Lung and Pleural Immunoglobulin G4-Related Disease: Two Contrasting Case Reports.

Immunoglobulin G4-related disease (IgG4-RD) is an immunomediated disease that can virtually affect any organ. Despite the pancreas being known as the most frequently involved organ, pulmonary and pleural IgG4-RD is being increasingly reported. The authors present two cases of IgG4-RD diagnosed in the same year, with different presentations and outcomes, in which the lung and pleural involvement were essential for the diagnosis. Recognizing IgG4-RD as a possible cause of chronic pleural effusion and/or thickening and lung abnormalities is important for an early diagnosis and prognosis improvement.

Post-infectious digital ischemia successfully treated with iloprost in a child / Isquemia digital postinfecciosa infantil tratada con éxito con iloprost

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The role of opioid analgesics in rheumatic disorders: a position paper from the Portuguese Rheumatology Society.

Pain is a common feature of most rheumatic diseases and it is often the main reason for the patient to seek for a clinical appointment. Chronic pain has a major impact on patient's quality of life, being frequently associated with functional incapacity, sleep and mood disorders. This leads to absenteeism and heavy consumption of health resources, both representing huge burdens on national economy. Managing musculoskeletal pain is pivotal but can be challenging. The use of the available pharmaceutical armamentarium should be parsimonious. Opioids are strong analgesic drugs that mostly act through their agonist action on µ-receptors in the central nervous system. Opioid-related side effects are not negligible and are mediated through both central and peripheral opioid receptors. The use of opioids is well established in the treatment of oncologic pain but their role in the management of musculoskeletal pain is still controversial. Inflammatory rheumatic diseases, osteoarthritis, osteoporotic fractures, chronic low back pain and fibromyalgia represent diverse major rheumatic conditions that frequently lead to chronic pain. In order to standardize and optimize management of musculoskeletal chronic pain in these prevalent diseases, the Portuguese Rheumatology Society elaborated this position paper. The objectives were: a) to define the importance of pain assessment and classification; b) to guide patient selection, appropriate choice of opioids, their management, and raise awareness of their adverse effects; c) to review the existent data on possible indications of opioid therapy on rheumatic diseases.

Cat scratch disease during adalimumab therapy: a case report / Enfermedad por arañazo de gato durante el tratamiento con adalimumab: a propósito de un caso

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Validation of Rheumatoid Arthritis Quality of Life (RAQoL) Questionnaire into Portuguese Language.

BACKGROUND: Rheumatoid Arthritis (RA) is a chronic inflammatory disease with high impact on patients´ quality of life (QoL). The Rheumatoid Arthritis Quality of Life Questionnaire (RAQoL) is a self-completed questionnaire designed to assess QoL in patients with RA. A Portuguese adaptation of the RAQoL was available but required formal validation. AIM: To validate the RAQoL into the Portuguese language. METHODS: Patients with diagnosis of RA were included. The Portuguese RAQoL was administered on two occasions, 14 days apart. Participants also completed the Nottingham Health Profile (NHP) and Health Assessment Questionnaire (HAQ) at Time 1. Internal consistency was assessed using Cronbach's alpha and reliability through Spearman's Correlation Coefficient. Construct validity was evaluated through Spearman's correlation analyses between RAQoL, NHP and HAQ. Known-group validity was tested comparing the RAQoL across different groups, considering self-perceived general health status, flare symptoms and RA severity through non-parametric tests. RESULTS: 178 RA patients (mean age 56.6, 82% female, mean disease duration 13.6 years) were included. The mean score of RAQoL was 11, with low rates of missing data (3.9%), floor (4.5%) and ceiling (2.2%) effects. Cronbach's α was 0.95 and test-retest reliability 0.92. RAQoL scores correlated moderately with scores on the NHP Physical Mobility (r=0.77), Emotional reactions (r=0.69), Pain (r=0.68) and Energy (r=0.65) section scores. RAQoL showed to be discriminative for groups according to self-perceived general health status, flare of arthritis and disease severity. CONCLUSION: The Portuguese version of the RAQoL is a valid, feasible and reliable questionnaire, suitable for use in clinical practice and research purposes.

New applications of sonoelastography in rheumatology: where are we now?

Ultrasound elastography (UE) is a non-invasive imaging method that allows the assessment of tissue elastic property. Different UE techniques are currently available (i.e. strain UE and acoustic radiation force impulse UE), with several potential clinical applications. Recent studies investigated the role of UE in two systemic rheumatic diseases and psoriasis. This research added interesting information to the already known applications of UE in the assessment of tendinopathies. In SS, acoustic radiation force impulse UE has shown a potential role in the diagnosis of the disease, with lower sensitivity than and similar specificity to salivary gland histology. In SSc, a potential use of UE in screening pre-clinical disease has been reported. In psoriasis, the use of strain UE in evaluating treatment response has been highlighted. UE is a promising tool in rheumatology, with a potential role in the evaluation of various tissues and pathologies.

Intra-articular epithelioid sarcoma of the knee: a diagnostic challenge.

Epithelioid sarcoma is a rare mesenchymal neoplasm characterized by aggregates of epithelioid cells. Intra-articular occurrence is exceedingly rare with only few reports described in the literature. A 22 year-old man presented a progressive mechanical knee pain. Initially, the investigation revealed a non-infectious unspecific synovitis. The patient gradually presented increasing knee enlargement and functional impairment. Intra-articular nodular proliferation with bone invasion was later observed on magnetic resonance imaging reevaluation. Pigmented villonodular synovitis hypothesis was considered. The biopsy ultimately revealed nodules of epithelioid cells with an immunoprofile compatible with epithelioid sarcoma diagnosis. The patient underwent neoadjuvant chemotherapy and radiotherapy and an above-knee amputation was performed.

Determinants of non-nociceptive pain in Rheumatoid Arthritis.

INTRODUCTION: Features suggestive of neuropathic pain (NP) have been described in RA in addition to nociceptive pain. We aimed to determine the clinical predictors of NP in RA patients and study its association with radiographic structural damage. METHODS: Cross-sectional study was performed with RA patients followed at our Rheumatology department. Patients with diagnosed neuropathy of other origin, non-RA related risk factors for NP (e.g. diabetes mellitus) or fibromyalgia according to expert opinion were excluded. Demographic and clinical data were collected and disease activity/functional measures were evaluated. Two questionnaires were applied to assess NP: the Leeds Assessment of Neuropathic Symptoms (LANSS) and the painDETECT questionnaire (PDQ). Radiographs performed in up to 12 months before/after the evaluation were classified according to the modified van der Heijde Sharp´s method. Univariate and multivariate logistic regression were performed to identify the predictors of NP. RESULTS: 112 patients were included. 86 (77%) were women, with a mean (SD) age of 55.1 (10.8) years and median disease duration of 13 [2-41] years. 45 (40%) patients had NP by the LANSS (≥12) and 28% had a possible/likely NP in the PDQ (≥13). Female sex was predictive of NP by both tests and disease duration was inversely associated with LANSS NP. After adjusting for those two variables, pain VAS and TJC were positive predictors of NP by both tests. The same was not true for SJC, ESR or CRP levels. DAS28-CRP was significantly associated with PDQ NP, losing its statistical significance after adjustment for TJC and pain VAS. The HAQ score increased the odds of NP for both tests, independently of DAS 28-CRP. Positivity for ACPA and previous/current hydroxychloroquine treatment had lower odds of NP. 90 patients performed radiographic evaluation. Joint narrowing score was a significant negative predictor of LANSS NP. After adjusting for global radiographic score, current methotrexate treatment had lower odds of LANSS NP and previous/current leflunomide was a positive predictor of NP by both tests. CONCLUSION: NP was associated with disease activity/functional scores but not with objective inflammatory measures. Greater structural damage, increased disease duration and ACPA positivity did not seem to increase the odds of NP. Possible association of NP and underlying csDMARD treatment was uncovered.