Marina Scolnik

Value of repeat biopsy in lupus nephritis flares

Objectives Renal flares are common in lupus nephritis (LN), and class switch is thought to be characteristic. There is no agreement on indications for performing a repeat renal biopsy. Our objective was to retrospectively review patients who had more than one renal biopsy performed on clinical indications, and analyse clinical, pathological and treatment changes after successive biopsies. Methods Forty-five patients with LN and one or more repeat renal biopsies were included, with a total of 116 biopsies. Results Of the 71 repeat biopsies, pathological transition occurred in 39 (54.9%). When having a previous biopsy with a proliferative lesion, class switch occurred in 55.6%, with 24.4% evolving into non-proliferative classes. When previous biopsy was class V, transition to other classes occurred in 58.3% and changes were all into proliferative classes. Conversion from one pure proliferative form to another (class III to class IV or vice versa) happened in 11.3% of the rebiopsies, with 62 rebiopsies (87.3%) leading to a change in the treatment regimen. Conclusions Histological transformations were common, and they occurred when the previous biopsy had non-proliferative lesions as well as when lesions were proliferative. Treatments were modified after repeat renal biopsy in the majority of patients. In this experience, kidney repeat biopsies were useful in guiding treatment of LN flares.

Incidence and prevalence of lupus in Buenos Aires, Argentina: a 11-year health management organisation-based study

Objectives Studies regarding the epidemiology of systemic lupus erythematosus (SLE) are lacking in Argentina. Our purpose was to estimate the incidence and prevalence of SLE in a university hospital-based health management organisation in Buenos Aires (HIMCP). Methods For incidence calculation, the population at risk included all adult members of the HIMCP, with continuous affiliation for at least 1 year from January 1998 to January 2009. Each person was followed until he/she voluntarily left the HIMCP, death or finalisation of the study. Multiple methods for case finding were used to ensure complete ascertainment: (a) patients with problem SLE, undifferentiated autoimmune disease or mixed connective tissue disease in the Computer-based Patient Record System, (b) patients with positive antinuclear antibody test, anti-Sm antibodies and/or anti-dsDNA antibodies in the laboratory database and (c) patients who consumed hydroxichloroquine, chloroquine, azathioprine, cyclophosphamide, mycophenolate, cyclosporine or rituximab, from the administrative HIMCP drugs database. Medical records of all patients found were reviewed, and only patients fulfilling ACR criteria for SLE were included. Global and gender incidence rate (IR) was calculated. Prevalence was estimated on 1 January 2009, and the denominator population was the number of active members >18 years at that date (n=127 959). Results In the study period, 68 patients developed SLE. The observed IR (per 100 000 person-years, (CI 95%)) was 6.3 (4.9 to 7.7) for total population; 8.9 (CI 6.6 to 11.2) for women and 2.6 (1.2 to 3.9) for men. On 1 January 2009, 75 prevalent cases were identified. Prevalence rates (cases per 100 000 habitants, (CI 95%)) were 58.6 (46.1 to 73.5) for total population; 83.2 (63.9 to 106.4) for women and 23 (CI 11.9 to 40.1) for men. Conclusions SLE incidence and prevalence rates in Argentina are in agreement with those of other studies from different parts of the world.

Validation in Spanish of a screening questionnaire for the detection of psoriatic arthritis in patients with psoriasis

OBJECTIVE A patient self-administered questionnaire [PsA Screening and Evaluation (PASE)] has been developed and validated in English, but has not been tried in Spanish speaking populations. This study aimed to adapt and validate PASE in Spanish to screen Spanish speaking psoriasis patients for signs and symptoms of inflammatory arthritis. METHODS Initial translation from English to Spanish (forward translation) was performed by two independent translators and the resulting versions were synthesized during a consensus meeting. The questionnaire was tried in a pilot study and resulted in a change in the agreement scale for a frequency scale with wording adaptation [Spanish PASE (PASE-S)]. RESULTS One hundred and eleven patients were screened with PASE-S; 25 with PsA (without previous treatments), 23 with psoriasis, 22 with psoriasis and OA and 41 with OA without psoriasis. The diagnosis of psoriasis was performed by a dermatologist, and a rheumatologist determined the diagnosis of PsA or OA. Patients with PsA had statistically significant higher symptoms, function and total PASE-S scores compared with those without PsA. Receiver operator curves showed an area under the curve of 0.79 (95% CI 0.69, 0.89) for the total score. A cut-off value ≥34 showed sensitivity of 76%, and specificity of 74.4% for the diagnosis of PsA. CONCLUSION The validated PASE questionnaire is a self-administered tool that can be used to screen for PsA among patients with psoriasis in a Spanish speaking population. PASE was able to distinguish between symptoms of PsA and OA.

Incidence and prevalence of polymyositis and dermatomyositis in a health management organization in Buenos Aires.

Background Polymyositis (PM) and dermatomyositis (DM) are infrequent diseases. Data on incidence and prevalence are scarce and conflicting. There are no such data in Latin America and in Argentina in particular. Objectives We undertook to examine the incidence and prevalence of PM/DM in the prepaid health maintenance organization (HMO) of our hospital, in the city of Buenos Aires. Methods Members of the HMO between January 1999 and June 2009 were identified from medical records of patients followed up by us at the HMO. Incident cases and prevalence were calculated at the end of the period. Results During the study period, 146,747 persons contributed a total of 937,902.6 person-years (mean age was 46.6 [SD, 18.4] years, and 59% were female). Ten incident cases were detected, 7 women and 3 men with a global incidence rate (IR) of 1.07 per 100,000 person-years (95% confidence interval [CI], 0.5–1.84). Three subjects had DM with an IR of 0.32 per 100,000 person-years (95% CI, 0.1–0.99), and 7 had PM with an IR of 0.75 per 100,000 person-years (95% CI, 0.35–0.16). On June 1, 2009, 17 prevalent cases were detected, with a mean age of 48.9 (SD, 17.7) years; 76% were female, representing a prevalence of 17.4 per 100,000 persons (95% CI, 10.1–27.8). Among the 17 patients with idiopathic inflammatory myopathy, 10 patients had DM, with a prevalence of 10.22 per 100,000 persons (95% CI, 4.9–18.8), and 7 had PM (prevalence, 7.2 per 100,000 persons [95% CI, 2.9–14.7]). Conclusions It is difficult to compare studies from different populations and using different ascertainment techniques. These first data from Latin America are in general agreement with many studies.

Incidence and Prevalence of Rheumatoid Arthritis in a Health Management Organization in Argentina: A 15-year Study

Objective. To estimate incidence and prevalence rates of rheumatoid arthritis (RA) in the city of Buenos Aires (CABA), Argentina, using data from a university hospital–based health management organization. Methods. Global, age-specific, and sex-specific incidence and prevalence rates were calculated for members of the Hospital Italiano Medical Care Program (HIMCP), age ≥ 18 years. Incidence study followed members with continuous affiliation ≥ 1 year from January 2000 to January 2015 until he/she voluntarily left the HIMCP, RA was diagnosed, death, or study finalization. Cases from the Rheumatology Section database, electronic medical records, laboratory database, and pharmacy database were filtered with the 2010 American College of Rheumatology/European League Against Rheumatism criteria. Prevalence was calculated on January 1, 2015, and standardized for CABA. Capture-recapture (C-RC) analysis estimated true population sizes. Results. In the study period, incidence rates (cases per 100,000 person-yrs) were 18.5 (95% CI 16.7–20.4) overall, 25.2 (95% CI 22.4–28.0) for women, and 8.8 (95% CI 6.8–10.8) for men. Prevalence rates (percentage of RA cases in the sample population) were 0.329 (95% CI 0.298–0.359) overall, 0.464 (95% CI 0.417–0.510) for women, and 0.123 (95% CI 0.093–0.152) for men. Standardized CABA prevalence rate was 0.300 (95% CI 0.292–0.307). C-RC adjusted rates were almost the same as unadjusted rates. Conclusion. This study’s incidence and prevalence rates are in the lower range of the rates found around the world. Our female to male prevalence ratio was 4:1. Our peak incidence age was in the sixth and seventh decades for both sexes.

Patient-reported outcome instruments for assessing Raynaud’s phenomenon in systemic sclerosis: A SCTC Vascular Working Group Report

The episodic nature of Raynaud’s phenomenon in systemic sclerosis has led to a reliance on patient-reported outcome instruments such as the Raynaud’s Condition Score diary. Little is known about the utilization in routine clinical practice and health professional attitudes toward existing patient-reported outcome instruments for assessing systemic sclerosis-Raynaud’s phenomenon. Members of the Scleroderma Clinical Trials Consortium Vascular Working Group (n = 28) were invited to participate in a survey gauging attitudes toward the Raynaud’s Condition Score diary and the perceived need for novel patient-reported outcome instruments for assessing patient-reported outcome. Nineteen Scleroderma Clinical Trials Consortium Vascular Working Group members (68% response rate) from academic units based in North America (n = 9), Europe (n = 8), South America (n = 1) and Australasia (n = 1) took part in the survey. There was broad consensus that Raynaud’s Condition Score diary returns could be influenced by factors including seasonal variation in weather, efforts made by patients to avoid or ameliorate attacks of Raynaud’s phenomenon, habituation to Raynaud’s phenomenon symptoms, evolution of Raynaud’s phenomenon symptom characteristics with progressive obliterative microangiopathy, patient-coping strategies, respondent burden and placebo effect. There was consensus that limitations of the Raynaud’s Condition Score diary might be a barrier to drug development (79% of respondents agree/strongly agree) and that a novel patient-reported outcome instrument for assessing systemic sclerosis-Raynaud’s phenomenon should be developed with the input of both clinicians and patients (84% agree/strongly agree). Perceived potential limitations of the Raynaud’s Condition Score diary have been identified along with concerns that such factors might impede drug development programs for systemic sclerosis-Raynaud’s phenomenon. There is support within the systemic sclerosis community for the development of a novel patient-reported outcome instrument for assessing systemic sclerosis-Raynaud’s phenomenon.

First Latin American clinical practice guidelines for the treatment of systemic lupus erythematosus: Latin American Group for the Study of Lupus (GLADEL, Grupo Latino Americano de Estudio del Lupus)–Pan-American League of Associations of Rheumatology (PANLAR)

Systemic lupus erythematosus (SLE), a complex and heterogeneous autoimmune disease, represents a significant challenge for both diagnosis and treatment. Patients with SLE in Latin America face special problems that should be considered when therapeutic guidelines are developed. The objective of the study is to develop clinical practice guidelines for Latin American patients with lupus. Two independent teams (rheumatologists with experience in lupus management and methodologists) had an initial meeting in Panama City, Panama, in April 2016. They selected a list of questions for the clinical problems most commonly seen in Latin American patients with SLE. These were addressed with the best available evidence and summarised in a standardised format following the Grading of Recommendations Assessment, Development and Evaluation approach. All preliminary findings were discussed in a second face-to-face meeting in Washington, DC, in November 2016. As a result, nine organ/system sections are presented with the main findings; an ‘overarching’ treatment approach was added. Special emphasis was made on regional implementation issues. Best pharmacologic options were examined for musculoskeletal, mucocutaneous, kidney, cardiac, pulmonary, neuropsychiatric, haematological manifestations and the antiphospholipid syndrome. The roles of main therapeutic options (ie, glucocorticoids, antimalarials, immunosuppressant agents, therapeutic plasma exchange, belimumab, rituximab, abatacept, low-dose aspirin and anticoagulants) were summarised in each section. In all cases, benefits and harms, certainty of the evidence, values and preferences, feasibility, acceptability and equity issues were considered to produce a recommendation with special focus on ethnic and socioeconomic aspects. Guidelines for Latin American patients with lupus have been developed and could be used in similar settings.

Complement levels and risk of organ involvement in patients with systemic lupus erythematosus

Objective Complement plays a major role in SLE. Complement participation has been linked to disease activity and damage. Our objective was to estimate the association of complement behaviour with clinical manifestations, visceral injury and mortality in patients with SLE. Methods Complement determinations (C3 and C4 levels) were analysed in patients with SLE (fulfilling American College of Rheumatology (ACR) or Systemic Lupus International Collaborating Clinics (SLICC)criteria) seen at a university hospital between 2000 and 2013. Patients were grouped in those with permanent C3 and/or C4 low values (low complement group), those with C3 and C4 constant normal values (normal complement group) and those with fluctuant values (periods of normal and periods of low values: fluctuant group). Clinical characteristics and mortality were analysed and compared between groups. Results 270 patients with SLE were included (242 females, 89.6%), mean age at diagnosis was 34.2 years (SD 15.8). 75 patients had fluctuant levels of complement, 79 patients had persistent low complement levels and 116 had normal complement levels. Lupus glomerulonephritis was more frequent in patients with fluctuant levels (75%, 56% and 49%, respectively, p=0002). The normal complement group had less frequency of haematological involvement and anti-double stranded DNA (dsDNA) antibodies. At the end of the follow-up, 53% of the patients had damage (SLICC/ACR ≥1). In a Cox proportional hazard model age at diagnosis, neurological impairment, thrombocytopaenia and corticosteroids were associated with more damage, while hydroxychloroquine was a protective factor. There were no differences between complements groups on accumulated damage. Ten-year survival rate was 93%, 93.5% and 92% for the normal complement group, the persistently low group and the fluctuant group, respectively. Conclusions Patients with constant normal complement had lower prevalence of haematological involvement and anti-dsDNA, while patients with fluctuant complement had higher renal impairment. Neither the persistent low complement nor the fluctuant complement groups had increased mortality and/or visceral damage.

Are There Clinical Differences in Limited Systemic Sclerosis according to Extension of Skin Involvement

Objectives. To examine the characteristics of our patients with limited systemic sclerosis (lSSc) for differences between Barnett Type 1 (sclerodactyly only) and Type 2 or intermediate (acrosclerosis-distal but may reach up to elbows and/or knees plus face) subsets. Methods. Records of patients between January 1, 2000, and December 31, 2011, with SSc or those with anti-Scl-70, anticentromere, or antinucleolar antibodies were reviewed. Only cases fulfilling ACR 1980 criteria were included and classified as diffuse or limited according to LeRoys criteria. Limited SSc was separated into sclerodactyly and acrosclerosis (Barnetts Types 1 and 2). Results. 234 SSc patients (216 females) fulfilled criteria. Female/male ratio was 12 : 1; 24% had dSSc and 76% lSSC (64% Type 1 and 12% Type 2). Total follow-up was 688 patient-years. Within lSSC, the Type 2 group had significantly shorter duration of Raynauds and more anti-Scl-70 and less anticentromere antibodies. In particular, interstitial lung disease (ILD) was significantly more prevalent in Type 2 group and similar to Type 3. Conclusions. These results appear to confirm that extension of skin involvement within limited SSc may identify two different subsets with clinical and serologic characteristics.