Systemic Lupus Erythematous: A Case Study

Ms. Jasmine Kaur

Assistant Professor, Desh Bhagat University School of Nursing, Mandi Gobindagarh

*Corresponding Author Email: dhumijasmine88@yahoo.com

ABSTRACT:

Background: Systemic lupus Erythematosus (SLE) is a multisystem autoimmune inflammatory disorder involving both humoral and cellular aspects of the innate and acquired immune systems. It is chronic condition that is characterised by various degree of increased disease activity that are generally followed by a less active, remitting.

Objective: To describe the clinical features and complications of SLE and discus the current management. Methods: Detailed history, physical examination and laboratory investigations.

Conclusion: This report underscores the importance of physical features which depicted multi-organ involvement and diagnostic evaluation especially ANA (Antinuclear Antibody) in the diagnosis of systemic lupus erythematosus (SLE). Management of this disease should be individualized and should include both pharmacological and non-pharmacological modalities for symptom relief and resolution for improved quality of life.

KEYWORDS: Systemic Lupus Erythematosus, Antinuclear antibody, cellular, Autoimmune, Remitting.

INTRODUCTION:

Systemic lupus Erythematosus (SLE) is a multisystem autoimmune inflammatory disorder involving both humoral and cellular aspects of the innate and acquired immune systems.¹ SLE is chronic condition that is characterised by various degree of increased disease activity that are generally followed by a less active, remitting. Typically, multiple bodies organs and systems are affected at different times, thus producing widespread damage to connective tissues, blood vessels and serous and mucus membranes.² The prevalence of SLE varies with race, ethnicity and socioeconomic status. In the general population, the incidence of SLE was 23.2/100,000.³ It has a multifactorial aetiology and effects mainly women during the childbearing years. The various factors include genetic, hormonal and environmental components.⁴ Further, insight into pathogenesis of systemic lupus erythematosus was enhanced by discovery of the lupus erythematosus cell phenomenon and antinuclear body.

These complexes get deposit on the tissue, results in tissue injury and ultimately bring about the symptoms of lupus. The cellular and molecular mechanisms of lupus are unknown but it is believed that genetic, non-genetic and immunological factors are involved.⁵ A variety of disease manifestations are exhibited by SLE patients with the heterogeneity of presentations often delaying diagnosis. Common manifestations include rashes, photosensitivity, arthritis, pleuritis, pericarditis, nephritis, neuropsychiatric disorders, and hematological disorders. There is also an array of less common but potentially hazardous complications.⁶ The diagnosis ofsystemic lupus erythematosus is based on clinical and laboratory criteria. Serological biomarkers hold a significant potential in diagnosis and monitoring of SLE given that the pathogenesis is most likely the result of immune dysregulation. Anti-double -stranded DNA (anti-ds DNA) is highly specific for lupus, with 70% of SLE patients being positive in comparison with only 0.5% of the healthy population or those with other autoimmune diseases.⁷ In contrast, antinuclear antibody is highly sensitive, being positive in 99% of SLE patients at some point in their illness, but is also found in 32% of the general population at a 1:40 dilution and in 5% at a 1:160 dilution. Routine laboratory testing is recommended for monitoring of SLE patients.⁸ Treatment of systemic lupus erythematosus (SLE) is guided by the individual patient's manifestations. Fever, rash, musculoskeletal manifestations, and serositis generally respond to treatment with hydroxychloroquine, nonsteroidal anti-inflammatory drugs (NSAIDS), and steroids in low to moderate doses, as necessary, for acute flares. Medications such as methotrexate may be useful in chronic lupus arthritis, and azathioprine and mycophenolate have been widely used in lupus of moderate severity.⁹

Case Report:

A 24 year-old young female was admitted in emergency intensive care unit of Dayanand Medical College, Ludhiana, Punjab with the history of skin lesions, bluish discoloration of tips and fingers, vasculitis from last four years and she was diagnosed with Systematic Lupus Erythematosus.

Clinical Evaluation revealed intermittent ferver, malar rash, discoid rash, oral ulcers, steroid induced severe headache which was not even relieved with NSAIDS or tramadol, arthritis of bilateral elbows and knees, alopecia, pedal edema and periorbital puffiness. Her pulse rate and respiratory rate were 120 and 30 per minute showed tachycardia and tachypnea. Her oral temperature was 100 ⁰F.

In the laboratory examination, haemoglobin level was 9.9 gm/dl which depicted the moderate anemia, WBC count =3800/mm³ showed leukopenia. Bilirubin level was in the normal range but urinary analysis showed mild pyuria and haematuria (WBC count upto 7 high power field and RBC count was 25-30/hpf). The results of laboratory test on the 5^th day of admission are presented in Table 1.

She was also further diagnosed with glaucoma and patient was advised for surgery but her family refused but she was also diagnosed with Pulmonary Koch with Chest X-ray, sputum test and started on Anti tuberculosis Drug currently on Continuation Phase Tab.Rifampcin and Tab. Isoniazid. It is believed that Methotrexate used to reduce the activity of systemic lupus erythematosus and of rheumatoid arthritis had decreased the activity of his immune system and tends to develop tuberculosis.

In the diagnostic evaluation, Ultrasonography examination depicted enlarged multiplecalcifications in the spleen, multiple cortical cysts in the bilateral kidney, small amount of fluid in the peritoneal cavity. CT scan was also done and depicted the bilateral abscess on orbital region and left anterior temporal region.

According to patient, she took treatment from various hospitals but symptoms get worsened. Then, Inj Macrogen (immunostimulant), Salmoterol (bronchodilator) and tramadol for severe headache was prescribed to the patient. This prescribed treatment was beneficial and patient’s symptoms were improved. After that, patient was discharged on 15^th day be advising her to come for regular follow ups.

Table 1: Rheumatologic and routine laboratory tests on the 5^th day after admission

Laboratory tests	Rheumatologic test
WBC = 4200/mm^3 Hb = 9.7 g/dl Platelet = 30000/mm^3 Amylase =103 IU/L D-Dimer = 1.1mg/dl Urea = 41 mg/dl Creatinine = 0.68 mg/dl Albumin = 1.5 gm/dl PT=20 seconds PTT= 60 seconds CK MB = 25 H U/L ESR = 60mm/hr	ANA = 1/140 titer AntiLKM = Negative ANCA =Negative

WBC: White Blood Cell; Hb: Hemoglobin; ALKP: Anti LKM: Anti Liver-Kidney Microsomes; PT: Prothrombin Time; PTT: Partial Thrombin time; ESR: Erythrocyte Segmentation Rate; CK-MB: Creatinine Kinase Myoglobin; ANA: Antinuclear Antibody; ANCA: Antinuclear Cytoplasmic Antibody

DISCUSSION:

Pathophysiology and Etiology:

The pathogenesis of SLE is summarized diagrammatically in Fig 1. Multiple genes confer susceptibility to disease development. Interaction of sex, hormonal milieu, the HPA axis, and defective immune regulation, such as clearance of apoptotic cells and immune complexes, modify this susceptibility. The loss of immune tolerance, increased antigenic load, excess T cell help, defective B cell suppression, and shifting of Th1 to Th2 immune responses lead to cytokine imbalance, B cell hyperactivity, and the production of pathogenic autoantibodies.In addition, environmental factors, such as chemicals and drugs, ultraviolet light, dietary factors, viruses, and environmental oestrogen are probably required to precipitate the onset of the disease.¹⁰

Figure 1: The pathogenesis of systemic lupus erthyematosus.APC, antigen presenting cell

MANAGEMENT:

There is no permanent cure for SLE. The goal of treatment is to relieve symptoms and protect organs by decreasing inflammation and the level of autoimmune activity in the body. The precise treatment is based on an individual basis. Many people with mild symptoms may need no treatment or only intermittent courses of anti –inflammatory medications. Hydroxychloroquine (Plaquenil) is an antimalarial medication found to be particularly effective for SLE people with fatigue, skin involvement, and joint disease. Consistently taking Plaquenil can prevent flare-ups of lupus. Researchers have found that Plaquenil significantly decreased the frequency of abnormal blood clots in people with systemic lupus. For resistant skin disease, alternative medications like include dapsone and retinoic acid (Retin-A

). Retin-A is often effective for an uncommon wart-like form of lupus skin disease.

In recent years, mycophenolate mofetil (CellCept) has been used as an effective medication for lupus, particularly when it is associated with kidney disease. Cell Cept has been helpful in reversing active lupus kidney disease (lupus renal disease) and in maintaining remission after it is established.

In SLE patients with serious brain (lupus cerebritis) or kidney disease (lupus nephritis), plasmapheresis is sometimes used to remove antibodies and other immune substances from the blood to suppress immunity.

Most recent research is indicating benefits of rituximab (Rituxan) in treating lupus. Rituximab is an intravenously infused antibody that suppresses a particular white blood cell, the B cell, by decreasing their number in the circulation.

Another new B-cell-suppressing treatment is belimumab (Benlysta). Belimumab blocks the stimulation of the B cells (a B-lymphocyte stimulator or BLyS-specific inhibitor) and is indicated for the treatment of adult patients with active, autoantibody-positive systemic lupus erythematosus who are receiving standard therapy.

Life style adaptations such as need more rest during periods of active disease. Researchers have reported that poor sleep quality was a significant factor in developing fatigue in people with SLE.¹¹

CONCLUSION:

SLE is a very complex disease with multifactorial etiology and multiple organ involvement making the diagnosis challenging. Clinical manifestations as well as immunological abnormalities assist in the diagnosis. Management of SLE depends on the level of disease activity and can include general measures, NSAIDs and steroids. Literature reveals an ongoing research to improve the quality of life and increase survival of patients affected by SLE.

ACKNOWLEDGEMENT:

Many thanks to the patient and Emergency Unit team of DMC Hospital who were actively involved in taking care of this patient.

REFERENCES:

1. Khan Saba, Alam Roshan, Moinuddin and Ali Asif. Oxygen Free Radical Modified DNA: Implication in the Etiopathogenesis genesis of Systemic Lupus Erythematosus. Indian Journal of Clinical Biochemistry.2009; 24(2):123.

2. Management of patients with systemic connective tissue diseases: Systemic lupus erythematosus (SLE) systemic sclerosis (SS) [cited 17 March 2015] Available from URL: http://www.ntranet.tdmu.edu.ua/.../09.%20MOP%20with%20systemic%20connective.

3. Candace Feldman, Linda T. Hiraki. Jun Liu, Michael A. Fischer, Daniel H. Solomom, Graciela S. Alarcon et.al. Epidemiology and Sociodemographic of Systemic Lupus Erythematosus and Lupus Nephritis among U.S. Adults with Medicaid Coverage, 2000–2004.J Arthritis Rheum.2013 March; 65(3):753-763.

4. Mandell GL and Petri WA. Antimicrobial agents: pencillins, cephalosporin’s and other B-lactam antibiotics. A review.

5. Hussain N, Jaffery G, Sabri A.N., Hasnain S, Mir N. Pulmonary Tuberculosis in a Patient with Systemic Lupus Erythematosus – A Case Study.Annals.2008;14(2):79-80.

6. ErezBen–Menachem, Systemic Lupus Erthyematosus: A review for Anesthesiologists.2010; 111(3):686.

7. Ratiman A, Isenberg DA. Systemic Lupus Erthyematosus. N Eng J Med 2008; 358:929-39.

8. Gill JM, Quisel AM, Rocca PV, Walters DT. Diagnosis of Systemic lupus erythematosus. Am Fan physician.2003; 68:2179-86.

9. Bartels M Christies. Systemic Lupus Erthyematosus Medication. A review [cited 17^th March 2012] Available from:emedicine.medscape.com/article/332244.

10. C. C. Mok, C S Lau. Pathogenesis of systemic lupus erythematosus. Clin Pathol.2003; 56:481-490.

11. Lupus Symptoms, Causes, Treatment. Available from < http:// www.medicinenet.com>arthritis list>systemic lupus index.

Received on 20.03.2015 Modified on 11.04.2015

Accepted on 21.04.2015 © A&V Publication all right reserved

Asian J. Nur. Edu. and Research 5(4): Oct.- Dec.2015; Page 501-504

DOI: 10.5958/2349-2996.2015.00103.2