Reporte de caso: vasculitis leucocitoclastica como forma de presentación de hepatitis b crónica / Case report: leukocytoclastic vasculitis as presenting manifestation of chronic hepatitis b virus infection

La vasculitis leucocitoclástica, también denominada angeitis cutánea leucocitoclástica, es la forma más común de vasculitis. Si bien la mayoría de los casos son idiopáticos, entre los agentes etiológicos que podemos nombrar se encuentran los agentes infecciosos, las enfermedades del tejido conectivos, las reacciones de hipersensibilidad a medicamentos y las neoplasias solidas o hematológicas. Si bien los procesos infecciosos son una causa conocida de vasculitis leucocitoclástica, la infección por virus de Virus de hepatitis B (VHB) es muy infrecuente. Presentamos una mujer de 47 años, sin antecedentes patológicos previos, que consultó por artralgias en rodillas y tobillos, mialgias en gemelos y rash purpúrico con leve prurito en ambos miembros inferiores, de un mes de evolución. La biopsia cutánea de las lesiones de miembros inferiores fue compatible con vasculitis leucocitoclástica. La serología de hepatitis B fue positiva por lo que inició tratamiento antiviral con Tenofovir y Prednisona con buena evolución de sus lesiones cutáneas

Leukocytoclastic vasculitis, also called leukocytoclastic cutaneous angiitis, is the most common form of vasculitis. Although most cases are idiopathic, etiologic agents include infectious agents, connective tissue diseases, drug hypersensitivity reactions, and solid or hematologic malignancies. Although infectious processes are a known cause of leukocytoclastic vasculitis, hepatitis B virus (HBV) infection is very rare. We present a 47-year-old woman, with no previous pathologic history, who consulted for arthralgias in the knees and ankles, myalgia's and purpuric rash with mild pruritus in both lower limbs, of one month evolution. Skin biopsy of lower extremity lesions was compatible with leukocytoclastic vasculitis. Hepatitis B serology was positive, so she started antiviral treatment with tenofovir and prednisone with good evolution of her skin lesions

Sudden Sensorineural Hearing Loss in Systemic Lupus Erythematosus and Antiphospholipid Syndrome: A Clinical Review.

BACKGROUND: Sudden sensorineural hearing loss (SSNHL) is defined as a sudden loss of hearing, usually unilateral, of more than 30 dB in 3 contiguous frequencies of the tonal audiometry. SSNHL estimates an incidence ranging from 5 to 20 per 100.000 people per year. In approximately 75% of cases, a cause cannot be identified. However, it could be a clinical manifestation of Systemic lupus erythematosus (SLE) and Antiphospholipid Syndrome (APS). OBJECTIVE: This review will focus on the clinical presentation, diagnosis, and management of the SLE and APS associated SSNHL. METHODS: We searched in PubMed, Scopus, Lilacs, and Cochrane reviewing reports of Sudden sensorineural hearing loss in SLE and/or APS. Articles written in English and Spanish, and were available in full text, were included. RESULTS: In patients with SLE, bilateral involvement was frequent. Antiphospholipid antibodies were positive in the majority of the patients. Corticosteroids were the mainstay of the treatment. The auditory prognosis was poor with total hearing loss recovery reached in only 22% of patients. On the other hand, most of the patients with SSNHL and APS were males and presented associated symptoms such as vertigo, tinnitus and/or headache, 75% had bilateral disease. Lupus anticoagulant and aCL were found in equal proportions, all patients were anticoagulated, and aspirin was associated in 25% of the cases. Complete resolution or improvement of symptoms was observed in 25% of the patients. CONCLUSION: Sudden sensorineural hearing loss, can be a clinical feature of SLE and APS. Treating physicians should be aware of this devastating complication, especially when bilateral involvement occurs.

Clinical Relevance of Galectin-1 and Galectin-3 in Rheumatoid Arthritis Patients: Differential Regulation and Correlation With Disease Activity.

Galectins, a family of animal lectins, play central roles in immune system regulation, shaping both innate and adaptive responses in physiological and pathological processes. These include rheumatoid arthritis (RA), a chronic multifactorial autoimmune disease characterized by inflammatory responses that affects both articular and extra-articular tissues. Galectins have been reported to play central roles in RA and its experimental animal models. In this perspective article we present new data highlighting the regulated expression of galectin-1 (Gal-1) and galectin-3 (Gal-3) in sera from RA patients under disease-modifying anti-rheumatic drugs (DMARDs) and/or corticoid treatment in the context of a more comprehensive discussion that summarizes the roles of galectins in joint inflammation. We found that Gal-1 levels markedly increase in sera from RA patients and positively correlate with erythrocyte sedimentation rate (ERS) and disease activity score 28 (DAS-28) parameters. On the other hand, Gal-3 is downregulated in RA patients, but positively correlates with health assessment questionnaire parameter (HAQ). Finally, by generating receiver-operator characteristic (ROC) curves, we found that Gal-1 and Gal-3 serum levels constitute good parameters to discriminate patients with RA from healthy individuals. Our findings uncover a differential regulation of Gal-1 and Gal-3 which might contribute to the anti-inflammatory effects elicited by DMARDs and corticoid treatment in RA patients.

Severe infections in patients with autoimmune diseases treated with cyclophosphamide.

INTRODUCTION: Infectious diseases are a significant cause of morbidity and mortality in patients with connective tissue diseases. Corticosteroids and immunosuppressive drugs, such as cyclophosphamide (CYC), increases the risk of infections. The objective of this study was to estimate the incidence rates of severe infections in patients who received treatment with CYC. PATIENTS AND METHODS: The records of 60 patients with systemic autoimmune diseases who received treatment with CYC were retrospectively reviewed. We evaluated the rate of severe infections that occurred during CYC therapy and the 3 subsequent months. RESULTS: Systemic lupus erythematosus was the most common disease, and diffuse proliferative glomerulonephritis the most frequent indication. Severe infection occurred in 9 patients (15%). Community acquired pneumonia was the most frequent infection with 3 cases (33%) followed by Herpes Zoster with 2 reports (22%). The cumulative dose of corticosteroid was the only significant risk factor for infection 32.8±16.7 vs. 20.1±15.3 P=.007. CONCLUSION: The use of lower doses of corticosteroids and an aggressive management of infectious complications, allows for an acceptable safety profile in patients treated with CYC.

Impact of genetic ancestry and sociodemographic status on the clinical expression of systemic lupus erythematosus in American Indian-European populations.

OBJECTIVE: American Indian-Europeans, Asians, and African Americans have an excess morbidity from systemic lupus erythematosus (SLE) and a higher prevalence of lupus nephritis than do Caucasians. The aim of this study was to analyze the relationship between genetic ancestry and sociodemographic characteristics and clinical features in a large cohort of American Indian-European SLE patients. METHODS: A total of 2,116 SLE patients of American Indian-European origin and 4,001 SLE patients of European descent for whom we had clinical data were included in the study. Genotyping of 253 continental ancestry-informative markers was performed on the Illumina platform. Structure and Admixture software were used to determine genetic ancestry proportions of each individual. Logistic regression was used to test the association between genetic ancestry and sociodemographic and clinical characteristics. Odds ratios (ORs) were calculated with 95% confidence intervals (95% CIs). RESULTS: The average American Indian genetic ancestry of 2,116 SLE patients was 40.7%. American Indian genetic ancestry conferred increased risks of renal involvement (P < 0.0001, OR 3.50 [95% CI 2.63- 4.63]) and early age at onset (P < 0.0001). American Indian ancestry protected against photosensitivity (P < 0.0001, OR 0.58 [95% CI 0.44-0.76]), oral ulcers (P < 0.0001, OR 0.55 [95% CI 0.42-0.72]), and serositis (P < 0.0001, OR 0.56 [95% CI 0.41-0.75]) after adjustment for age, sex, and age at onset. However, age and sex had stronger effects than genetic ancestry on malar rash, discoid rash, arthritis, and neurologic involvement. CONCLUSION: In general, American Indian genetic ancestry correlates with lower sociodemographic status and increases the risk of developing renal involvement and SLE at an earlier age.

Elastofibroma dorsi: review of 4 cases.

Elastofibroma dorsi is a benign, uncommon fibroelastic tissue condition, more common in women after the fifth decade of life. It is usually located in the subscapular region, and can sometimes be bilateral. We present 4 patients, between 53 and 73 years of age, with this disease. It is often an asymptomatic lesion that can manifest, even at its apex, with mild pain when moving the scapula. All our patients had pain.The diagnosis is based on clinical findings and imaging studies, especially ultrasound, computed tomography, and nuclear magnetic resonance. The biopsy is reserved for patients who have no characteristic signs on imaging. In our series, surgical excision was necessary in one of the patients, and in the others, clinical and imaging studies allowed us to arrive at a definitive diagnosis.

Coronary artery aneurysms in Wegener's granulomatosis.

Atherosclerosis is the main cause of coronary artery aneurysm, however they can be observed in connective tissue diseases such as systemic lupus erythematosus and vasculitis. Kawasaki's disease and polyarteritis nodosa (PAN) are the systemic vasculitis that more often present coronary artery aneurysms. There are descriptions in the literature that small vessel vasculitis such as microscopic polyangiitis and PAN could develop coronary artery aneurysm, which are infrequent in other ANCA-associated vasculitis. Here, we report the case of a 25-year-old man who developed an extensive anterior myocardial infarct. The coronary angiogram showed coronary artery aneurysms, on laboratory ANCA C positivity with elevated levels of anti-proteinase 3 antibodies were present. He was treated with high doses of corticosteroids and cyclophosphamide with resolution of the aneurysms.

A girl with cutaneous lesions, polyarthritis, and antinuclear antibodies positivity.

On October 1996, a 14-year-old girl was admitted to the hospital because cutaneous lesions, asthenia, and arthralgias. On examination, there was nonscarring hair thinning with a widened part over the frontal hairline, polymorphic papulosquamous rash on her face, neck, arms, and trunk, and livedo reticularis in her legs. Multiple aphtous ulcers were present on the buccal and nasal mucosa. There was polyarthritis involving the wrist, metacarpophalangeal joints, proximal interphalangeal joints, and metatarsophalangeal joints of both hands and feet. Skin biopsy of the face was compatible with subacute cutaneous lupus erythematosus. She started on prednisone 60 mg/d without improvement, and later hdroxhchloroquine (HCQ) 6 mg/kg/d was added for one year. Cutaneous lesions were almost healed, with just a hypopigmented macules left. Over the last 14 years, she has not shown any cutaneous or systemic manifestations.

Shoulder manifestations of diabetes mellitus.

The musculoskeletal system can be affected by diabetes in a number of ways. The shoulder is one of the frequently affected sites. One of the rheumatic conditions caused by diabetes is frozen shoulder (adhesive capsulitis), which is characterized by pain and severe limited active and passive range of motion of the glenohumeral joint, particularly external rotation. This disorder has a clinical diagnosis and the treatment is based on physiotherapy, non-steroidal anti-inflammatory drugs (NSAIDs), corticosteroid injections and, in refractory cases, surgical resolution. As with adhesive capsulitis, calcific periarthritis of the shoulder causes pain and limited joint mobility, although usually it has a better prognosis than frozen shoulder. Reflex sympathetic dystrophy, also known as shoulder-hand syndrome, is a painful syndrome associated with vasomotor and sudomotor changes in the affected member. Diabetic amyotrophy usually affects the peripheral nerves of lower limbs. However, when symptoms involve the shoulder girdle, it must be considered in the differential diagnosis of shoulder painful conditions. Osteoarthritis is the most common rheumatic condition. There are many risk factors for shoulder osteoarthritis including age, genetics, sex, weight, joint infection, history of shoulder dislocation, and previous injury, in older age patients, diabetes is a risk factor for shoulder OA. Treatment options include acetaminophen, NSAIDs, short term opiate, glucosamine and chondroitin. Corticosteroid injections and/or injections of hyaluronans could also be considered. Patients with continued disabling pain that is not responsive to conservative measures may require surgical referral. The present review will focus on practice points of view about shoulder manifestations in patients with diabetes.