Ultrasound characterization of the nail bed in patients with systemic lupus erythematosus.

OBJECTIVE: To characterize the ultrasound findings of the nail plate and nail bed in systemic lupus erythematosus (SLE) and its association with nail dystrophy. METHODS: Thirty-two SLE patients, 36 patients with osteoarthritis (OA) and 20 healthy individuals were studied. High-frequency linear ultrasound was performed in nails of the second to fifth fingers in all participants. Disease activity (SLEDAI-2K index), accrued organ damage (SLICC/ACR index), autoantibody profile, and Raynaud's phenomenon were also assessed in SLE patients. RESULTS: Nail bed thickness in SLE patients was higher than in healthy individuals (1.25 ± 0.31 mm vs 1.17 ± 0.29 mm; P = 0.01) but lower than in OA (1.39 ± 0.37 mm; P < 0.001), while nail plate thickness was similar among groups. Nail dystrophy was found more frequently in SLE and OA than in healthy individuals. SLE patients with nail dystrophy were older than their counterparts with no dystrophy (39.4 ± 10.4 years vs 27.8 ± 5.6 years; P = 0.004), although nail dystrophy showed no association with SLICC/ACR, SLEDAI-2K, nail bed vascularity, or autoantibodies. CONCLUSIONS: Nail bed in SLE patients is thicker than in healthy individuals but thinner than in OA patients. Nail dystrophy in SLE is associated with advanced age, but not with accrued organ damage, disease activity, Raynaud's phenomenon, or DIP synovitis assessed by ultrasound.

Pachymeningitis in granulomatosis with polyangiitis: case series with earlier onset in younger patients and literature review.

The objective of this study is to describe the characteristics of patients with pachymeningitis (PM) in granulomatosis with polyangiitis (GPA) from Latin America, including three young patients. This is a retrospective case series. Patients were classified according to the ACR criteria, the 2012 Chapel Hill Consensus Conference Nomenclature and the EMA algorithm. Demographic, clinical, serological, and neuroimaging characteristics are described. Thirteen patients (nine females, four males) were identified. Mean age ± SD of PM diagnosis was 35.5 ± 20.4 years (median 48, range 8-71 years). Mean time ± SD between GPA first symptom and PM diagnosis was 59.8 ± 70.1 months (median 48, range 2-252 months). An important difference between children and adults was the median time elapsed between first GPA symptoms and PM diagnosis (range 2-4 months vs 5-252 months, respectively). Chronic headache was present in all, followed by intracranial hypertension (n = 5), single cranial nerve palsy and orbital mass (n = 4), seizures (n = 3), cavernous sinus syndrome and multiple cranial nerve palsies (n = 2), and meningism and cerebellar syndrome (n = 1 each). At time of PM diagnosis, mean BVAS/WG (Birmingham Vasculitis Activity Score for Wegener's granulomatosis) was 4 ± 2.4 and mean VDI of 2 ± 1.6, mostly due to ENT damage. Gadolinium-enhanced brain MRI showed dural thickening in 12 patients and leptomeningeal enhancement in one. All received a combination of glucocorticoids plus immunosuppressants, rituximab being used favourably in one refractory case. Improvement was observed in 12 patients. Chronic headache should lead to suspect PM. PM predominates in localised GPA. Children may present it earlier in the disease course than adults. Treatment is non-standardised and remains difficult.

Anti-Ro/SSA antibodies are associated with severe mitral valve regurgitation in patients with systemic lupus erythematosus.

OBJECTIVE: To assess whether anti-Ro/SSA antibodies are associated with cardiac valve disease in lupus. METHODS: A single-center, medical chart review was performed. Lupus patients were divided according to its anti-Ro/SSA status and subgroups were compared for valvular abnormalities and other characteristics. Dependence of anti-Ro/SSA reactivity to anti-Ro52/TRIM21 antibodies was also evaluated. RESULTS: Eighty-nine lupus patients were analyzed. The most common valvular abnormalities were tricuspid (60%), mitral (41%) and pulmonary (14%) regurgitation. Thirty-six patients were positive and 53 negative for anti-Ro/SSA antibodies. In patients positive to anti-Ro/SSA, a difference was noted for anti-dsDNA (67 versus 45%; p = 0.04) and anti-La/SSB (19 versus 2%; p = 0.004) antibodies. An association between anti-Ro/SSA antibodies and severe mitral regurgitation was observed; indeed, 4/15 patients with anti-Ro/SSA and mitral regurgitation had severe forms of valvulopathy as compared to only 1/22 patients with mitral regurgitation but negative to such antibody (27 versus 5%; p = 0.02). Anti-Ro/SSA antibodies significantly elevated the risk of severe mitral regurgitation (OR = 5). Anti-Ro52/TRIM21 levels (103 ± 29 versus 42 ± 43 U/mL; p = 0.03) and anti-Ro52/TRIM21: anti-Ro/SSA ratios (0.88 ± 0.02 versus 0.35 ± 0.37; p = 0.03) were higher in patients with mitral valve regurgitation than in those with no valvulopathy. CONCLUSION: Anti-Ro/SSA antibodies, mainly against Ro52/TRIM21 antigens, may be pathologically involved in lupus-associated mitral valve regurgitation.

Performance of the 2012 Systemic Lupus International Collaborating Clinics and the 1997 American College of Rheumatology classification criteria for systemic lupus erythematosus in a real-life scenario.

OBJECTIVE: To evaluate the performance of the 2012 Systemic Lupus International Collaborating Clinics (SLICC) criteria in classifying systemic lupus erythematosus (SLE) in an uncontrolled real-life scenario. METHODS: Chart review study was performed in which each criterion from the 1997 American College of Rheumatology (ACR) and the 2012 SLICC criteria to classify SLE was applied to patients from an outpatient rheumatology clinic. The clinical diagnosis was used as the gold standard. RESULTS: The sensitivity and specificity of the 2012 SLICC criteria were 92% and 99%, respectively, compared with the 1997 ACR criteria, which were 97% and 99%, respectively. The 2012 SLICC criteria were similar to the 1997 ACR criteria in terms of positive (98.9% versus 99%) and negative (92.5% versus 97.1%) predictive values as well as positive (92 versus 97) and negative (0.08 versus 0.03) likelihood ratios. A concordance of 0.96 (95% confidence interval [95% CI] 0.92­1.00) was observed between clinical diagnosis and the 1997 ACR criteria, while the concordance was 0.91 (95% CI 0.85­0.97) for the 2012 SLICC criteria. Seven SLE patients classified by the 1997 ACR criteria did not meet the 2012 SLICC criteria because of either the new definition for lymphopenia (2 patients) or the presence of isolated cutaneous involvement (5 patients), while 2 SLE patients who were classified by the 2012 SLICC criteria did not meet the 1997 ACR criteria because of either the presence of erosive arthritis or biopsy-proven nephritis with circulating antinuclear antibodies. CONCLUSION: Overall, the 1997 ACR and the 2012 SLICC criteria are similar to classify SLE in an uncontrolled real-life scenario, although several new items contained in the 2012 SLICC criteria could represent a step forward for research purposes in selected clinical settings.