A case report of old age systemic lupus erythematosus

Manuscript type : Case Report | Article Date : 2015/08/16

  • Autors

    1. Almasi Simin
    2. Ebrahimi Nafiseh
    3. Zamani Kazem
  • Abstract

    Systemic lupus erythematosus (SLE) is a chronic autoimmune disease that predominantly affects young women. Old age lupus occurs after the age 50 years and the disease occurs in 10-20% of patients over 50 years. This case was a 78-year-old woman who admitted to our hospital with polyarthritis, dyspnea, chest pain, typic malar rash, thrombocytopenia, lymphopenia:, positive antinuclear antibody (ANA):,and high titer anti-dsDNA from 3 weeks before admission. However older patients are more likely to have an insidious presentation and anti-ds DNA was very high and the other hand malar rash, polyarthritis and thrombocytopenia was reported very rare in old age in literatures bur our patients had acute presentation and her disease pattern similar pattern of disease in young patients.
  • Description

    INTRODUCTION

    Systemic lupus erythematosus (SLE) is a chronic autoimmune disease predominantly affecting young people and showing a well-known female predilection. Lupus occurs in 10-20% of older patient. Old age lupus is the type of SLE whose manifestations begin after the age of 50 years.1 However, in recent studies age of 65 years or greater has been recommended.2 Studies suggest that the clinical manifestation and serological features of old age lupus differ from lupus in younger patients. The female: male sex ratio declines from a 10:1 ratio in the younger patient to a 4:1 ratio in the older patient.3 This reduction has been related to the absence of sex hormones.4 Caucasian are more affected old age lupus in studies in multi-ethnic groups.5 Unlike to patients with regular lupus, old age lupus at the beginning of its course is characterized by the presence of nonspecific symptoms such as weight loss, arthralgia, weakness, fatigue, and cognitive disorders.6 Serositis, sicca symptoms, raynaud phenomenon, lung disease, fever and neuropsychiatric manifestation are more common in patients with old age lupus, while malar rash, discoid lupus and glomerulonephritis are less common in old age lupus compared with younger lupus patients. Unlike to younger patients in old age lupus the onset is gradual.7 Majority of old age lupus patients have a positive anti-nuclear antibody test (ANA), but anti-double-stranded DNA and hypocomplementaemia is lower in elderly-onset lupus patients. Rheumatoid factor (RF), anti-Ro/ (SS) A and anti-La/SSB are more positive in old age lupus patients.8 Aging is associated with decreased immune function. The thymus becomes smaller in old age however some thymic function intact.9 Often oligoclonal expansion develops, particularly of CD8+ T cells.10 It is also associated with reduction of B-cell lymphopoiesis.11 There is a shift from Th1 to Th2 cytokine with inceasing age and in old age lupus there are increased some autoantibody production such as RF, anti-Ro and anti-La antibodies.12 In old age lupus pro-inflammatory cytokines IL-6 and tumor necrosis factor-a may increased and this can explain the development of autoimmune disease in elderly individuals. On the other hand, a reduction of IL-2 (with the reduction in the activation of Tregulatory cells) has been shown with aging.13 The diagnosis of old age lupus may be delayed for many months: insidious onset, low prevalence and similarity to other more common disorders make the diagnosis of lupus challenging in this population. This may give rise to a delay in diagnosis due to lack of typical clinical manifestations. Here we report a case of late onset-SLE associated with acute and typical lupus manifestation similar young people lupus.

    CASE REPORT

    A 78-year-old woman was admitted to our hospital with polyarthritis in upper and lower limbs, dyspnea (functional class 3), retrosternal chest pain, pitting edema in lower limbs, malar rash from 3 weeks ago. Upon admission he was afebrile vital signs on presentation included blood pressure 120/70 mmHg, pulse rate 85 per minute with regular rhythm, body temperature 37.0 C°, and respiration 26 per minute. In joint examination symptoms of arthritis included swelling, erythema and severe tenderness was in all MCP (metacarpophalangeal) joints, knees, elbow and ankle joints. In lung examination crackle were heard at the base of both lung and 2+ pitting edema in lower extremities and jugular vein was prominent. The abdomen was soft and flat, and the spleen was not palpable, other abdominal physical exams were unremarkable. Neurological status was normal. Laboratory evaluation (Table 1), (Table 2) revealed thrombocytopenia and positive FANA and high titer anti-dsDNA. Urine analysis is normal without cast or protein and also Hbs-Ag, HCV-Ab, HIV-Ab all were negative. Bone marrow aspiration and biopsy was unremarkable and malignant cells were not found. Abdominal and pelvic sonography was normal. In echocardiography moderate aortic insufficiency, EF(ejection fraction) 30% and, PAP(pulmonary arterial pressure)33 mmHg and Doppler ultrasonography of the lower limbs unremarkable and in lung CT-Scan fibrotic band, diffuse bronchiectasis , honeycomb in left lung was seen. The patient had no history of taking medication that causes drug lupus. Ischemic changes were seen in patients" ECG (electrocardiography) in 1, avL, V1-V6 leads. Based on clinical manifestation, history and laboratory test our diagnosis was lupus and on the basis 1997 American College of Rheumatology (ACR) revised criteria for classification of SLE, in view of the presence of lymphopenia, positive ANA, high titer anti-ds DNA, poly arthritis, malar rash and thrombocytopenia. She was admitted in CCU and treated with prednisolon and hydroxychloroquine, her heart failure was treated and after two weeks the patient was discharged in good condition.

    DISCUSSION

    considered that the degree of organ damage is greater in old age lupus3; therefore, early diagnosis and treatment is necessary for these patients. In this patient’s serositis, interstitial lung disease (ILD) and Sjögren’s syndrome are more prevalent. Arthritis, arthralgia and cytopenia are initial manifestation of old age lupus and in our case it was the same, although it has been reported that anti-dsDNA and anti-Sm are present less frequently in late onset-SLE.14 But in our case anti-dsDNA was very high, our patient had malar rash and polyarthritis and thrombocytopenia that this manifestation was reported very rare in literatures. Diagnosis of SLE in elderly individuals is difficult because of the lack of typical physical and laboratory features that are usually present in younger patients. In conclusion SLE occurs rarely in patients of advanced age since our patient was very old and Review in literature shown that the incidence of the disease is extremely rare in this age and we were reported this patient because disease is very rare in this age. Therefore, careful attention needs to be paid to latent symptoms and laboratory findings.

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