How to Monitor Disease Activity of Axial Spondyloarthritis in Clinical Practice.

PURPOSE OF REVIEW: Treatment guided by periodic and quantitative data assessment results in better outcomes compared to using clinical gestalt. While validated generic as well as specific disease activity measures for axial spondyloarthritis (axSpA) are available, there is vast scope to improve their actual utilization in routine clinical practice. In this review, we discuss available disease activity measures for axSpA, describe results from the survey conducted among general rheumatologists as well as Spondyloarthritis Research and Treatment Network (SPARTAN) members about disease activity measurement in daily practice, and discuss ways to improve axSpA disease activity using technological advances. We also discuss the definitions of active disease and target for the treatment of axSpA. RECENT FINDINGS: The 2019 American College of Rheumatology (ACR)/Spondylitis Association of America (SAA)/Spondyloarthritis Research and Treatment Network (SPARTAN) axSpA treatment guidelines conditionally recommend the regular monitoring of disease activity using a validated measure such as Bath Ankylosing Spondylitis Disease Activity Index (BASDAI) or Ankylosing Spondylitis Disease Severity Index (ASDAS). Assessment of Spondyloarthritis International Society (ASAS)-European Alliance of Associations for Rheumatology (EULAR) guidelines recommend ASDAS as the most appropriate instrument for the assessment of disease activity, preferably calculated using C-reactive protein (CRP). ASAS has selected a core set of variables which were updated recently and have been endorsed by the Outcome Measures in Rheumatology Clinical Trials (OMERACT) group in order to bring homogeneity in assessment of axSpA. In a recent study, Patient-Reported Outcomes Measurement Information System (PROMIS®) measures were able to discriminate inactive, moderate, and high-very high ASDAS activity groups. A newly developed semi-objective index P4 (pain, physical function, patient global, and physician global) correlates well with BASDAI and ASDAS in axSpA and can also be used for other rheumatic diseases in busy clinical practices. Regular disease activity monitoring is critical for long-term management of axSpA and shared decision-making. The integration of electronic health records and smart devices provides a great opportunity to capture patient-reported data. Automated capture of electronic patient-reported outcome measures (ePROMs) is a highly efficient way and results in consistent regular monitoring and may improve the long-term outcomes. While currently used measures focus only on musculoskeletal symptoms of axSpA, a composite disease activity measure that can also incorporate extra-articular manifestations may provide a better assessment of disease activity.

The impact of hospitalization on mortality in patients with connective tissue disease-associated interstitial lung disease: a medical records review study.

BACKGROUND: Interstitial lung disease (ILD) remains one of the most important causes of morbidity and mortality in patients with Connective Tissue Diseases (CTD). This study evaluated the impact of hospitalization on mortality in an ethnically and racially diverse cohort of CTD-ILD patients. METHODS: We conducted a medical records review study at Montefiore Medical Center, Bronx, NY. We included 96 patients and collected data on demographic characteristics, reasons for hospitalization, length of stay, immunosuppressant therapy use, and mortality. We stratified our patients into two cohorts: hospitalized and non-hospitalized. The hospitalized cohort was further subdivided into cardiopulmonary and non-cardiopulmonary admissions. Two-sample tests or Wilcoxon's rank sum tests for continuous variables and Chi-square or Fisher's exact tests for categorical variables were used for analyses as deemed appropriate. RESULTS: We identified 213 patients with CTD-ILD. Out of them, 96 patients met the study's inclusion criteria. The majority of patients were females (79%), and self-identified as Hispanic (54%) and Black (40%). The most common CTDs were rheumatoid arthritis (RA) (29%), inflammatory myositis (22%), and systemic sclerosis (15%). The majority (76%) of patients required at least one hospitalization. In the non-hospitalized group, no deaths were observed, however we noted significant increase of mortality risk in hospitalized group (p = 0.02). We also observed that prolonged hospital stay (> 7 days) as well as older age and male sex were associated with increased mortality. CONCLUSIONS: Prolonged (> 7 days) hospital stay and hospitalization for cardiopulmonary causes, as well as older age and male sex were associated with an increased mortality risk in our cohort of CTD-ILD patients.

The impact of hospitalization on mortality in patients with connective tissue disease-associated interstitial lung disease: a medical records review study

Abstract Background Interstitial lung disease (ILD) remains one of the most important causes of morbidity and mortality in patients with Connective Tissue Diseases (CTD). This study evaluated the impact of hospitalization on mortality in an ethnically and racially diverse cohort of CTD-ILD patients. Methods We conducted a medical records review study at Montefiore Medical Center, Bronx, NY. We included 96 patients and collected data on demographic characteristics, reasons for hospitalization, length of stay, immunosuppressant therapy use, and mortality. We stratified our patients into two cohorts: hospitalized and nonhospitalized. The hospitalized cohort was further subdivided into cardiopulmonary and non-cardiopulmonary admissions. Two-sample tests or Wilcoxon's rank sum tests for continuous variables and Chi-square or Fisher's exact tests for categorical variables were used for analyses as deemed appropriate. Results We identified 213 patients with CTD-ILD. Out of them, 96 patients met the study's inclusion criteria. The majority of patients were females (79%), and self-identified as Hispanic (54%) and Black (40%). The most common CTDs were rheumatoid arthritis (RA) (29%), inflammatory myositis (22%), and systemic sclerosis (15%). The majority (76%) of patients required at least one hospitalization. In the non-hospitalized group, no deaths were observed, however we noted significant increase of mortality risk in hospitalized group (p = 0.02). We also observed that prolonged hospital stay (> 7 days) as well as older age and male sex were associated with increased mortality. Conclusion Prolonged (> 7 days) hospital stay and hospitalization for cardiopulmonary causes, as well as older age and male sex were associated with an increased mortality risk in our cohort of CTD-ILD patients.

Clinical, histological features, and predictors of relapse in patients with idiopathic granulomatous mastitis.

Idiopathic granulomatous mastitis is a benign, inflammatory disease of breasts characterized by non-caseating granulomas. Our study aims to identify distinguishing clinical and histopathological features of relapsing disease compared to those in complete remission. We queried databases at our institution (1990-2021) to include females ≥18 years with biopsy-proven diagnosis of idiopathic granulomatous mastitis, excluding patients with breast cancer, lymphoproliferative disorders, solid organ malignancy, foreign body reaction in breast, plasma cell mastitis, and ductal ectasia. Remission was defined as a 3-month period without recurrence of symptoms or imaging findings. Relapse was defined as recurrence after 3 months of remission. Clinical and histopathological features were compared using 2-sample t tests and chi-squared tests. Of the 27 patients that met our inclusion criteria, the mean age at diagnosis was 35.8 years (± standard deviation 9.4 years) with a mean body mass index of 31.7 kg/m2 (± standard deviation 6.7 kg/m2). 11 (41%) were Hispanic, 25 (93%) had at least one previous full-term pregnancy prior to diagnosis and 8 (30%) were on oral contraceptives. Remission was seen in 18 patients (66%) and 9 (33%) had relapse. Six of these patients received steroids after antibiotics, while 5 patients received methotrexate. Three (33%) patients with relapse and 14 (77%) with remission, had abscess formation confirmed on histopathology (P = .04). Patients with remission had a higher number of abscesses on histopathology and history of oral contraceptive use was associated with more relapse. By identifying key clinical and histopathological findings in this population may guide prognosis and treatment of these patients.

Hospitalization, Critical Illness, and Mortality Outcomes of COVID-19 in Patients With Rheumatoid Arthritis.

OBJECTIVE: To investigate the clinical outcomes of patients with rheumatoid arthritis (RA) with COVID-19. METHODS: This retrospective study consisted of 361 patients with RA+ and 45,954 patients with RA- (March 2020 to August 2022) who tested positive for SARS-CoV-2 by polymerase-chain-reaction in the Montefiore Health System, which serves a large low-income, minority-predominant population in the Bronx and was an epicenter of the initial pandemic and subsequent surges. Primary outcomes were hospitalization, critical illness, and all-cause mortality associated with SARS-CoV-2 infection. Comparisons were made with and without adjustment for covariates, as well as with 1083 matched controls of patients with RA- and COVID-19. RESULTS: Patients with RA+ and COVID-19 were older (62.2 ± 23.5 vs. 45.5 ± 26.3; P < 0.001), were more likely females (83.1% vs. 55.8%; P < 0.001), were Black (35.5% vs. 30.3%; P < 0.05), and had higher rates of comorbidities (P < 0.05), hospitalization (52.4% vs. 32.5%; P < 0.005), critical illness (10.5% vs. 6.9%; P < 0.05), and mortality (11.1% vs. 6.2%; P < 0.01) compared with patients with RA- and COVID-19. Patients with RA+ with COVID-19 had higher odds of critical illness (adjusted odds ratio [aOR] = 1.46, 95% confidence interval [CI]: 1.09-1.93; P = 0.008) but no differences in hospitalization (aOR = 1.18 [95% CI: 0.93-1.49]; P = 0.16) and mortality (aOR = 1.34 [95% CI: 0.92-1.89]; P = 0.10) after adjusting for covariates. Odds ratio analysis identified age, hospitalization status, female sex, chronic kidney disease, chronic obstructive pulmonary disease, and Black race to be significant risk factors for COVID-19-related mortality. Pre-COVID-19 steroid and biologic therapy to treat RA were not significantly associated with worse outcomes (P > 0.05). Outcomes were not different between patients with RA+ and propensity-matched RA- controls (P > 0.05). CONCLUSION: Our findings suggest that risk factors for adverse COVID-19 outcomes were not attributed to RA per se but rather age and preexisting medical conditions of patients with RA.

Difficult to treat psoriatic arthritis - how should we manage?

Psoriatic arthritis (PsA) is a chronic, multi-domain immune-mediated inflammatory arthritis with a high disease burden. PsA patients have significant co-morbidities like obesity, depression, fibromyalgia which can impact disease activity assessment. The management of PsA has undergone a paradigm shift over the last decade due to the availability of multiple biologic and targeted synthetic disease modifying anti-rheumatic drugs. Despite the availability of multiple therapeutic agents, it is not uncommon to find patients not responding adequately and continuing to have active disease and/or high disease burden. In our review, we propose what is "difficult to treat PsA", discuss differential diagnosis, commonly overlooked factors, co-morbidities that affect treatment responses, and suggest a stepwise algorithm to manage these patients.

Physical Activity Habits Among Older Adults Living With Rheumatic Disease.

OBJECTIVE: To describe levels of physical activity (PA) in older adults with rheumatic and musculoskeletal diseases (RMDs) and study the association between PA level and patient-reported outcomes. METHODS: Using data from FORWARD, a cross-sectional analysis was performed among adults aged 65 years and older with RMDs to assess the levels of PA. PA was categorized as high (vigorously active for at least 30 minutes, 3 times per week), moderate (moderately active for at least 3 times per week) or low (seldom active). We assessed the self-reported levels of PA among patients with different types of RMDs and assessed the association between levels of PA and PROs, including the 29-item Patient Reported Outcomes Measurement Information System (PROMIS-29) assessment. RESULTS: Among the 3343 eligible participants, rheumatoid arthritis (68%) was the most common RMD. High PA was reported by 457 (13.6%) participants, and 1820 (54.4%) reported moderate activity. Overall, participants reported a median of 7 (IQR 0-15) days of moderate to vigorous level of PA for ≥ 30 min per month. Obese participants were significantly more likely to report low levels of activity (44% of obese compared to 25% of nonobese individuals). Participants with low PA levels had higher (worse) pain scores, higher (worse) Health Assessment Questionnaire-Disability Index scores, higher depression rates, and worse PROMIS-29 scores related to pain, sleep and fatigue. CONCLUSION: Among patients with RMDs, levels of high PA were relatively low among older patients. These observations, though descriptive, support a relationship between physical inactivity and obesity, depression, poor sleep, and fatigue in patients with RMDs.

Sarcoidosis-Associated Immune Thrombocytopenic Purpura and Focal Segmental Glomerulosclerosis.

Sarcoidosis is well known for heterogeneity of its presentation and multisystem organ involvement. It commonly involves respiratory tract, skin, eyes, and lymph nodes, as well as hematologic and renal systems. While anemia and lymphopenia are the most common hematologic abnormalities seen in sarcoidosis, immune thrombocytopenic purpura (ITP) is considered rare. Renal abnormalities, although infrequent, are usually more likely to involve tubules rather than glomeruli. In this report, we present a case of sarcoidosis-associated ITP and focal segmental glomerulosclerosis (FSGS), refractory to first-line therapy, but successfully treated with Rituximab and thrombopoietin-receptor agonist.

Psoriatic Arthritis: The Influence of Co-morbidities on Drug Choice.

Psoriatic arthritis (PsA) is associated with a higher burden of co-morbidities such as obesity, cardiovascular disease, non-alcoholic fatty liver disease, inflammatory eye disease, inflammatory bowel disease, skin cancer and depression compared to the general population. In the last 20 years, the therapeutic options for PsA have increased exponentially with the availability of tumor necrosis factor-alpha (TNF) inhibitors, interleukin (IL)-17 inhibitors, IL-12/23 inhibitors and Janus kinases/signal transducer and activator of transcription proteins (JAK/STAT) inhibitors. The articular and extra-articular manifestations of PsA usually dictate the treatment choice but important consideration must be given to the corresponding co-morbidities while deciding the drug therapy due to associated safety profile, effect on disease activity, etc. This review provides a comprehensive review of common co-morbidities in PsA and how they can influence treatment choices.

The Role of Early Subspeciality Consultation in the Timing of Hemophagocytic Lymphohistiocytosis Diagnosis and Management.

OBJECTIVE: The aim of this study was to investigate the relation between timing of subspeciality consult and hemophagocytic lymphohistiocytosis (HLH) consideration, immunosuppression initiation, and in-hospital mortality in patients with HLH. METHODS: We conducted a medical records review study of patients 18 years or older with definite or probable HLH at Montefiore Medical Center between 2006 and 2019. Earlier subspeciality consultation (rheumatology, hematology, and infectious disease) was defined as consultation in less than or equal to 18 hours from time of admission. Demographic, clinical characteristics, and outcomes were compared between patients with early and later subspecialty consultation. RESULTS: A total of 28 patients were included. The median age was 40 years, and 61% of patients were male. Infection was identified as a cause of HLH in 13 patients (46%). Fifteen patients (54%) were classified as having an earlier subspeciality consultation with a median time (interquartile range) to HLH consideration of 1.0 day (0.3-4.2 days) compared with 7.9 days (3.1-9.9 days) for the later consultation group (p = 0.002). The median time (interquartile range) to immunosuppression initiation was 4.6 days (1.7-7.8 days) versus 10.9 days (5.1-13.4 days) (p = 0.01), respectively. Five patients (33%) had in-hospital deaths in the early consultation group compared with 7 patients (54%) in later consultation group (p = 0.27). Among the subset of patients who survived to discharge, the 90-day readmission rate was higher in the later consultation group (83% vs 30%, p = 0.12). CONCLUSIONS: In patients with HLH, earlier subspeciality consultation may play a role in earlier HLH consideration and treatment initiation.

When sarcoidosis mimics immunodeficiency.

Sarcoidosis is a granulomatous disease with a wide spectrum of clinical manifestations. A 28-year-old previously healthy woman presented with multifocal pneumonia, mediastinal lymphadenopathy which was thought to be caused by actinomyces infection. Despite antibiotics, she developed cavitary lung lesions and had worsening lymphadenopathy prompting evaluation for alternative aetiologies like malignancy, autoimmune or immunodeficiency disorders. Further workup also revealed low CD4, CD8 cell counts, elevated soluble interleukin-2 levels. Over the due course of time, she developed granulomatous pan-uveitis, classical skin lesions leading to the diagnosis of sarcoidosis. Our case highlights the rare manifestations of sarcoidosis which can mimic immunodeficiency disorders especially when these patients develop secondary infections. Our goal is to raise awareness among clinicians about these atypical presentations of sarcoidosis which can lead to substantial delay in diagnosis thus leading to progression of the disease in the absence of appropriate treatment.

Educational needs and challenges in axial spondyloarthritis.

PURPOSE OF REVIEW: Axial spondyloarthritis (axSpA) affects 0.5-1% of the population in many regions of the world. This review summarizes the challenges in medical education around axSpA with attention to evidence around delayed diagnosis, clinician familiarity with typical axSpA features, such as inflammatory back pain and adherence to accepted management principles. RECENT FINDINGS: Clinicians who commonly manage patients with chronic back pain or other typical axSpA features are not consistently aware of the concept of inflammatory back pain and common extra-spinal manifestations. Further, clinicians may not be familiar with the nonradiographic spectrum of axSpA. Management of patients with possible axSpA does not consistently follow principles that would establish an axSpA diagnosis, and diagnosis of axSpA remains delayed by 6-7 years on average, with evidence suggesting management disparities on the basis of sex and race in some cases. Referral recommendations have increased the probability of axSpA diagnosis up to about 40% and, may complement educational efforts in axSpA. SUMMARY: Educational efforts in axSpA should focus on providing front-line clinicians with a better understanding of inflammatory back pain, the nonradiographic form of axSpA, and accepted principles in axSpA management.

Early and Significant Reduction in C-Reactive Protein Levels After Corticosteroid Therapy Is Associated With Reduced Mortality in Patients With COVID-19.

BACKGROUND: Corticosteroids may be beneficial in a subset of patients with coronavirus disease 2019 (COVID-19), but predictors of therapeutic response remain unknown. C-reactive protein (CRP) is a routinely measured biomarker, and reduction in its levels after initiation of therapy may predict inpatient mortality. METHODS: In this retrospective cohort study, the charts of patients who were admitted to Montefiore Medical Center between March 10, 2020, and May 2, 2020 for the management of COVID-19 were examined. Of all patients who met inclusion criteria, patients who received corticosteroid treatment were categorized as CRP responders (≥50% CRP level reduction) and CRP nonresponders (<50% CRP level reduction) based on change in CRP within 72 hours of corticosteroid treatment initiation. The outcomes of interest were two-fold: (1) CRP response after treatment with corticosteroid, and (2) differences in mortality among patients with CRP response compared those without. RESULTS: Of 2,707 patients admitted during the study period, 324 received corticosteroid treatment. Of patients who received corticosteroid treatment, CRP responders had reduced risk of death compared with risk among CRP nonresponders (25.2% vs 47.8%; unadjusted odds ratio [OR], 0.37; 95% CI, 0.21-0.65; P <.001). This effect remained strong and significant after adjustment for potential confounders (adjusted OR, 0.27; 95% CI, 0.14-0.54; P <.001). CONCLUSION: Reduction in CRP by 50% or more within 72 hours of initiating corticosteroid therapy potentially predicts inpatient mortality. This may serve as an early biomarker of response to corticosteroid therapy in patients with COVID-19.

Rapid Implementation of a Multidisciplinary COVID-19 Cytokine Storm Syndrome Task Force.

OBJECTIVE: Patients with coronavirus disease 2019 (COVID-19) can progress to a state of unregulated inflammation called cytokine storm syndrome (CSS). We describe formation and operation of a COVID-19 multidisciplinary consultation service that was allowed to individualize treatment for critically ill patients with COVID-19 during the pandemic. METHODS: Institutional experts from different subspecialties formed a COVID-19 CSS task force at Montefiore Medical Center, Bronx, NY. They agreed on a set of four clinical and six laboratory parameters that can help early identify COVID-19 CSS. We describe the formation and implementation of the COVID-19 task force. The case series description of the COVID-19 CSS consultation cohort highlights consultation volume, baseline characteristics, clinical and laboratory parameters, and how biologic treatments were allocated to these patients. RESULTS: Between April 4,2020, and May 7,2020, the COVID-19 CSS task force was formed, consisting of adult and pediatric rheumatologists and allergy and immunology physicians. The task force evaluated a total of 288 patients, of whom 197 (68%) were male, the median (interquartile range [IQR]) age was 62 (51-70) years, 122 (42%) were Hispanic, and 88 (31%) were Black or African American. The common presenting symptoms in all referred patients were dyspnea (85%) and diarrhea (80%). Thirty-one patients who received biologic therapy were younger, with a median (IQR) age of 53 (32-63) years, as opposed to 62.5 (52-70) years in the nonbiologic group (P = 0.008). A higher proportion receiving biologics was in the critical care setting (26 [84%] vs 151 [59%]; P = 0.006). CONCLUSION: To the best of our knowledge, this is the first multidisciplinary collaborative effort to provide individualized patient recommendations for evaluation and treatment of patients with COVID-19 who may have CSS. This working model helped to devise an approach that may have identified patients who were most likely to benefit from biologic therapy in the absence of evidence-based guidelines.

Obesity and Psoriatic Arthritis: A Narrative Review.

Patients with psoriatic arthritis (PsA) have a higher burden of cardio-metabolic comorbidities like obesity, hypertension, diabetes, and cardiovascular disease compared to the general population. Adipose tissue is thought to promote a chronic low grade inflammatory state through inflammatory mediators like tumor necrosis factor alpha (TNFα), interleukin-6 (IL-6), leptin, and adiponectin. A higher body mass index (BMI) is a risk factor for development of PsA and affects disease activity and response to therapy including both disease-modifying anti-rheumatic drugs (DMARDs) and tumor necrosis factor inhibitors (TNFi). Obesity has an impact on the morbidity in PsA, particularly cardiovascular and/or metabolic. Patients with PsA have a higher cardiovascular risk and obesity may have an additive impact on morbidity and mortality. This review explores the relationship between obesity and PsA.