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Background/objective: The 2019 American College of Rheumatology/European League Against Rheumatism Classification Criteria (2019 AECC) for IgG4-related disease (IgG4-RD) is considered a significant advancement in the study of this condition. Most studies evaluating their performance have focused on White and Asian patients, leaving a knowledge gap regarding Latin American populations. Therefore, this study aimed to assess the performance of the 2019 AECC for IgG4-RD in a cohort of Latin American patients., Methods: A multicenter medical records review study was conducted, involving centers from Argentina, Chile, Mexico, Peru, and Uruguay. Data on IgG4-RD patients and mimicker conditions were collected through a standardized online form. The criterion standard for diagnosing IgG4-RD was based on the fulfillment of the Comprehensive Diagnostic Criteria for IgG4-RD and/or the Consensus Statement on Pathology. The 2019 AECC was retrospectively applied., Results: We included 300 patients, with 180 (60%) having IgG4-RD and 120 (40%) having mimicker conditions. The 2019 AECC had a sensitivity of 66.7% and a specificity of 100%. Sensitivity increased to 73.3% when disease-specific autoantibody items were removed, without affecting specificity. The true-positive cases had more involved organs, a higher availability of biopsy results, and were more likely to belong to the Mikulicz/systemic and proliferative phenotypes., Conclusions: The use of the 2019 AECC for IgG4-RD in a Latin American population confirms its high specificity in excluding those without the disease. The presence of concomitant autoimmune diseases and clinically nonsignificant disease-specific autoantibodies excludes a significant number of patients from fulfilling the criteria., Competing Interests: The authors declare no conflict of interest., (Copyright © 2024 Wolters Kluwer Health, Inc. All rights reserved.)

Background/objective: Data on IgG4-related disease (IgG4-RD) come almost exclusively from cohorts from Asia, Europe, and North America. We conducted this study to describe the clinical presentation, phenotype distribution, and association with sex, ethnicity, and serological markers in a large cohort of Latin American patients with IgG4-RD., Methods: We performed a multicenter medical records review study including 184 Latin American IgG4-RD patients. We assigned patients to clinical phenotypes: group 1 (pancreato-hepato-biliary), group 2 (retroperitoneal/aortic), group 3 (head and neck-limited), group 4 (Mikulicz/systemic), and group 5 (undefined). We focused the analysis on how sex, ethnicity, and clinical phenotype may influence the clinical and serological presentation., Results: The mean age was 50.8 ± 15 years. Men and women were equally affected (52.2% vs 48.8%). Fifty-four patients (29.3%) were assigned to group 1, 21 (11.4%) to group 2, 57 (30.9%) to group 3, 32 (17.4%) to group 4, and 20 (10.8%) to group 5. Male sex was associated with biliary tract (odds ratio [OR], 3.4; 95% confidence interval [CI], 1.36-8.26), kidney (OR, 3.4; 95% CI, 1.28-9.25), and retroperitoneal involvement (OR, 5.3; 95% CI, 1.45-20). Amerindian patients presented more frequently with atopy history and gallbladder involvement. Group 3 had a female predominance., Conclusions: Latin American patients with IgG4-RD were younger, and men and women were equally affected compared with White and Asian cohorts. They belonged more commonly to group 1 and group 3. Retroperitoneal and aortic involvement was infrequent. Clinical and serological features differed according to sex, ethnicity, and clinical phenotype., Competing Interests: The authors declare no conflict of interest., (Copyright © 2022 Wolters Kluwer Health, Inc. All rights reserved.)

Background: The tendency of IgG4-related disease (IgG4-RD) to form pseudotumors, as well as its multisystemic nature, makes it the perfect mimicker of many conditions. Moreover, some clinical, serological, radiological, or histological features of the disease might be shared with some mimickers.Recently, 4 clinical phenotypes have been identified, and patients grouped in each phenotype have distinctive demographic, clinical, and serological features and outcomes, and, as expected, for each phenotype, a set of differential diagnoses should be considered., Summary of the Literature: The main differential diagnoses for the pancreato-hepato-biliary phenotype are pancreatic adenocarcinoma and cholangiocarcinoma. Other differential diagnoses include type 2 autoimmune pancreatitis and primary sclerosing cholangitis. In patients with retroperitoneal/aortic phenotype, inflammatory conditions such as idiopathic retroperitoneal fibrosis and large vessel vasculitides should be ruled out, and most of the time, a biopsy will be needed to exclude malignancies. In head and neck limited phenotype, autoimmune conditions (eg, granulomatosis with polyangiitis, Graves orbitopathy, sarcoidosis), malignancies, and histiocytosis should be ruled out, whereas the main differential diagnoses of the Mikulicz/systemic phenotype are Sjögren syndrome, granulomatosis with polyangiitis, and multicentric Castleman disease., Conclusions: Approaching a patient with probable IgG4-RD through a clinical phenotype framework will ease the diagnostic algorithm and facilitate the prompt recognition of the disease. There are certain clinical, serological, radiological, and histological features in each clinical phenotype that, if present, increase the likelihood that a patient may have IgG4-RD instead of the mimicker condition. Those clues that point toward IgG4-RD diagnosis should be actively sought in the workup of patients., Competing Interests: The authors declare no conflict of interest., (Copyright © 2021 Wolters Kluwer Health, Inc. All rights reserved.)

Background: There is controversy in medical literature over the outcome of patients with lupus nephritis (LN) class II. The aim of this study was to explore the risk of histological transformation (HT) and possible factors related to negative response to treatment in patients with mesangial LN class II., Methods: A retrospective and multicenter study was carried out that includes patients who had received a diagnosis of LN class II on their first renal biopsy. Creatinine, urine sediment, and proteinuria were recorded at the time of the first biopsy, 6 months, and 1, 2, and 5 years after the first biopsy. Response to treatment, HT, and long-term outcome were evaluated., Results: Forty-one patients were included. The manifestation at first biopsy was proteinuria greater than 0.5 g/d in 28 patients (68.29%; 8 [28.57%] of 28 patients had nephrotic syndrome), hematuria in 18 patients (43.90%), and deterioration of renal function in 3 patients (7.31%). During the follow-up (median, 8 years; range, 1-35 years), a new biopsy was performed in 18 patients (43.90%), and in 17 patients (17/18 [94.44%]), there was HT. Median time at rebiopsy was 32 months (range, 11-305 months). Of the 18 patients who had a second biopsy, 10 (55.55%) were on hydroxychloroquine versus 100% (19/19) of patients who did not undergo the procedure (P = 0.001). A year after the first renal biopsy, there are data available from 34 patients; of them, 24 patients (70.58%) had achieved response, and 10 patients (29.41%) had no response (NR) (missing data in 7). A higher 24-hour urinary protein at 6 months was predictor of worse outcome at 1 year, with statistical significance difference for the nonresponder group (median proteinuria, 2.3 g/d [range, 0-4.7 g/d]) compared with responders (median proteinuria, 0.28 g/d [range, 0-1.7 g/d]) (P = 0.0133).In the long-term follow-up (5 years), HT was the main cause of unfavorable outcome and was measured in 78.57% of patients (11/14 patients)., Conclusions: This series shows a high rate of HT in long-term follow-up. Proteinuria at 6 months made it possible to set aside patients who will have an unfavorable outcome in the long term and who will thus benefit from a more aggressive treatment. The results suggest that hydroxychloroquine had a nephroprotective effect., Competing Interests: The authors declare no conflict of interest.

Objective: The objective of this study was too show findings at finger nails level revealed by high-frequency gray-scale ultrasound (US) and power Doppler in patients with psoriatic arthritis (PsA),and cutaneous psoriasis compared with rheumatoid arthritis and control subjects., Methods: We studied 35 patients with PsA, 20 with cutaneous psoriasis, and control groups (28 control subjects and 27 patients with rheumatoid arthritis). All nails of both hands were observed (1097 nails, 3 excluded because of trauma). In every patient, we classified the morphologic abnormalities disclosed in ventral and dorsal nail plates. We also measured the distance between ventral plate and the bone margin of the distal phalanx at the right index finger., Results: All patients and control subjects presented abnormalities in the US imaging. Those with psoriatic arthritis and cutaneous psoriasis showed a higher number of compromised nails. When classifying those abnormalities using the typology of Wortsman et al, patients with psoriatic arthritis showed loosening of the borders of the ventral plate (type II), whereas patients with cutaneous psoriasis showed focal hyperechoic involvement of the ventral plate without involvement of the dorsal plate (type I). Patients of the control group could not be classified, although 31 of 55 showed thinning of the ventral plate without hyperechoic deposits. Nineteen of 35 patients with psoriatic arthritis, and 10 of 20 patients with cutaneous psoriasis did not show any nail clinical alterations. Nevertheless, the US detected type II alterations in the first group and type I in the second group. Patients with psoriatic arthropathy had power Doppler increases in distal interphalangeal joints and nail beds in a statistically significant form (P = 0.0001).When measuring the distance between the ventral plate and the bone margin of the distal phalanx, there was homogeneity among samples in patients and control subjects. A receiver operating characteristic curve analysis determined that a cut point of 2 mm clearly defined these 2 groups. There was a significant difference (P < 0.0001) between the mean distance ventral plate-osseous margin of the distal phalanx in psoriatic arthritis patients (P = 0.001) and patients with cutaneous psoriasis (P = 0.005) versus rheumatoid arthritis patients (P = 0.548)., Conclusions: As a predominant finding in our study, we observed abnormalities of the ventral plate in patients with PsA (type II) and abnormalities (type I) in patients with cutaneous psoriasis. We found a significant difference between the mean distance ventral plate-osseous margin of the distal phalanx in patients with PsA and patients with cutaneous psoriasis versus control subjects. Ultrasound imaging could be a feasible and sensitive tool to describe, measure, and follow morphologic characteristics and changes of the nail in psoriatic and/ or psoriatic arthritis patients with or without clinical nail lesions.

Background: The increased mortality reported among patient with rheumatoid arthritis (RA) has been attributed to cardiovascular disease. Metabolic syndrome (MS) is a cluster of major risk factors for cardiovascular disease such as dyslipidemia, obesity, hypertension, and diabetes. There is a lack of reporting on the prevalence of MS in RA patients in Argentina., Objectives: The objectives of this study were to determine and compare the frequency of MS in patients with RA and a control group and to assess the factors associated with MS., Methods: This is a cross-sectional study involving 1033 (409 RA and 624 age- and sex-matched control subjects) patients, followed up at 9 different rheumatology units in Argentina. Metabolic syndrome was defined according to the Adult Treatment Panel III (ATP III) and the International Diabetes Federation (IDF). The relationship between demographic variables, clinical data (disease duration, disease activity by Disease Activity Score of 28 joints, presence of rheumatoid factor [RF] and/or anti-cyclic citrullinated peptide antibody, presence of extra-articular manifestations), pharmacological treatment, and MS was examined by descriptive statistics. Variables with P ≤ 0.10 in these analyses were then examined by logistic regression., Results: The frequency of MS in RA patients and the control group was 30% versus 39% (P = 0.002) when defined as per the ATP III and 35% versus 40% (P = 0.10) as per the IDF. Variables independently associated with MS in RA patients were age (odds ratio [OR], 1.03; 95% confidence interval [CI], 1.01-1.06 [P = 0.01] for the ATP III and OR, 1.03; 95% CI, 1.01-1.05 [P < 0.001] for the IDF), the presence of RF and/or anti-cyclic citrullinated peptide antibody (OR, 2.91; 95% CI, 1.11-7.61 [P = 0.02] for the ATP III and OR, 2.37; 95% CI, 1.09-5.16 [P = 0.02] for the IDF), and the use of hydroxychloroquine (OR, 0.48; 95% CI, 0.23-0.97 [P = 0.04] only for the IDF)., Conclusions: In this study, we were not able to demonstrate a higher frequency of MS in RA patients. However, older patients with positive RF or CCP have a higher risk of MS. A protective effect to develop MS was seen in the population treated with hydroxychloroquine.

We describe four patients with widespread cutaneous necrosis as a manifestation of the antiphospholipid antibody syndrome. In three of the patients, the lesions were the first manifestation of the syndrome, and they were located in upper and lower extremities. Vascular thrombosis was present in the skin biopsy in all of the patients. Patients were effectively treated with anticoagulants, and healing occurred within 60 days. Such widespread cutaneous necrosis is a rare manifestation of the syndrome, and it must be regarded as major thrombotic event. Long-term treatment with anticoagulants must be considered. The recognition of these lesions is important, because they may be the onset of the syndrome, allowing its early diagnosis and treatment.

Hansen's disease is a chronic granulomatous disease of infectious origin. It has a worldwide distribution and a variety of clinical manifestations often involving the skin, nasal mucosa, and peripheral nerves. Lepromatous leprosy characterizes the condition of a large group of patients with little or no resistence to the infection. Several forms of lepromatous leprosy are recognized, including macular, nodular, and diffuse. Lucio's phenomenon is a rare but distinctive skin eruption seen in patients with diffuse lepromatous leprosy. The diffuse lesions of Lucio's phenomenon have a predilection for the extremities, can include nodules, and heal with atrophic stellate scars; histologically, a necrotizing vasculitis accompanied by a nonspecific inflammatory reaction may be seen. We describe two patients with Lucio's phenomenon who presented with nontender, painless, skin lesions with nodules in part perceptible only by palpation. Both patients were treated with multidrug therapy, and immunosuppressive doses of steroids as the suggested optimal treatment for this reactional state. However, Lucio's phenomenon is frequently fatal as a result of bacterial infection or sepsis, and both patients reported here died. We call attention to this particular and unusual skin manifestation of lepromatous leprosy, which can mimic rheumatic disease and other causes of vasculitis. This is especially likely to be unrecognized in nonendemic countries but cases will occasionally be seen in this age of extensive international travel.

We present the case of a 71-year-old patient with rugger-jersey vertebrae who was referred to us with a tentative diagnosis of Paget's disease. After considering other conditions causing similar bone sclerosis, such as metastases, lymphoma, renal osteodystrophy, myelofibrosis, thalassemia, and Gaucher's disease, we agreed on a diagnosis of osteopetrosis. This is a rare entity of genetic origin, manifest by variable generalized symmetric skeletal sclerosis caused by defective bone resorption.

We describe four patients who presented with seronegative inflammatory peripheral polyarthritis with pitting edema. All of these patients had spontaneous resolution of their disease over 9-18 months. None of them developed erosions or relapse after prolonged follow-up. Recognition of the features described may allow for conservative therapy with less toxic drugs.