Identification and characteristics of patients with potential difficult-to-treat Psoriatic Arthritis: exploratory analyses of the Greek PsA registry.

OBJECTIVE: To present the characteristics of patients with potential difficult-to-treat (D2T) psoriatic arthritis (PsA). METHODS: We used data from the Greek multicentre registry of PsA patients. D2T-PsA was defined as follows: patients with at least 6-months disease duration, who have failed to at least 1 csDMARD and at least 2 bDMARDs/tsDMARDs with a different mechanism of action and have either at least moderate disease activity (MODA) defined as DAPSA > 14, and/or are not at minimal disease activity (MDA). Demographic and clinical characteristics were compared between D2T and non-D2T PsA patients. In two sensitivity analyses, patients classified as D2T solely according to the MODA or MDA criterion were examined separately. RESULTS: Among 467 patients included, 77 (16.5%) were considered D2T and 390 non-D2T PsA. Compared with non-D2T, patients with D2T PsA presented more commonly with extensive psoriasis (p< 0.0001) and were more likely to have higher BMI (p= 0.023) and a history of inflammatory bowel disease (p= 0.026). In the MODA and MDA sensitivity analyses, 7.5% and 12.5% of patients were considered D2T, respectively. In both sensitivity analyses, extensive psoriasis was again identified as an independent variable for D2T PsA (p= 0.001 and p= 0.008, respectively). Moreover, female gender (p= 0.034) in the MODA analysis and axial disease (p= 0.040) in the MDA analysis were independent variables for D2T PsA. CONCLUSION: Despite the availability of therapies, D2T PsA is common in real-life cohorts of patients with PsA and extensive psoriasis. High BMI, female gender, axial-disease, and history of IBD were also associated with D2T PsA.

Disease Profile and Achievement of Therapeutic Goals in a Modern, Nationwide Cohort of 923 Patients with Psoriatic Arthritis.

Background: Psoriatic arthritis (PsA) is a heterogenous chronic inflammatory disease affecting skin, joints, entheses, and spine with various extra-musculoskeletal manifestations and comorbidities. The reported patient, disease and treatment characteristics in the modern therapeutic era are limited. Methods: In this cross-sectional, multi-centre, nationwide study, we recorded the demographic, clinical, and therapeutic characteristics as well as the comorbidities of patients with PsA seen for 1 year (1/1/2022-31/12/2022). Results: 923 patients (55% females) with a median (IQR) age of 57 (48-65) years and a mean disease duration of 9.5 years were enrolled. Family history of psoriasis and PsA was noted in 28.3% and 6.3%, respectively. Most patients had limited psoriasis (BSA<3: 83%) while enthesitis, dactylitis, nail and axial involvement reported in 48.3%, 33.2%, 43% and 25.9% of patients, respectively. Regarding comorbidities, approximately half of patients had dyslipidaemia (42%) or hypertension (45.4%), 36.8% were obese and 17% had diabetes while 22.7% had a depressive disorder. Overall, 60.1% received biologics and among them more patients treated with anti-IL-17 or -12/23 agents were on monotherapy (64.2%) compared to those on TNFi monotherapy (49.4%, p=0.0001). The median PsA activity as assessed by the DAPSA score was 6 (IQR: 2.3 - 13.1) with 46% of patients reaching minimal disease activity status (MDA). Conclusion: In this large, real life, modern cohort of patients with PsA with frequent comorbidities who were treated mainly with biologics, almost half achieved minimal disease activity. These results show the value of existing therapeutic approaches while at the same time highlight the existing unmet needs.

Polycythemia Vera in a Patient With Heterozygous Beta-Thalassemia: Coincidence or Causal Relationship?

Polycythemia vera (PV) and heterozygous beta-thalassemia (HBT) have opposing effects on the hematocrit (Hct) and may mask the presence of each other. Missing the diagnosis of PV may have serious consequences, mainly by exposing the patient to the risk of thromboses. We present a case where the diagnosis of PV was delayed due to the coexistence of HBT, and review the relevant literature. It can be postulated that "stress erythropoiesis", known to occur in patients with thalassemic syndromes, increases the probability of somatic JAK2 mutations leading to development of PV.

Differential performance of nailfold video capillaroscopic parameters in the diagnosis and prognosis of systemic sclerosis.

OBJECTIVES: The contribution of nailfold video capillaroscopy (NVC) in identifying patients with Raynaud's phenomenon (RP) at risk for systemic sclerosis (SSc) is well established. Herein we comparatively assess the performance of different capillaroscopic parameters in diagnosing SSc among patients with RP and evaluate the prognostic capacity of NVC in SSc. METHODS: At baseline we clinically and capillaroscopically evaluated 242 consecutive patients referred to our department for NVC (138 with SSc); 175 were reevaluated after 3.38±1.47 years. Sixty-two healthy volunteers served as controls. Capillaroscopy pattern (normal/early/active/late) was qualitatively defined. Capillary loss, dilated, giant or ramified capillaries and micro-haemorrhages were scored semi-quantitatively. RESULTS: Capillary loss score had the highest diagnostic accuracy at discriminating patients with an SSc-spectrum disorder from patients with RP of different etiology and controls, as defined by ROC curve analysis [AUC (95% CI)=0.905 (0.869-0.942)], followed by dilatation score [0.863 (0.818-0.907)] and giant score [0.835 (0.787-0.884)]. By contrast, micro-haemorrhages [0.720 (0.662-0.779)] and ramifications scores [0.604 (0.539-0.670)] performed worse. Multivariate analysis in 94 SSc patients indicated that active (OR=3.305, p=0.043) and late (OR=6.900, p=0.023) baseline capillaroscopy pattern predicted occurrence of a combined adverse disease outcome [forced vital capacity (FVC) deterioration>10% and/or DLCO deterioration>15% and/or mRSS deterioration>3.5 and/or first occurrence of digital ulcers and/or death)] at 3 year follow-up. CONCLUSIONS: Dilatation score performs best of all semi-quantitative NVC parameters in diagnosing SSc. In addition, our study confirms earlier reports that worse capillaroscopy pattern at baseline correlates with higher likelihood for adverse prognosis.