Systemic Lupus Erythematosus
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PowerPoint Presentation Systemic Lupus Erythematosus Sheelendra Shakya Dept. of Pediatrics, KMCTH Introduction A complex disorder of multifactorial origin resulting from interactions among genetic, hormonal and environmental factors acting in concert to cause activation of helper T cells and B cells that results in the secretion of several species of autoantibodies. In this complex web, each factor may be necessary but not enough for the clinical expression of the disease; the relative importance of various factors may vary from individual to individual. Topics of Discussion Pathogenesis Clinical presentation Diagnosis Useful investigations Lupus nephritis Treatment Prognosis Pathogenesis Fundamental defect is a failure of the regulatory mechanisms that sustain self-tolerance. Anti-bodies identified against Nuclear components Cytoplasmic components Cell surface antigens of blood elements incl. phospholipids Pathogenesis Genetic factors Hormonal Influences Environmental Triggers Immune Dysregulation Pathogenesis Pathogenesis Most visceral lesions are mediated by immune complexes (Type III hypersensitivity) Autoantibodies against red cells, white cells and platelets mediate their effects via Type II hypersensitivity. In tissues, nuclei of damaged cells react with ANAs, lose their chromatin pattern, and become homogeneous, to produce LE bodies. Case study Swastika Maharjan 8yrs/F from Baneshwor DOA: 065.1.3 c/o puffiness of face, swelling of lower limbs, oral ulcer and skin rash, fever since 3 days. Started e fever followed by facial puffiness and generalized body swelling 1 mth back. Diagnosed as AGN at Kanti and treated with antibiotics, diuretics and steroid. Discharged on improvement. On follow up, antibiotics changed, Urine albumin +++; Diuretics continued. After 12 days of stopping diuretics, swelling reappeared. Diagnosed as Nephrotic Syndrome e culture proven UTI. Treated with Ofloxacin. HPI After 7 days of treatment with Oflo, child developed oral ulcers and skin rashes suspected to be drug rash and was switched to Cefixime. Was brought to KMC. Also h/o fever, multiple oral ulcers and skin rashes, more on face and few on the thigh, erythematous, macular, non itching e h/o decreased urine output. Occassional c/o joint pains. No h/o SOB, photosensitivity, burning micturition. CRP >6mg/dl, Urine RME : RBC and pus plenty, albumin + TC8400cumm N82L17M1 Hb 13gm% ESR 52mm in 1st hr O/E Ill looking, oral ulcers, facial puffiness, malar rash Pallor +, B/L pitting edema of lower limbs Chest: dullness from 5th ics downwards on Rt. and 3rd ics downwards on Lt.; decreased B.S. on lt. inframammary region CVS: S1+ S2+ M0 P/A: umbilicus everted, mild distension, soft, non tender, no organomegaly, shifting dullness +, fluid thrill â Pigmented rash on lateral aspect of rt. thigh Investigations CBC:TC 3500 N63L36M1;Hb 6.9gm%; PLT 42000/cumm ESR 72mm in 1st hour Peripheral Smear: normocytic, normochromic to mildly hypochromic e mild anisocytosis. Plt reduced on smear Biochemistry: Urea 96mg/dl Cr 1.1mg/dl; Na 123meq/L, K 4meq/L Total protein: 4.3g/dl Albumin: 3.6g/dl Total Cholesterol 210 mg/dl HDL: 27mg/dl Triglycerides: 697mg/dl LDL could not be calculated. ANA , CRP, ASO, RA factor- negative Urine: pus 6-8/hpf, RBC plenty; Albumin 2 +, cast 0-2 24 hour urine volume: 350ml 24 hour urinary protein 2717mg. Urine C/S- insignificant bacteriuria Montoux test: no induration after 72 hrs Reticulocytes: 4% Albumin repeated after 2 weeks: 1.8mg/dl Anti-dsDNA by ELISA- positive (69.12 IU/ml) USG: B/L medicorenal disease B/L pleural effusion Minimal pericardial effusion. Ascites CXR: B/L pleural effusion Repeat Urine RMEs: persistent albumin, RBC and pus cells. Diagnosis SLE with secondary Nephritic Syndrome with Culture proven UTI Child was started on Steroid and diuretics. Antibiotics for UTI continued and she was closely monitored. During the course of treatment, she got symptomatically better but ascites increased and her BP was also in a rising trend. Due to parental wish and the need for specialist nephrologist expertise, she was referred to Kathmandu Nursing Home where she was continued with the same medication. Clinical Presentation Skin manifestations Renal disease Neuropsychiatric Arthritis Cardiac Respiratory Haematology Gastrointestinal Muscles and bones Joint/muscle pain, myositis, proximal myopathy. Any joint can be affected, but the most common spots are the hands, wrists, and knees. Usually the same joints on both sides of the body are affected. The pain can come and go, or it can be long lasting. Non-erosive polyarthritis with periarticular and tendon involvement. Skin manifestations Butterfly rash Alopecia Oral ulcers Photosensitivity Skin manifestations Discoid lupus Ears, cheeks, scalp, forehead, chest 3 stage rash â Erythema Pigmented hyperkeratotic oedematous papules Atrophic depressed lesions Livedo reticularis (net-like rash) Raynaudâs purpura Urticaria Conjunctivitis Bullae Renal Proteinuria Casts Oedema Uraemia Acute glomerulonephritis Mesangial proliferative lupus nephritis Advanced sclerosis lupus nephritis Membranous lupus nephritis Neuropsychiatric Central Nervous System Headache Mood disorder Cognitive dysfunction Seizure disorder Acute confusional state Anxiety disorder Cerebrovascular disease Psychosis Movement disorder Demyelinating syndrome Aseptic meningitis Myelopathy Peripheral Nervous System GBS Autonomic neuropathy Mononeuropathy Myasthenia gravis Cranial neuropathy Plexopathy Polyneuropathy Neuropsychiatric syndromes in SLE as defined by American College of Rheumatology Respiratory Pleuritis (+/-Pleural effusion) Acute pneumonitis Chronic interstitial lung disease Pulmonary haemorrhage Pulmonary Oedema Shrinking Lung Syndrome Restrictive lung impairment with no respiratory symptoms Cardiac Cardiac Pericarditis (+/- effusion) Myocarditis Coronary Artery Disease Libman-Sacks endocarditis Valvular insufficiency Cardiomegaly Myocardial perfusion defects - IHD Hematological Thrombocytopenia Leucopenia Lymphopenia Anaemia due to chronic disease or secondary to Coombâs positive haemolytic anaemia INR, ESR increased CRP normal, unless intercurrent infection. Gastrointestinal Hepatosplenomegaly Nausea and vomiting Constitutional Fatigue Fever Weight loss Lymphadenopathy Diagnostic criteria Malar rash Discoid lupus Photosensitivity Oral or nasal ulceration Non-erosive arthritis Serositis Nephritis Neurological Haematological Immunological Anti-nuclear antibody Fixed erythema, flat or raised, over malar eminences, tending to spare nasolabial folds Erythematous raised patches of adherent keratotic scaling and follicular plugging. Atrophic scarring may be seen in older lesions. Skin rash as a result of unusual reaction to sunlight (UV rays) Oral or nasopharyngeal ulcers, usually painless, observed by a physician Non-erosive arthritis involving 2 or more peripheral joints, characterised by tenderness, swelling or effusion Pleuritis â pleuritic pain, rub heard or pleural effusion, or, Pericarditis â documented by ECG or rub or pericardial effusion Persistent proteinuria > 0.5g/day not > 3+. Cellular casts: red cell, haemoglobin, granular, tubular or mixed. Seizures or psychosis in the absence of offending drugs or metabolic derangement. Haemolytic anaemia with reticulocytes, or: Leucopenia < 4000/mm3 on 2 or more occassions, or: Lymphopenia