Stefania Vio

Anterior Chest Wall Involvement in Early Stages of Spondyloarthritis: Advanced Diagnostic Tools

Objective. Anterior chest wall (ACW) involvement is difficult to evaluate in patients with spondyloarthritis (SpA). Bone scan is sensitive to ACW involvement, while magnetic resonance imaging (MRI) detects early alterations in SpA. We compared the sensitivity and specificity of bone scans and MRI in assessing ACW in early SpA. Methods. Out of 110 patients with early SpA attending the Outpatient Rheumatology Unit Clinic of Padua University from January 2008 to December 2010, the 40 complaining of pain and/or tenderness [60% with psoriatic arthritis (PsA), 12.5% with ankylosing spondylitis, and 27.5% with undifferentiated SpA] underwent bone scans and MRI. Results. At clinical examination, sternocostoclavicular joints were involved in 87.5% on the right, 77.5% on the left, and 35% on the sternum. Bone scan was positive in 100% and MRI in 62.5% of these patients. Early MRI signs (bone edema, synovial hyperemia) were observed in 27.5%, swelling in 5%, capsular structure thickness in 37.5%, erosions in 15%, bone irregularities in 15%, osteoproductive processes in 12.5%, and osteophytes in 5%. A higher prevalence of Cw6, Cw7, B35, and B38 was found in 15%, 48%, 28%, and 12%, respectively, of the patients with PsA who had bone scans. Conclusion. Noted mainly in women, ACW involvement was frequent in early SpA. Both bone scans and MRI are useful in investigating ACW inflammation. Bone scans were found to have high sensitivity in revealing subclinical involvement, but a low specificity. MRI provides useful information for therapeutic decision making because it reveals the type and extent of the process. The significant associations of HLA-Cw6 and Cw7 with PsA could suggest that genetic factors influence ACW involvement.

Joint and bone assessment in hand osteoarthritis

Hand osteoarthritis (OA) is a common disease frequently affecting middle-aged women. Prevalence estimates for OA vary widely depending on the age and sex of the population studied, the assessment tools used, and the specific joint sites analyzed OA is characterized by the degradation of articular cartilage, subchondral bone changes and osteophyte formation at the joint margins leading to joint failure. The pathogenesis of the disease and its evolution are multifactorial involving biomechanical, metabolic, hormonal, and genetic factors. Moreover, the role of inflammation has recently been advanced as pivotal in OA onset and progression. In particular, an uncommon variant of hand OA, erosive hand OA, is characterized by inflammatory and degenerative interphalangeal proximal and distal joints. The diagnosis of different types of hand OA is centered on clinical and laboratory investigations which can distinguish the peculiar aspects of these forms. Joint and bone assessments in hand OA are widely studied but there is no agreement with regard to established parameters to make a definitive diagnosis. This report focuses on the laboratory and clinimetric assessments that can be used to distinguish hand OA subtypes and addresses the debatable association with low bone mineral density in osteoporosis.

Spine and sacroiliac joints on magnetic resonance imaging in patients with early axial spondyloarthritis: prevalence of lesions and association with clinical and disease activity indices from the Italian group of the SPACE study

Our aim was to determine the prevalence of spine and sacroiliac joint (SIJ) lesions on magnetic resonance imaging (MRI) in patients with early axial spondyloarthritis (axSpA) and their correlation with disease activity indices. Sixty patients with low back pain (LBP) (≥3 months, ≤2 years, onset ≤45 years), attending the SpA-clinic of the Unità Operativa Complessa Reumatologia of Padova [SpondyloArthritis-Caught-Early (SPACE) study], were studied following a protocol including physical examination, questionnaires, laboratory tests, X-rays and spine and SIJ MRI. Positive spine and SIJ MRI and X-rays images were scored independently by 2 readers using the SPARCC method, modified Stoke ankylosing spondylitis spine score and New York criteria. The axial pain and localization of MRI-lesions were referred to 4 sites: cervical/thoracic/lumbar spine and SIJ. All patients were classified into three groups: patients with signs of radiographic sacroiliitis (r-axSpA), patients without signs of r-axSpA but with signs of sacroiliitis on MRI (nr-axSpA MRI SIJ+), patients without signs of sacroiliitis on MRI and X-rays (nr-axSpA MRI SIJ-). The median age at LBP onset was 29.05±8.38 years; 51.6% of patients showed bone marrow edema (BME) in spine-MRI and 56.7% of patients in SIJ-MRI. Signs of enthesitis were found in 55% of patients in the thoracic district. Of the 55% of patients with BME on spine-MRI, 15% presented presented a negative SIJMRI. There was a significant difference between these cohorts with regard to the prevalence of radiographic sacroiliitis, active sacroiliitis on MRI and SPARCC SIJ score. The site of pain correlated statistically with BME lesions in thoracic and buttock districts. Since positive spine-MRI images were observed in absence of sacroiliitis, we can hypothesize that this finding could have a diagnostic significance in axSpA suspected axSpA.

BCR kinase inhibitors, idelalisib and ibrutinib, are active and effective in Richter syndrome

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Splenic marginal zone lymphoma with a de novo t(8;14)(q24;q32) and a prolymphocytoid evolution responsive to rituximab-bendamustine

Dear Editor, Splenic marginal zone lymphoma (SMZL) is a low-grade B cell non-Hodgkin lymphoma (NHL) characterized by the involvement of the spleen, bone marrow, and peripheral blood [1, 2]. Cytogenetically, deletion or translocation of 7q32 is reported in 40% of patients, complete or partial trisomy of 3q in 30–80% and gain of 12q in 15–20% [3–5]. While a t(8;14) IGH/MYC may occur in the setting of transformation to a high-grade NHL [6], these alterations are extremely rare at diagnosis [7]. Also, a prolymphocytic evolution has been rarely recognized and it has been associated with a poor outcome [8]. We describe here a unique case of SMZL that displayed concurrently a t(8;14)(q24;q32) at diagnosis and a prolymphocytic evolution. In a 51-year-old female admitted to the Padua University Hospital for recurrent bronchopneumonitis, a clonal B lymphocytosis of CD5small mature cells with compacted nuclear chromatin, moderately abundant slightly basophilic cytoplasm and typical membrane blebs and protrusions in peripheral blood and in the bone marrow (5–10% infiltrate) was detected (Fig. 1a, b). A CT scan was negative. The patient was asymptomatic and followed over time. Reassessment after 5 years showed spleen enlargement (18 cm longest axis) and a bone marrow 70% infiltrate of CD20 clonal B cells (Fig.1c). A cytogenetic analysis revealed a clone of 47 chromosomes characterized by a balanced chromosomal translocation t(8;14)(q24;q32) accompanied by a trisomy 12: 47,XX,t(8;14)(q24;q32),+12 [25]. FISH analysis with locus-specific probes for MYC and IGH genes (LSI IGH/MYC, CEP8 tricolor, dual fusion translocation, Abbott, Des Plaines, IL, USA) confirmed the presence of IGH/MYC rearrangement in metaphase on derivative chromosome 14 as well as on derivative chromosome 8 in this sample and retrospectively in interphase, on frozen cells from the diagnosis (Fig. 1d–f). The subsequent clinical course was character ized by the emergence of a fast-growing prolymphocytoid cells exceeding 80% of total peripheral blood lymphocytes (Fig. 1g), mild anemia, thrombocytopenia, and hypogammaglobulinemia. The clinical behavior remained indolent and the disease was responsive to treatment with rituximab 375 mg/m on day 1 plus bendamustine 90 mg/m on days 1 and 2 every 4 weeks for a total of 6 cycles (Fig. 1h, i). The disease remains in remission after 3 years. To the best of our knowledge, before our observation, only one case of SMZL with a de novo t(8;14)(q24;q32) has been described [7]. Similarly, as in our case, the clinical, histopathological, and laboratory features of the SMZL were typical and no aggressive course was observed. However, our case displayed a further exceptionally rare feature for a SMZL, i.e., a prolymphocytoid evolution. To this regard, only a series of four cases of clear prolymphocytoid transformation of SMZL has been reported in the literature [8]. * Francesco Piazza francesco.piazza@unipd.it

Biomarkers, imaging and disease activity indices in patients with early axial spondyloarthritis: the Italian arm of the SpondyloArthritis-Caught-Early (SPACE) Study

The study aimed to evaluate biomarkers facilitating early diagnosis of axial spondyloarthritis (axSpA) and correlations between them and disease activity parameters and imaging indexes. Patients with low back pain (LBP) (≥3 months, ≤2 years, onset ≤45 years) participating in the Italian arm of the SpondyloArthritis-Caught-Early SPACE study underwent a physical examination, questionnaires, laboratory tests, X-rays and MRI of the spine and sacroiliac joints (SIJ). An expert rheumatologist formulated axSpA diagnosis in accordance with Assessment of SpondyloArthritis International Society (ASAS) criteria. Disease activity and physical functioning were assessed using imaging, clinical and serological indices. Spine and SIJ MRI and X-rays were scored independently by 2 readers using the SPARCC, mSASSS and NY-criteria. Patients were classified as: subjects with signs of radiographic sacroiliitis (r-axSpA), subjects with signs of sacroiliitis on SIJ-MRI but not on X-rays (nr-axSpA MRI SIJ+) or subjects with no signs of sacroiliitis on MRI/X-rays but with >2 SpA features and signs of bone oedema on MRI spine (nr-axSpA MRI SIJ-/undifferentiated SpA). Significant differences were found in the prevalence of radiographic sacroiliitis, active sacroiliitis on MRI and SPARCC SIJ scores. Biomarker levels were not significantly increased in any of the patient groups. The correlations between IL-17 and IL-23 and other indices were not significant; correlations were found between IL-22 and BASFI, BASG1, HAQ, VAS pain, between mSASSS and MMP3, and between the latter and hsCRP. Although not significantly higher in any of the three groups, IL-22, MMP3 and hsCRP values were correlated with some disease activity indexes and with mSASSS. Large observational studies are required to confirm these preliminary findings.

Spontaneous pneumomediastinum complicating severe acute asthma exacerbation in adult patients

ABSTRACT Objectives: The real incidence of pneumomediastinum (PNM) in adult patients with severe acute asthma exacerbation continues to be unknown. The current study aims to investigate the occurrence of PNM in an adult population of patients presenting a severe asthma attack and to evaluate the risk factors associated to its development. Methods: The 45 consecutive subjects who were admitted to our Division between January 1, 2015 and December 31, 2016 for severe acute asthma exacerbation underwent a diagnostic protocol including a standard chest X-ray and continuous monitoring of arterial oxygen saturation (SaO2) during the first 24 hours following admission. The patients showing persistence or deterioration of oxyhemoglobin desaturation were prescribed a chest Computed Tomographic (CT) scan. Results: Five out of the 45 patients (11.1%) with severe acute asthma exacerbation were diagnosed with PNM, in one case on the basis of an X-ray image and in four on the basis of a chest CT scan. Data analysis showed that the PNM patients were younger [21 (17–21) vs 49.5 (20–73) yrs; p < 0.001] and more likely to show sensitization to Alternaria (2/5 vs 0/40; p = 0.0101) with respect to their non-PNM counterparts. The duration of hospital stay was similar in the two groups [8 (4–12) vs 7 (3–15) days; p = 0.6939]. Conclusions: PNM is a common clinical entity in young adults with severe acute asthma exacerbation, particularly in those with unsatisfactory response to initial medical therapy. Although generally benign, patients with suspected PNM should be closely monitored because of the risk of developing severe hypoxemia.

Low back pain in young women: an unusual case.

Paola Frallonardo. This is a case of a 28-year-old woman from Syria who was admitted to the Rheumatology Unit of the University of Padua Medical Center, Italy, because of severe low back pain (LBP). Laboratory investigations were performed to define the diagnose. The magnetic resonance (MR) images showing bone marrow involvement, could be suggestive of acute infarction. The hyperpyrexia, the elevation of LDH, ALP, AST and ALT levels, the reduction in the aptoglobina, the hyperbilirubinemia, noted at the 5th day of hospitalization, were suggestive of hemolitic anemia. The suspicion led us to evaluate the haemoglobin electrophoresis and the sickle cell plus beta (HbS/β+)-thalassemia became evident. This case emphasizes the importance of maintaining a broad differential diagnosis for a diffuse musculoskeletal disorder such as LBP. Therefore, in this case report, the clinical and instrumental data together with the laboratory investigations were useful to define the undiagnosed mild HbS/β+ thalassemia in geographic areas without newborn screening.