discharge records for 62 140 knee replacements and 42 392 hip replacements from the 2015 AHRQ Healthcare Cost and Utilization Project State Inpatient Discharge database and hospital characteristics from the 2015 American Hospital Association survey. Multi-level linear estimation models controlling for patient and payer characteristics were employed to assess the impact of specific organizational and operating environment factors. We found that although patient and payer characteristics significantly impacted the inpatient cost of care, there is significant variation between hospitals and among physicians within a hospital beyond what can be explained by patient, payer and local price effect characteristics. Organizational and physician characteristics that had the most significant impact on cost of care included the volume of services provided, urban location, and for-profit ownership. These factors can inform future policy and program design and evaluation.

Association of Care Coordination Experience and Health Services Use with Main Provider Type for Children with Inflammatory Bowel Disease.

OBJECTIVES: To describe care coordination experience for families of children with inflammatory bowel disease (IBD) and compare use of health services between families who identified a primary care physician (PCP) vs a gastroenterologist as a child's main provider. STUDY DESIGN: This is a cross-sectional survey of care coordination experiences and health services use for children 6-19 years old receiving care in the IBD program at a children's hospital during 2018. English-speaking parents completed the Family Experiences with Coordination of Care Survey about their child's main provider and reported past-year health services. Bivariate testing and multivariate logistic regression explored differences in care coordination experience and health services by main provider, adjusted for demographic and clinical variables. RESULTS: A total of 113 of 270 (42%) invited patients participated. Among 101 patients with complete data, 41% identified a PCP main provider. Performance on 5 of 16 Family Experiences with Coordination of Care indicators was higher for patients reporting a gastroenterologist vs a PCP main provider. However, having a PCP vs gastroenterologist main provider was associated with greater use of any past-year primary care services (adjusted proportion 94% vs 75%; P = .01) and of mental health services when needed (95% vs 60%; P < .01). Need for IBD-related hospitalization and emergency department visits did not differ between groups. CONCLUSIONS: Children with IBD may experience trade-offs in care coordination quality and important, non-disease-focused health services based on whom parents perceive as the main provider. Efforts to enhance cross-team coordination among families and primary and specialty care teams are needed to improve overall care quality.

What makes health services usable?: Insights from a qualitative study of caregivers of children with disabilities.

BACKGROUND: The concept of usability from the field of user-centered design addresses the extent to which a system is easy to use, including under extreme conditions. Apart from applications to technologies, however, little attention has been given to understanding what shapes usability of health services more generally. Health service usability may impact the extent to which patients avail themselves of and benefit from those services. PURPOSE: The aim of the study was to develop the concept of usability as it applies to health services, particularly for a high-need, complex patient population. APPROACH: We conducted interviews and focus groups with 66 caregivers of children with disabilities and analyzed data through inductive coding and constant comparison. RESULTS: We find that before health services can be rendered usable for patients with complex health conditions, work is often required to develop trusting relationships with individual providers and to manage time demands and attendant challenges of physical access. In addition, our findings show that actions crucial to receiving benefits from one service often entail difficult tradeoffs either with other services or with other important features in the patient's life-world. Finally, we propose the concept of configuration to capture the complex interdependent arrangement of connections to multiple health services, often for multiple household members, and other life-world factors (e.g., employment, transportation, living conditions). These configurations are dynamic, fragile, and vulnerable to shocks-events that destabilize them, often negatively impacting the relative usability of services and of the entire configuration. Collectively, these findings illustrate health service usability as a relational, situated, emergent property rather than an inherent feature of the service itself. PRACTICE IMPLICATIONS: System-centered design perspectives produce services that are usable for the mythical "ideal" user. To be truly "patient centered," designs must "decenter" the health service and recognize it as one component of the patient's life-world configuration.

For Rural Hospitals That Merged, Inpatient Charges Decreased and Outpatient Charges Increased: A Pre-/Post-Comparison of Rural Hospitals That Merged and Rural Hospitals That Did Not Merge Between 2005 and 2015.

PURPOSE: To determine whether inpatient and outpatient charges changed at rural hospitals after a merger. METHODS: Hospital mergers were derived from proprietary Irving Levin Associates data through manual review and validation. Hospital-level characteristics were derived from HCRIS, CMS Impact File Hospital Inpatient Prospective Payment System, Hospital MSA file, AHRF, and US Census data. A difference-in-differences approach was used to determine whether inpatient and outpatient charges changed at rural hospitals after a merger. The comparison group, rural hospitals that did not merge at any point during the sample period, was weighted using inverse probability of treatment weights. Key outcome measures were total inpatient and total outpatient charges (logged). FINDINGS: Hospitals that merged billed 17.73% more inpatient charges and 12.66% more outpatient charges at baseline compared to hospitals that did not merge. Our results indicate that merging was associated with a 3.04% decrease in inpatient charges (P < .001) and a 1.07% increase in outpatient charges (P = .082). Merging was also associated with a 4.38% decrease in total revenue, a 3.58% decrease in net patient revenue, and no change in total inpatient discharges or average daily census. CONCLUSIONS & IMPLICATIONS: Merging was strongly associated with a decrease in inpatient charges and somewhat associated with an increase in outpatient charges for rural hospitals. Future work could build upon this work to determine whether acquirers reduce or eliminate certain services at rural hospitals after a merger, and ultimately how changes in service delivery could impact patients in those rural communities.

Capital Expenditures Increased at Rural Hospitals That Merged Between 2012 and 2015.

EXECUTIVE SUMMARY: The number of rural hospital mergers has increased substantially in recent years. A commonly reported reason for merging is to increase access to capital. However, no empirical evidence exists to show whether capital expenditures increased at rural hospitals after a merger. We used a difference-in-differences approach to determine whether total capital expenditures changed at rural hospitals after a merger. The comparison group (rural hospitals that did not merge during the 2012 through 2015 study period) was weighted using inverse probability of treatment weights. The key outcome measure was logged total capital expenditures.Merging resulted in a 26% increase in capital expenditures and also was associated with a significant improvement in plant age. The postmerger improvement in plant age may have been partially attributable to merger-related accounting changes and partially attributable to increased capital expenses, possibly on long-term asset renovations and replacement.These findings suggest that through mergers, rural hospital board members and executives who have accepted or are considering a merger may improve a hospital's ability to increase capital expenditures. Further, increased capital investments in rural hospitals may be an important signal to the community that the acquirer intends to keep the rural hospital open and continue providing some volume and level of services within the community. Future research should determine how capital is spent after a merger.

Rural Hospital Mergers Increased Between 2005 and 2016-What Did Those Hospitals Look Like?

The objective of this study is to determine whether key hospital-level financial and market characteristics are associated with whether rural hospitals merge. Hospital merger status was derived from proprietary Irving Levin Associates data for 2005 through 2016 and hospital-level characteristics from HCRIS, CMS Impact File Hospital Inpatient Prospective Payment System, Hospital MSA file, AHRF, and U.S. Census data for 2004 through 2016. A discrete-time hazard analysis using generalized estimating equations was used to determine whether factors were associated with merging between 2005 and 2016. Factors included measures of profitability, operational efficiency, capital structure, utilization, and market competitiveness. Between 2005 and 2016, 11% (n = 326) of rural hospitals were involved in at least one merger. Rural hospital mergers have increased in recent years, with more than two-thirds (n = 261) occurring after 2011. The types of rural hospitals that merged during the sample period differed from nonmerged rural hospitals. Rural hospitals with higher odds of merging were less profitable, for-profit, larger, and were less likely to be able to cover current debt. Additional factors associated with higher odds of merging were reporting older plant age, not providing obstetrics, being closer to the nearest large hospital, and not being in the West region. By quantifying the hazard of characteristics associated with whether rural hospitals merged between 2005 and 2016, these findings suggest it is possible to determine leading indicators of rural mergers. This work may serve as a foundation for future research to determine the impact of mergers on rural hospitals.

The effect of an accountable care organization on dental care for children with disabilities.

OBJECTIVES: Accountable care organizations (ACOs) are a new model of health-care delivery that aim to improve care through increased provider collaboration and financial rewards for meeting cost and quality targets for a defined patient population. In this study, we examined a state policy change that effectively moved some children with disabilities into a Medicaid-serving pediatric ACO on dental service use. We hypothesize that ACOs' emphasis on prevention, care coordination, and reduction in emergency department use will extend to dental services. STUDY DESIGN/METHODS: We used Ohio Medicaid administrative claims data for year 2011-2016 to examine changes in patterns of dental service use by Medicaid-eligible children with disabilities before and after enrolling in an ACO compared with similar children enrolled in non-ACO managed care plans. RESULTS: Dental utilization is relatively low among Medicaid-eligible children with disabilities. We find that preventive dental visits increased 3.1% points (P < 0.05) from a baseline in the control group of 33.9 percent among ACO-enrolled children, especially among adolescent children, compared to similar children that were not in the ACO, representing an 11 percent increase in the rate of preventive dental visits relative to the comparison group. However, overall dental utilization did not increase for children with disabilities who were part of the ACO compared to similar children who were not in the ACO. CONCLUSIONS: Access to dental care is a continuing challenge for children covered by Medicaid. ACOs that serve Medicaid children are well positioned to include dental services and could play an important role in improving access to dental care and increasing dental utilization.

Why Do Some Caregivers of Children with Disabilities Participate in Care Management While Others Do Not? Lessons from a Population in a Pediatric Accountable Care Organization.

Accountable care organizations (ACOs) offer care coordination services in an attempt to lower costs while improving the quality of care; however, not all families participate. We conducted focus groups and individual interviews with caregivers of children who recently joined a pediatric ACO and evaluated why some caregivers of children with disabilities engage in care coordination while others do not. Four common themes emerged as factors influencing the degree of caregiver engagement in care coordination services. These themes include: (1) availability, (2) alignment of services with family need, (3) ease or difficulty of engagement, and (4) timing of services. These findings suggest that considering caregiver perspectives across stages of program development and implementation could encourage more caregivers to engage in care coordination programs.

How does being part of a pediatric accountable care organization impact health service use for children with disabilities?

OBJECTIVE: To examine the impact of a Medicaid-serving pediatric accountable care organization (ACO) on health service use by children who qualify for Medicaid by virtue of a disability under the "aged, blind, and disabled" (ABD) eligibility criteria. DATA SOURCES/STUDY SETTING: We evaluated a 2013 Ohio policy change that effectively moved ABD Medicaid children into an ACO model of care using Ohio Medicaid administrative claims data for years 2011-2016. STUDY DESIGN: We used a difference-in-difference design to examine changes in patterns of health care service use by ABD-enrolled children before and after enrolling in an ACO compared with ABD-enrolled children enrolled in non-ACO managed care plans. DATA COLLECTION/EXTRACTION METHODS: We identified 17 356 children who resided in 34 of 88 counties as the ACO "intervention" group and 47 026 ABD-enrolled children who resided outside of the ACO region as non-ACO controls. PRINCIPAL FINDINGS: Being part of the ACO increased adolescent preventative service and decreased use of ADHD medications as compared to similar children in non-ACO capitated managed care plans. Relative home health service use decreased for children in the ACO. CONCLUSIONS: Our overall results indicate that being part of an ACO may improve quality in certain areas, such as adolescent well-child visits, though there may be room for improvement in other areas considered important by patients and their families such as home health service.

The Medicare Shared Savings Program and Outcomes for Ischemic Stroke Patients: a Retrospective Cohort Study.

BACKGROUND: Post-stroke care delivery may be affected by provider participation in Medicare Shared Savings Program (MSSP) Accountable Care Organizations (ACOs) through systematic changes to discharge planning, care coordination, and transitional care. OBJECTIVE: To evaluate the association of MSSP with patient outcomes in the year following hospitalization for ischemic stroke. DESIGN: Retrospective cohort SETTING: Get With The Guidelines (GWTG)-Stroke (2010-2014) PARTICIPANTS: Hospitalizations for mild to moderate incident ischemic stroke were linked with Medicare claims for fee-for-service beneficiaries ≥ 65 years (N = 251,605). MAIN MEASURES: Outcomes included discharge to home, 30-day all-cause readmission, length of index hospital stay, days in the community (home-time) at 1 year, and 1-year recurrent stroke and mortality. A difference-in-differences design was used to compare outcomes before and after hospital MSSP implementation for patients (1) discharged from hospitals that chose to participate versus not participate in MSSP or (2) assigned to an MSSP ACO versus not or both. Unique estimates for 2013 and 2014 ACOs were generated. KEY RESULTS: For hospitals joining MSSP in 2013 or 2014, the probability of discharge to home decreased by 2.57 (95% confidence intervals (CI) = - 4.43, - 0.71) percentage points (pp) and 1.84 pp (CI = - 3.31, - 0.37), respectively, among beneficiaries not assigned to an MSSP ACO. Among discharges from hospitals joining MSSP in 2013, beneficiary ACO alignment versus not was associated with increased home discharge, reduced length of stay, and increased home-time. For patients discharged from hospitals joining MSSP in 2014, ACO alignment was not associated with changes in utilization. No association between MSSP and recurrent stroke or mortality was observed. CONCLUSIONS: Among patients with mild to moderate ischemic stroke, meaningful reductions in acute care utilization were observed only for ACO-aligned beneficiaries who were also discharged from a hospital initiating MSSP in 2013. Only 1 year of data was available for the 2014 MSSP cohort, and these early results suggest further study is warranted. REGISTRATION: None.

Implications of eligibility category churn for pediatric payment in Medicaid.

OBJECTIVES: To describe the extent and implications of "churn" between different Medicaid eligibility classifications in a pediatric population: (1) aged, blind, and disabled (ABD) Medicaid eligibility, determined by disability status and family income; and (2) Healthy Start Medicaid eligibility, determined by family income alone. STUDY DESIGN: As a result of a 2013 policy change, children with ABD eligibility transitioned from fee-for-service to capitated care. We used Ohio Medicaid claims data from July 2013 through June 2015 to explore the relationships among instability in eligibility category, demographics, and utilization. METHODS: To examine the potential financial effect of categorical churn, an effective capitation rate was created to capture the proportion of the maximum potential capitation rate that was realized. RESULTS: More than 20% of children exited ABD-based eligibility at least once. Switching was associated with younger age and rural residence and was not associated with healthcare use. CONCLUSIONS: Switching between eligibility categories is common and affects average capitation but not health service use.

Gender Differences in Hospital CEO Compensation: A National Investigation of Not-for-Profit Hospitals.

Gender pay equity is a desirable social value and an important strategy to fill every organizational stratum with gender-diverse talent to fulfill an organization's goals and mission. This study used national, large-sample data to examine gender difference in CEO compensation among not-for-profit hospitals. Results showed the average unadjusted annual compensation for female CEOs in 2009 was $425,085 compared with $581,121 for male CEOs. With few exceptions, the difference existed across all types of not-for-profit hospitals. After controlling for hospital- and area-level characteristics, female CEOs of not-for-profit hospitals earned 22.6% less than male CEOs of not-for-profit hospitals. This translates into an earnings differential of $132,652 associated with gender. Explanations and implications of the results are discussed.

Impact of Accountable Care Organizations on Utilization, Care, and Outcomes: A Systematic Review.

Since 2010, more than 900 accountable care organizations (ACOs) have formed payment contracts with public and private insurers in the United States; however, there has not been a systematic evaluation of the evidence studying impacts of ACOs on care and outcomes across payer types. This review evaluates the quality of evidence regarding the association of public and private ACOs with health service use, processes, and outcomes of care. The 42 articles identified studied ACO contracts with Medicare ( N = 24 articles), Medicaid ( N = 5), commercial ( N = 11), and all payers ( N = 2). The most consistent associations between ACO implementation and outcomes across payer types were reduced inpatient use, reduced emergency department visits, and improved measures of preventive care and chronic disease management. The seven studies evaluating patient experience or clinical outcomes of care showed no evidence that ACOs worsen outcomes of care; however, the impact on patient care and outcomes should continue to be monitored.

Coping with interdependencies related to patient choice: Boundary-spanning at four accountable care organizations.

BACKGROUND: Accountable care organizations (ACOs) are responsible for outcomes that are only partially under their control because patients may choose to self-refer outside the ACO, overuse resource-intensive services, or underuse evidence-based care. ACOs must devise boundary-spanning practices to manage these interdependencies related to patient choice. PURPOSE: The aim of this study was to identify, conceptualize, and categorize ACO efforts to cope with interdependencies related to patient choice. APPROACH: We conducted qualitative organizational case studies of four ACOs. We interviewed 89 executives, mid-level managers, and physicians and analyzed the data through multiple rounds of inductive coding. RESULTS: We identified 15 boundary-spanning practices, in which two or more ACOs engaged in efforts to understand, cope with, or alter interdependencies related to patient choice. Analysis of these practices revealed five categories of factors that appeared to shape patient choices in ways that may impact ACO performance: the availability of services, interactions with patients, system complexities, care provided to ACO patients by non-ACO providers, and uncertainties related to the environment. Our findings provide a process theory of ACO boundary-spanning: Each individual boundary-spanning practice contributes to a broader strategic goal, through which it may impact a particular aspect of interdependence and thereby reduce underuse, overuse, or leakage (i.e., provision of services outside the ACO). PRACTICE IMPLICATIONS: In identifying ACO boundary-spanning practices and proposing how they may impact interdependence, our theory highlights conceptual relationships that researchers can study and test. Similarly, in identifying key aspects of interdependencies related to patient choice and a broad assortment of ACO boundary-spanning practices, our findings provide managers with a tool for evaluating and developing their own boundary-spanning efforts.

Satisfaction With Care Coordination for Families of Children With Disabilities.

INTRODUCTION: Children with disabilities have significant health care needs, and receipt of care coordinator services may reduce caregiver burdens. The present study assessed caregivers' experience and satisfaction with care coordination. METHOD: Caregivers of Medicaid-enrolled children with disabilities (n = 2,061) completed a survey (online or by telephone) collecting information on the caregivers' experiences and satisfaction with care coordination using the Family Experiences with Coordination of Care questionnaire. RESULTS: Eighty percent of caregivers with a care coordinator reported receiving help making specialist appointments, and 71% reported help obtaining community services. Caregivers who reported that the care coordinator helped with specialist appointments or was knowledgeable, supportive, and advocating for children had increased odds of satisfaction (odds ratio = 3.46, 95% confidence interval = [1.01, 11.77] and odds ratio = 1.07, 95% confidence interval = [1.03, 1.11], respectively). DISCUSSION: Findings show opportunities for improving care coordination in Medicaid-enrolled children with disabilities and that some specific elements of care coordination may enhance caregiver satisfaction with care.

Patterns of Cardiometabolic Health as Midlife Women Transition to Menopause: A Prospective Multiethnic Study.

CONTEXT: Cardiometabolic conditions increase in midlife, but early customized prevention strategies are not established for such women. OBJECTIVE: To characterize and identify factors longitudinally related to constellations of cardiometabolic risk components in multiracial/ethnic women in midlife. DESIGN: We conducted a prospective, longitudinal, multiethnic cohort study of 3003 midlife women undergoing menopausal transition (MT). Metabolic syndrome (MetS) was defined as having at least three of five components: high fasting triglyceride (hTG) level, low high-density lipoprotein cholesterol (lHDL-C) level, high fasting plasma glucose (hGluc) level, large waist circumference (abdominal obesity; Ob), and hypertension (HTN). We described the patterns of constellations and estimated hazard ratios (HRs) for constellations at (i) incident MetS and (ii) recovery from MetS, using multivariable-adjusted Cox regression. SETTING: Seven US sites. PARTICIPANTS: In all, 1412 non-Hispanic white, 851 black, 272 Japanese, 237 Hispanic, and 231 Chinese women. EXPOSURES: Race/ethnicity, lifestyle factors, and MT stage. MAIN OUTCOMES MEASURES: Cardiometabolic constellations, incident MetS, and MetS recovery. RESULTS: Central obesity was the most frequent component. Having no components was the most frequent (31%) baseline constellation. Physical activity (HR = 1.68; 95% CI: 1.06, 2.68) and lower caloric intake (HR = 0.96; 95% CI: 0.93, 0.99 per 100 cal/d) were associated with recovery from MetS. Ob/hTG/lHDL-C (18%), Ob/HTN/lHDL-C (16%), and Ob/HTN/hGluc (14%) were frequent incident constellations. Physically active women had 26% to 62% lower hazards of incident MetS than inactive women. CONCLUSIONS: Modifiable lifestyle behaviors were related to recovery from MetS and decreased risk of the most frequent MetS constellations in midlife women.

Calcinosis in scleroderma.

PURPOSE OF REVIEW: To provide an update on the available literature regarding the epidemiology, pathophysiology, diagnosis, and treatment of calcinosis cutis in patients with systemic sclerosis (SSc). RECENT FINDINGS: We identified observational studies that describe the frequency of calcinosis in SSc and associated clinical features; molecular studies exploring potential pathogenic mechanisms; and case reports and case series describing new diagnostic approaches and treatments. SUMMARY: Calcinosis cutis is the deposition of insoluble calcium in the skin and subcutaneous tissues. It represents a major clinical problem in patients with SSc affecting at least one quarter of patients. It is associated with longer disease duration, digital ulcers, acro-osteolysis, positive anticentromere antibody, and positive anti-PM/Scl antibody. Although pathogenesis is unknown, there is evidence supporting local trauma, chronic inflammation, vascular hypoxia, and dysregulation of bone matrix proteins as potential mechanisms. Diagnosis can be made clinically or with plain radiography. Several pharmacologic therapies have been tried for calcinosis with variable and modest results, but surgical excision of calcium deposits remains the mainstay of treatment.