Gerardo Quintana

Lupus arthropathy: a case series of patients with rhupus

Among the clinical manifestations of systemic lupus erythematosus (SLE) is an arthropathy, which is usually nonerosive. In many cases the joint involvement is mild. A subset of patients have deforming, nonerosive Jaccoud’s arthropathy, and a minority have an arthropathy with clinical findings similar to rheumatoid arthritis (RA) that has been called “rhupus.” We report our series of eight patients (seven female, one male) with rhupus arthropathy. Patients were between the ages of 17 and 38 years (average: 30.3 years) at disease onset. All had deforming or Jaccoud∝s arthropathy, and three had erosive disease. The arthritis was typically the first disease manifestation. Other symptoms of lupus including vasculitis and glomerulonephritis appeared after an average of 2.8 years. All had positive antinuclear antibody and rheumatoid factor. Rhupus arthritis is not a combination of RA and SLE, but should be regarded as a variant of the arthropathy of lupus.

Lupus arthropathy: historical evolution from deforming arthritis to rhupus

Systemic lupus erythematosus is an autoimmune and inflammatory disease with multiple clinical manifestations, including arthropathy. The clinical presentation of articular involvement is variable, ranging from arthralgia without erosions or deformity to an erosive arthropathy and severe functional disability. A subset of patients with this articular involvement have Jaccoud’s arthropathy, and others have an arthropathy with clinical findings similar to rheumatoid arthritis that has been called “rhupus.” In this paper we review the historical evolution of concepts of lupus arthropathy, from deforming arthritis to rhupus, and conclude that rhupus is not a combination of rheumatoid arthritis and lupus. Instead, rhupus arthropathy should be regarded as a variant of the arthropathy of systemic lupus erythematosus.

The use of procalcitonin determinations in evaluation of systemic lupus erythematosus.

Background:Procalcitonin (PCT), the precursor of the calcitonin, is synthesized in the parafollicular C-cells of the thyroid. It has been used to detect and to differentiate systemic bacterial infections from flares of systemic lupus erythematosus (SLE). PCT in serum increases in severe bacterial and fungal infections, but not, or only slightly in viral infections. Objective:To measure PCT levels in patients with active SLE and to compare them with patients without lupus activity and to determine the possible association between activity and elevation of the PCT. Design:Prospective case control study. Patient and Methods:Measurements were made of PCT (METHOD: Essay immunoluminometric - and ultrasensitive - BRAHMS Diagnostika, Berlin, Germany), C-reactive protein, erythrocyte sedimentation rate, and blood and urine cultures. The index of activity of SLE was determined by Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) score of a serial group of patients seen by our rheumatology service. Samples from 53 patients were analyzed. The patients were divided in 2 groups: group I (n = 21) with little or no activity for SLE; group II (n = 32) with activity for SLE (SLEDAI >5). None of the patients had severe bacterial infection, sepsis, or systemic multiorgan failure. Results:Group I had a SLEDAI score of 1.8 [95% confidence interval (CI) 1.09–2.51] with mean levels of PCT 0. 08 ng/mL (Negative smaller than 0. 5 ng/mL). Group II SLEDAI score was 14.6 (95% CI 11.95–17.23) with mean levels of PCT 0.418 ng/mL with standard deviation 1.0021 (95% CI 0.0628–0.773). The measure of association calculated by Fisher method was not significant (1.927) (P = 0. 282). In the group II, 3 patients had frankly positive PCT (3.18, 3.42, and 3.95 ng/mL) and high activity indices (14, 13, and 24). None presented with severe infection, sepsis, or systemic multiorgan failure. They had pneumonia, renal failure (PCT 3.42 ng/mL) and urinary tract infection without systemic symptoms (3.95 ng/mL). Infection was not detected in the other patient (3.18 ng/mL) that was interpreted as a false positive. Conclusions:This study demonstrates that there is no association between the activity of SLE and PCT levels. The utility of the PCT resides is in raising suspicion of a concurrent bacterial or mycotic infection in the evaluation of patients with active autoimmune diseases.

Serum Endoglin Levels in Patients Suffering from Systemic Sclerosis and Elevated Systolic Pulmonary Arterial Pressure

Background. Pulmonary arterial hypertension (PAH) is the main cause of morbimortality in systemic sclerosis (SSc). Increased Eng expression has been demonstrated in SSc patients. Objective. Ascertaining serum levels of Eng in SSc patients with and without elevated systolic pulmonary arterial pressure (sPAP) and comparing them with that of healthy volunteers. Methods. A cross-sectional study was carried out. A commercial ELISA kit was used for measuring serum concentrations of Eng in 60 subjects: 40 patients with SSc with and without elevated sPAP, compared to 20 healthy control subjects. Elevated sPAP was detected by echocardiogram. Results. No association between positive Eng and elevated sPAP was found when compared to the SSc without elevated sPAP group (OR = 2.85; 0.65–12.88 95% CI; P = .11); however, an association was found between positive Eng and elevated sPAP compared to healthy controls (OR = 23.22; 2.46–1050.33 95% CI; P = .001), and weak association was found between the positive Eng with SSc without elevated sPAP group compared to healthy controls (OR = 8.14, 0.8–393.74 95% CI; P = .046). Conclusion. Raised serum levels of Eng in SSc patients compared to healthy controls were found, suggesting a role for Eng in SSc vasculopathy and not just in elevated sPAP. However, prospective studies are needed to verify such observations.

Single anti-P ribosomal antibodies are not associated with lupus nephritis in patients suffering from active systemic lupus erythematosus.

BACKGROUND In clinical practice, it is sometimes difficult to diagnose a relapse in patients suffering from systemic lupus erythematosus (SLE) and lupus nephritis (LN) having potential complications, including renal failure and death. Some immunological markers can help to determine their association with LN and, therefore, diagnose the early onset of complications. OBJECTIVES Evaluating the association between systemic and/or kidney activity and anti-P ribosomal and anti-dsDNA antibodies in patients suffering from active SLE. METHODS 389 patients were evaluated, 140 of whom were subsequently included in the study. The patients were divided into two groups by means of case-control studies, including Colombian patients having American College of Rheumatology (ACR) classification criteria for SLE (1997). The SLE disease activity index (SLEDAI) was applied and all patients presenting an increase of 5 or more compared to their last evaluation, as well as presenting renal manifestations, were considered to be cases; all patients had an activity score. An ELISA kit and the indirect immunofluorescence method with Crithidia luciliae were used for determining the presence of anti-P ribosomal and anti-dsDNA antibodies, respectively. RESULTS No association was found between anti-P ribosomal antibodies and LN (p=0.2971) but anti-P ribosomal antibodies showed association with a >5 SLEDAI score (OR=4.87; 1.32-17.98 95% CI; p=0.008). The coexistence of anti-P ribosomal and anti-dsDNA antibodies was associated with LN (OR=3.52; 1.07-13.42 95% CI; p=0.019) and anti-dsDNA was associated with LN (p=0.001). CONCLUSION There was no association between anti-P ribosomal antibodies and LN but anti-P ribosomal antibodies coexisting with anti-dsDNA antibodies was associated with LN, thereby suggesting that the coexistence of two antibodies is nephritogenic to a greater extent. Additional studies are needed to evaluate the coexistence of kidney-specific antibodies in SLE to determine the biological nature of LN.

A goalkeeper strategy in robot soccer based on Danger Theory

Artificial Immune Systems (AIS) have been successfully modeled and implemented in several engineering applications. In this work, a goalkeeper strategy in robot soccer based on Danger Theory is proposed. Danger Theory is a recent immune theory which has not been widely applied so far. The proposed strategy is implemented and evaluated using middle league SIMUROSOT from FIRA. Experiments carried out yielded promising results.

Necrotizing Lymphocytic Vasculitis Limited to the Peripheral Nerves: Report of Six Cases and Review

Background. The systemic vasculitides are syndromes characterized by inflammation and injury (necrosis or thrombosis) of blood vessels, resulting in clinical manifestations according to the affected vascular bed, but not classically in stocking-glove neuropathy. Objective. To describe a form of primary vasculitis affecting strictly peripheral nerves manifesting as stocking-glove neuropathy. Methods. Case series of 110 patients seen in three centers in Bogotá who presented with symptoms and signs of polyneuropathy and/or were identified with vasculitis affecting only the peripheral nerves, and who underwent sural nerve biopsy. Results. Six patients had a vasculitis affecting only the peripheral nerves diagnosed on sural nerve biopsy which demonstrated a mixed infiltrate of monocytes/macrophages and lymphocytes especially in the small epineurial blood vessels. Over time, all had worsening of symptoms, with grip weakness and motor deficits in the hand and feet. Serologies and acute phase reactants were normal in all patients. Treatment response to immunosuppression was satisfactory in 5 patients; 1 patient had progressive neurologic damage. Conclusions. There is a distinct form of primary vasculitis of the peripheral nervous system characterized by distal sensory polyneuropathy with stocking-glove distribution with good prognosis, few and minor relapses and good response to treatment even after delayed diagnosis.

Enfermedad de Still del Adulto: Estudio de Cohorte

Resumen Objetivo Describir las caracteristicas clinicas, paraclinicas y manejo de la enfermedad de Still del adulto (E.S.A.). Metodos Analisis retrospectivo de una cohorte de 24 pacientes. Resultados Las caracteristicas clinicas fueron: fiebre (100%), rash (79.1%) y artritis (66.66%). Leucocitosis (75%) fue la alteracion paraclinica mas frecuente, seguida por un incremento de la PCR, VSG y ferritina en 70.8%, 66.65% y 62.5%, respectivamente. El 50% de los pacientes presentaron un curso monociclico; el 33%, policiclico sistemico y el 17%, policiclico articular. No se presentaron casos de monociclico poliarticular. Se evidenciaron manifestaciones inusuales de enfermedad tales como: urticaria en 7 pacientes (29%); enfermedad pulmonar intersticial, en 6 pacientes (25%); meningitis aseptica, 4 pacientes (16.6%) y SDRA, en 4 pacientes (16.6%). Por su parte, todos los pacientes recibieron esteroides y 9 de ellos (37.5%) necesitaron tratamiento con pulsos de metilprednisolona. Dentro de las drogas modificadoras, el metotrexate fue el medicamento mas usado en 15 casos (62.5%), seguido por cloroquina sola o combinada en 11 pacientes (45.8%). Ademas, 3 pacientes (12.5%), requirieron tratamiento con terapia anti TNF por pobre respuesta a la terapia convencional. Fallecieron 2 pacientes (8.4%) asociados a cuadros de SDRA. Conclusiones La ESA tiene un amplio y dificil diagnostico diferencial a pesar de manifestaciones clasicas. La presencia de expresiones clinicas poco usuales puede retardar el diagnostico y tratamiento de ESA. La ESA no es una enfermedad benigna y puede cursar con alta mortalidad cuando se asocia a manifestaciones pulmonares severas.

Acute adult-onset still's disease presenting as pulmonary hemorrhage, urticaria, angioedema and leukemoid reaction: a case report and literature review

IntroductionAdult-onset Still’s disease is a rare systemic inflammatory disorder of unknown aetiology characterized by the classic triad of persistent high spiking fevers, joint pain and a distinctive salmon-colored bumpy rash however, the multiorgan involvement can be present.Case descriptionA 40-year-old woman previously healthy was referred to our hospital with 7 days of high fever and generalized arthralgia, The physical exam revealed angioneurotic edema detected on soles, palms and tongue and widespread red, urticated plaques in a symmetrical distribution affecting the arms, dorsal hands, upper and lower chest and back. Followed 5 days later by fever, the patient presented dyspnea, cough and hypoxemia, the imaging studies showed unilateral consolidation and pleural effusion. The bronchoscopy with bronchoalveolar lavage and skin biopsy were consistent with neutrophilic urticarial. The hematological disorders, infections and other autoimmune diseases were excluded.Discussion and evaluationThe diagnosis of adult-onset Still’s disease can be very difficult. There are no specific tests and reliance is usually placed on a symptom complex and the well described typical rash seen in most patients. In recent years, however, other cutaneous manifestations of Adult-onset Still’s disease have been reported but these are not so well known.ConclusionsThe evidence of rare manifestations is growing and the early clinical presentation of Adult-onset Still’s is extremely variable, making diagnosis difficult. For this reason, data on early clinical presentation of the disease are of interest. We reported the first case of acute Adult-onset Still’s disease with the association of pulmonary hemorrhage, urticaria and angioedema including a rare systemic manifestation as leukemoid reaction.

Clinical, serologic, and immunogenetic characterization (HLA-DRB1) of late-onset lupus erythematosus in a Colombian population.

Introduction Late-onset systemic lupus erythematosus (SLE) represents a specific subgroup that is defined as onset after 50 years of age. Late-onset lupus may have a different clinical course and serological findings, which may delay diagnosis and timely treatment. Objectives The objective of this paper is to determine the clinical, serologic, and immunogenetic differences among Colombian patients with late-onset SLE versus conventional SLE patients. Methodology This was a cross-sectional study in a Colombian population. Patients and their medical records were analyzed from the services of Rheumatology in Bogotá and met the criteria for SLE, according to the American College of Rheumatology (ACR) revised criteria for the classification of SLE. In a reference group of late-onset SLE patients (98 participants, with an onset after 50 years of age) and a group of conventional SLE patients (72 participants, with an onset of age of 49 years or less), multiple clinical variables (age, clinical criteria for lupus, alopecia, weight loss, fever, Raynaud’s phenomenon) and multiple serological variables (blood count, blood chemistry profile, autoantibodies) were analyzed. Additionally, the HLA class II (DRB1) of all the patients was genotyped, including an additional group of patients without the autoimmune disease. Statistical analysis was performed using the STATA 10.0 package. Results In the group of late-onset lupus, there was a higher frequency of pleurisy (p = 0.002), pericarditis (p = 0.026), dry symptoms (p = 0.029), lymphopenia (p = 0.007), and higher titers of rheumatoid factor (p = 0.001) compared with the group of conventional SLE. Late-onset SLE patients had a lower seizure frequency (p = 0.019), weight loss (p = 0.009), alopecia (p < 0.001), and Raynaud’s phenomenon (p = 0.013) compared to the conventional SLE group. In late-onset SLE, HLA DR17 (DR3) was found more frequently compared with individuals without autoimmune disease (OR 3.81, 95% CI 1.47 to 10.59) (p = 0.0016). Conclusion In the Colombian SLE population analyzed, there may be a probable association of several clinical and serologic variants, which would allow the differentiation of variables in the presentation of the disease among patients with late-onset SLE vs. conventional SLE.