Impact of psoriasis remains important in psoriatic arthritis patients with low musculoskeletal disease activity.

OBJECTIVES: Achieving low disease activity (LDA) is important in patients with psoriatic arthritis. It is of value to know if health-related quality of life (HRQoL) of patients who reached musculoskeletal low disease activity can be further improved by additionally achieving remission of their psoriasis. So, the aim of this study was to assess HRQoL in patients with active psoriasis who reached disease activity in psoriatic arthritis (DAPSA) LDA after one year of follow-up. METHODS: Data were collected from the Dutch south west Psoriatic Arthritis cohort. Musculoskeletal disease activity was measured using DAPSA. Patients who reached DAPSA-LDA after one year were divided based on reaching psoriasis remission (Psoriasis Area and Severity Index [PASI] <1). HRQoL and work productivity were compared between both groups. RESULTS: After one year, 230 (44%) patients with active psoriasis at baseline reached DAPSA-LDA, of which 108 (47%) patients achieved psoriasis remission. The group of patients with active psoriasis (n=122, 53%) contained more men (p=0.023) and scored lower on the 12-item Psoriatic Arthritis Impact of Disease questionnaire (p=0.012). On the Skindex-17 psychosocial subscale, 31% experienced moderate to high impairment and on the symptoms subscale 28% experienced a lot of symptoms. Work productivity did not differ between both groups. CONCLUSIONS: The majority of patients with DAPSA-LDA and active psoriasis after one year has a good HRQoL. However, a proportion of these patients still experiences considerable skin burden. We recommend rheumatologists to continue assessing and treating psoriasis to reduce skin burden in PsA patients who achieved musculoskeletal low disease activity.

The burden of psoriasis in patients with early psoriatic arthritis.

OBJECTIVES: Psoriasis impacts health-related quality of life (HRQoL) in PsA patients. However, this is not adequately measured with a general HRQoL questionnaire. The aim of this study was to quantify the degree of psoriasis evolution in PsA patients over the first year of follow-up and to evaluate whether the impact of psoriasis on HRQoL can be adequately measured with a dermatology-specific HRQoL questionnaire. METHODS: Data were used from PsA patients in the Dutch south west Early Psoriatic Arthritis cohort. Psoriasis severity was measured with the Psoriasis Area and Severity Index (PASI). Dermatology-specific HRQoL was assessed with the Skindex-17 questionnaire. We used a Sankey diagram to illustrate the evolution of psoriasis severity during the first year of follow-up. To assess the association between psoriasis severity and the symptoms and psychosocial subscale of the Skindex-17, a linear regression analysis with hierarchical variable selection and zero-inflated negative binominal regression analysis were performed, respectively. RESULTS: We included 644 patients; 109 (17%) patients had no psoriasis (PASI = 0), 456 (71%) had mild psoriasis (PASI < 7), 56 (9%) had moderate psoriasis (PASI 7-12) and 23 (4%) had severe psoriasis (PASI > 12). Psoriasis severity did not fluctuate much during the first year. PASI was significantly associated with both subscales of the Skindex-17 at baseline and 12 months. CONCLUSION: Psoriasis severity in PsA patients is mostly mild but impacts HRQoL when measured using a dermatology-specific HRQoL questionnaire. For optimal management of PsA patients, we recommend rheumatologists acquire information on skin burden by using a dermatology-specific HRQoL questionnaire.

Comparison of disease activity measures in early psoriatic arthritis in usual care.

OBJECTIVES: To compare responsiveness and longitudinal validity of Disease Activity Score 28 (DAS28), Disease Activity index for PSoriatic Arthritis (DAPSA), Composite Psoriatic Disease Activity Index (CPDAI), Psoriatic ArthritiS Disease Activity Score (PASDAS), GRAppa Composite scorE (GRACE) and Minimal Disease Activity (MDA) in usual care PsA patients, within 1 year after diagnosis. METHODS: Data collected in the Dutch southwest early PsA cohort (DEPAR) were used. Responsiveness was assessed using effect size (ES), standardized response mean (SRM), and discrimination between different general health states. Longitudinal validity was tested using mixed models with outcomes health-related quality of life (HRQOL), productivity and disability. RESULTS: Responsiveness was highest for PASDAS, with ES 1.00 and SRM 0.95, lowest for DAPSA, with ES 0.73 and SRM 0.71, and in between for DAS28, CPDAI and GRACE. Differences in general health were best discriminated with PASDAS and GRACE. Patients reporting stable or worsening general health could not be distinguished by DAS28 or CPDAI. Discrimination was better using DAPSA, but worse than when using PASDAS and GRACE. Longitudinal evolvement of HRQOL and productivity had the highest association with low disease activity according to GRACE, followed by PASDAS, MDA, DAPSA, DAS28, with the lowest association for CPDAI. CONCLUSION: PASDAS and GRACE were superior with respect to responsiveness, and together with MDA best related to longitudinal evolvement of HRQOL, productivity and disability. Responsiveness and longitudinal validity of most outcomes were inferior for DAS28, DAPSA and CPDAI. As alternatives to the continuous measure DAPSA, use of PASDAS or GRACE should be considered.

Window of opportunity in psoriatic arthritis: the earlier the better?

OBJECTIVES: To investigate whether there is a window of opportunity for psoriatic arthritis (PsA) patients and to assess which patient characteristics are associated with a longer diagnostic delay. METHODS: All newly diagnosed, disease-modifying antirheumatic drug-naïve PsA patients who participated in the Dutch southwest Early PsA cohoRt and had ≥3 years of follow-up were studied. First, total delay was calculated as the time period between symptom onset and PsA diagnosis made by a rheumatologist and then split into patient and physician delays. The total delay was categorised into short (<12 weeks), intermediate (12 weeks to 1 year) or long (>1 year). These groups were compared on clinical (Minimal Disease Activity (MDA) and Disease Activity index for PSoriatic Arthritis (DAPSA) remission) and patient-reported outcomes during 3 years follow-up. RESULTS: 708 PsA patients were studied of whom 136 (19%), 237 (33%) and 335 (47%) had a short, intermediate and long total delay, respectively. Patient delay was 1.0 month and physician delay was 4.5 months. Patients with a short delay were more likely to achieve MDA (OR 2.55, p=0.003) and DAPSA remission (OR 2.35,p=0.004) compared with PsA patients with a long delay. Patient-reported outcomes showed numerical but non-significant differences between the short and long delay groups. Female patients and those presenting with enthesitis, chronic back pain or normal C-reactive protein (CRP) had a longer delay. CONCLUSIONS: In PsA, referral and diagnosis within 1 year is associated with better clinical outcomes, suggesting the presence of a window of opportunity. The most gain in referral could be obtained in physician delay and in females, patients with enthesitis, chronic back pain or normal CRP.

Determinants of radiographic progression in early psoriatic arthritis: insights from a real-world cohort.

OBJECTIVE: Persistent articular inflammation in psoriatic arthritis (PsA) is associated with radiographic damage. Despite advances in diagnosis and therapy, radiographic structural damage remains prevalent in PsA. To elucidate this topic, we studied which baseline clinical characteristics determine radiographic progression. METHODS: For this analysis, data were used from DEPAR (Dutch South West Psoriatic Arthritis) Study, a real-world cohort of patients with newly diagnosed PsA. Radiographic changes were assessed using the modified Total Sharp/van der Heijde Score (mTSS) for PsA. Univariable-multivariable mixed-effects negative binomial regression analysis was applied to define baseline predictors for radiographic progression over time. RESULTS: The study included 476 patients with early PsA with 1660 hand and feet radiographs from four different time points (baseline, first, second and third year). The progressive group (n=71) had a higher mTSS compared with the non-progressive group (n=405) at diagnosis (17 (3-36) vs 0 (0-1)). A comparison of the two groups revealed that the progressive group had significantly older (59 (12) vs 49 (13)) and a higher rate of the presence of swollen joints (93% vs 78%) at diagnosis. Multivariable analysis identified age (incidence rate ratio (IRR)=1.10, p=0.000), sex (female) (IRR=0.48, p=0.043) and baseline mTSS (IRR=1.11, p=0.000) as significant determinants of radiographic change over time. For the progressive subset, additionally, the multivariable analysis highlighted baseline Disease Activity in PSoriatic Arthritis (IRR=1.05, p=0.006) and swollen joint count (IRR=1.07, p=0.034) as predictors. CONCLUSIONS: According to this real-world cohort, patients with early PsA exhibit minimal radiographic progression under current treatment protocols. This study indicates that while old age and initial radiographic damage predict progression, female sex confers a protective effect on it. Furthermore, disease activity score and swollen joints emerged as predictors for radiographic changes during the follow-up in progressive patients.

Construct validity and responsiveness of feasible composite disease activity measures for use in daily clinical practice in patients with psoriatic arthritis.

OBJECTIVE: There is a need for a widely accepted comprehensive disease activity measure for use in daily practice in patients with psoriatic arthritis (PsA). For this reason, the 3-item Visual Analogue Scale (3VAS) and 4-item Visual Analogue Scale (4VAS) were developed. This study aimed to test construct validity and responsiveness of the 3VAS and 4VAS in a population of patients with newly diagnosed PsA receiving usual care. METHODS: Components of the 3VAS (physician global, patient global, patient skin) and 4VAS (physician global, patient pain, patient joint, patient skin) were scored on 0-10 VAS scales. Agreement of low disease activity (LDA) state between 3VAS/4VAS and other composite measures was tested using Venn diagrams. Construct validity and responsiveness (3-month interval) were assessed using Spearman correlation coefficients and standardised response means (SRM) with effect sizes (ES), respectively, following hypothesis generation. Both 3VAS/4VAS were also compared with several patient-reported outcome measures. RESULTS: Data from 629 patients were used. Both 3VAS (ES=0.48, SRM 0.52) and 4VAS (ES=0.48, SRM=0.50) showed responsiveness similar to Disease Activity in PSoriatic Arthritis (DAPSA) and Disease Activity Score-28 (DAS28). Both measures had a strong correlation with DAPSA (r=0.80-0.87), Psoriatic Arthritis Disease Activity Score (PASDAS) (r=0.89) and Routine Assessment of Patient Index Data 3 (RAPID3) (r=0.84-0.92). 3VAS and 4VAS had the highest agreement with PASDAS in categorising patients to LDA at 12 months. CONCLUSION: This is the first study assessing the performance of the 3VAS and 4VAS in an observational cohort of patients with early PsA. Both measures have promising performance characteristics, showing strong correlations and good discrimination with existing composite measures. The 4VAS may be the preferred version with better face validity.

A Practical Guide for Assessment of Skin Burden in Patients With Psoriatic Arthritis.

OBJECTIVE: Rheumatologists play a pivotal role in the management of patients with psoriatic arthritis (PsA). Due to time constraints during clinic visits, the skin may not receive the attention needed for optimal patient outcome. Therefore, the aim of this study was to select a set of core questions that can help rheumatologists in daily rheumatology clinical practice to identify patients with PsA with a high skin burden. METHODS: Baseline data from patients included in the Dutch South West Psoriatic Arthritis (DEPAR) cohort were used. Questions were derived from the Skindex-17 and Dermatology Life Quality Index (DLQI) questionnaires. Underlying clusters of questions were identified with an exploratory principal component analysis (PCA) with varimax rotation, after which a 2-parameter logistic model was fitted per cluster. Questions were selected based on their discrimination and difficulty. Subsequently, 2 flowcharts were made with categories of skin burden severity. Clinical considerations were specified per category. RESULTS: In total, 413 patients were included. The PCA showed 2 underlying clusters: a psychosocial domain and a domain assessing physical symptoms. We selected these 2 domains. The psychosocial domain contains 3 questions and specifies 4 categories of skin burden severity. The physical symptoms domain contains 2 questions and categorizes patients in 1 out of 3 categories. CONCLUSION: We have selected a set with a maximum of 5 questions that rheumatologists can easily implement in their consultation to assess skin burden in patients with PsA. This practical guide makes the assessment of skin burden more accessible to rheumatologists and can aid in clinical decision making.

Adenocarcinoma of the lower esophagus after placement of a gastric band.

A 43-year-old man presented with severe back pain. He had a history of morbid obesity, for which an esophagogastric silicone band was placed 2 years before presentation. Magnetic resonance imaging of the vertebral column showed multiple osseous metastases. On the computerized tomography scan of the abdomen, a tumor of the lower esophagus just proximal to the esophagogastric band was seen, of which histological examination revealed an esophageal adenocarcinoma. Esophageal adenocarcinoma after bariatric surgery has been described previously in only a few cases. Although there is no evidence for a causal relationship with bariatric surgery, one should bear in mind that the incidence of esophageal adenocarcinoma is increased in patients with morbid obesity because of the higher incidence of gastroesophageal reflux disease. Also, the symptoms of adenocarcinoma might be masked after bariatric surgery.

Time to minimal disease activity in relation to quality of life, productivity, and radiographic damage 1 year after diagnosis in psoriatic arthritis.

BACKGROUND: In a cohort of patients with newly diagnosed psoriatic arthritis (PsA) who received usual care, we investigated the impact of time elapsed to minimal disease activity (MDA) on health-related quality of life (HRQoL), work productivity, and radiographic damage throughout the first year after diagnosis. METHODS: Data collected in the Dutch southwest early PsA cohort (DEPAR) study were analyzed. These three-monthly data encompassed disease activity, HRQOL was measured with the Short Form 36 (SF36) Physical Component Scale (SF36-PCS) and Mental Component Scale, and productivity was measured with the Productivity Cost Questionnaire. Radiographic damage was scored at baseline and at 12 months with the PsA-modified Sharp/van der Heijde score. Patients were classified by time to MDA as in early (within 3 months), late (at 6-12 months), and never MDA in the first year. RESULTS: We included 296 patients who had had their 1-year outpatient visit (mean age 51 years, 53% male). Ninety-six (32%) were classified as early MDA, 78 (26%) as late MDA, and 98 (33%) as never MDA. Data of 24 patients (8%) were missing. SF36-PCS and productivity scores improved after gaining MDA, but remained low in never MDA patients. At 1 year, SF36-PCS and productivity scores were similar in early and late MDA patients. Radiographic progression rate was low and similar in all groups. CONCLUSION: Gaining MDA was associated with considerable improvement in HRQoL and functioning, irrespective of time to first MDA. In the one third of patients not in MDA in the first year, the disease had a substantial health impact.

A novel mutation in a Turkish patient with Hermansky-Pudlak syndrome type 5.

The Hermansky-Pudlak syndrome (HPS) is a rare genetically heterogeneous autosomal recessive disorder, characterized by tyrosinase-positive oculocutaneous albinism, platelet dysfunction and lysosomal ceroid lipofuscin storage. This is caused by defects in lysosome-related organelles. In humans eight different types of the syndrome are known, of which a short overview is given. The clinical features and a novel mutation of a patient with HPS type 5 are described here.

Influence of Disease Manifestations on Health-related Quality of Life in Early Psoriatic Arthritis.

OBJECTIVE: Psoriatic arthritis (PsA) is a multifaceted disease. Affecting joints, skin, entheses, and dactylitis, its effect on health-related quality of life (HRQOL) could be substantial. We aim to assess HRQOL in patients newly diagnosed with PsA and analyze its associations with disease manifestations. METHODS: Data collected at time of diagnosis from patients with PsA included in the Dutch south-west Early Psoriatic Arthritis cohort (DEPAR) study were used. HRQOL was assessed using 8 domains of the Medical Outcomes Study Short Form-36 (SF-36) questionnaire. Patients were classified based on primary manifestation in arthritis subtypes (i.e., mono-, oligo-, or polyarthritis) and other subtypes (i.e., enthesitis, dactylitis, and axial disease). In all patients, presence of arthritis, enthesitis, dactylitis, psoriasis, and chronic inflammatory back pain was determined. Multivariable linear regression was used to determine associations of PsA manifestations with HRQOL. RESULTS: Of 405 patients, primary manifestation was peripheral arthritis in 320 (78 monoarthritis, 151 oligoarthritis, and 91 polyarthritis), enthesitis in 37, axial disease in 9, and dactylitis in 39. Mean scores of SF-36 domains were lower than the Dutch reference population and similar across arthritis subtypes. A higher number of enthesitis locations and tender joints, and presence of chronic back pain, were independently associated with worse SF-36 scores. Psoriasis and dactylitis were not associated with worse scores. CONCLUSION: HRQOL was diminished in PsA at time of diagnosis compared to the Dutch reference population, and tender joints, enthesitis at clinical examination, and back pain as indicators of pain affected HRQOL.

Burden of Psoriatic Arthritis According to Different Definitions of Disease Activity: Comparing Minimal Disease Activity and the Disease Activity Index for Psoriatic Arthritis.

OBJECTIVE: Treat-to-target strategies have improved outcomes in rheumatic diseases. In psoriatic arthritis (PsA), the proposed targets are the multidimensional target minimal disease activity (MDA) and the articular target Disease Activity index for PsA (DAPSA). The aim of this study was to compare the disease burden of PsA in patients with low disease activity according to the 2 definitions, MDA and DAPSA low disease activity (DAPSA-LDA), 1 year after diagnosis. METHODS: We obtained data on MDA, DAPSA-LDA and disease burden 1 year after diagnosis for patients included in the Dutch southwest early PsA cohort. Disease burden was assessed in 2 domains: "Body functions," including the Short Form 36 bodily pain (SF-36 BP) measure, and "Activity," including the Health Assessment Questionnaire (HAQ). RESULTS: Among the 292 patients included, 48% achieved MDA and 74% achieved DAPSA-LDA. Average scores for Body functions and Activity were better in patients who achieved MDA and those who achieved DAPSA-LDA. The scores were significantly better in the 46% of patients who achieved both MDA and DAPSA-LDA than in the 29% of patients who achieved only DAPSA-LDA. The average SF-36 BP score was higher in patients achieving both targets (73.8; 95% confidence interval [95% CI] 71.1-76.5) than in patients achieving only DAPSA-LDA (57.6; 95% CI 54.5-60.8). Similarly, mean HAQ scores measuring Activity were 0.21 (95% CI 0.15-0.26) and 0.63 (95% CI 0.53-0.72), respectively. CONCLUSION: Among patients with newly diagnosed PsA, 48% achieved MDA and 74% achieved DAPSA-LDA after 1 year of receiving usual care. The average disease burden was better in patients who achieved MDA and those who achieved DAPSA-LDA. Also, patients who achieved only DAPSA-LDA reported worse outcomes than those who also achieved MDA.

Systemic lupus erythematosus (SLE): an unusual cause of ileocolic intussusception.

Intussusception is a rare diagnosis in adults. Gastrointestinal involvement is not unusual in systemic lupus erythematosus (SLE).1 A case of intussusception as first presenting symptom of SLE is described. A 50-year-old woman of Chinese origin came to the emergency room with symptoms of high fever, nausea, vomiting and weight loss of 10 kg. Three months before, an episode with abdominal pain due to ileal invagination in the colon occurred. Laboratory analysis showed: thrombocytopaenia, lymphopaenia, positive antinuclear antibody, antidouble-stranded DNA, Coombs tests and low C3 in combination with an active urine sediment and proteinuria. CT and positron emission tomography scan showed lymphadenopathy, but no other abnormalities. Axillar lymph node biopsy showed no abnormalities. The diagnosis new-onset SLE was made. Prednisone treatment was started and soon thereafter tapered, azathioprine and hydroxychloroquine were added. During follow-up, the SLE remained in remission. SLE is a rare cause of ileocolic intussusception and ileocolic intussusception may be the first presenting symptom.

Painful knees and hearing-loss: a rare presentation of meningococcal disease.

A 49-year-old woman with a medical history of rheumatoid arthritis presented to the emergency room, with high fever and painful knees. In addition, she had had a mild headache for several days and some hearing loss over several months. We saw an ill patient with arthritis of both knees, from which purulent fluid was aspirated. Antibiotics were started for septic arthritis of both knees and her condition improved rapidly. However, the headache persisted and the hearing loss worsened. At the time, meningitis was suspected. Initial knee aspiration culture was positive for Neisseria meningitidis PCR of the cerebrospinal fluid sample also was positive for N. meningitidis The patient was finally diagnosed with bilateral septic gonarthritis secondary to a bacterial meningitis caused by N. meningitidis.

Levamisole-contaminated cocaine: a hairy affair.

Levamisole-contaminated cocaine can induce severe systemic vasculitis. The diagnosis can be challenging, especially when substance abuse is uncertain. We present the case of a 42-year-old woman suffering from vasculitis due to levamisole-contaminated cocaine, who persistently denied substance abuse. Symptoms included ulcerating skin lesions, arthralgia and myalgia, and the occurrence of an ileal intussusception. The definitive diagnosis was made using hair testing for toxins. She recovered through cocaine abstinence, but re-exposure resulted in a severe relapse with glomerulonephritis. Importantly, at time of the relapse, the patient became positive for both myeloperoxidase-antineutrophil cytoplasmic antibody (ANCA) and proteinase 3-ANCA. Cocaine-levamisole-induced vasculitis poses a great clinical challenge. The proper diagnostic strategy and therapy is still controversial. We highlight our diagnostic and therapeutic considerations, including hair testing for definitive proof of exposure.

A second primary tumor in a patient with retinoma.

AIM: To evaluate the frequency of the co-occurrence of a retinoma and another primary tumor. METHOD: Presentation of a case report and review of the literature. RESULTS: A cutaneous melanoma was observed in a 52-year-old man, who was known to have two retinomas in the right eye. Five other cases were found in a review of the literature, suggesting the possibility of an increased risk of developing a second primary tumor in patients with retinoma. The occurrence of cutaneous melanoma as a second primary tumor after retinoma and/or retinoblastoma is discussed. CONCLUSION: There might be an increased risk for patients with retinoma to develop another primary tumor, necessitating regular follow-up of these patients.