SYSTEMIC LUPUSERYTHROMATOSUS MANIFESTING AS NEPHROTIC SYNDROME BY DR.TAMPHASANA WAIROKPAM, 1Yr Resident DEPT. OF INTERNAL MEDICINE, KVGMCH, SULLIA
• Herewith I am presenting an interesting case- 22 yrs old female patient ,married from Periyapattna, who was referred to us from Dermatology dept for e/o edema & facial puffiness .
• Her primary problem, which brought her to dermat. dept was facial rash of 1 yr duration, the rash used to aggravate & cause burning pain on exposure to sun- suggestive of photosensitivity- malar rash .• However she did not had rashes over other sun exposed area.
• She developed Facial puffiness & edema of legs for 1 month, apparently more in the morning hours, but there was no oliguria or haematuria.• On further enquiry she had chronic mild Intermittent ,on & off fever, headache, easy fatiguability , weight loss ,anorexia, recurrent oral ulcers, & alopecia.• She had bilateral wrist and knee joint pains for last 1 year.
• She did not had similar problems prior to 1yr.• She was not on any regular medication & there was no history of drug allergy .• She was on mixed diet , she had decreased appetite & decreased sleep.• Her Bowel and bladder habits regular.• She attained menarche at 13 yrs , cycles regular(4/30) &there was no h/o menstrual irregularity.• She is married & there was no h/o pregnancy loss• No similar complaints in the family
To summarise• Young married lady with – Fever, Arthralgia, Malar rash, photosensitivity, & other constitutional symptoms for ~ 1 yr , now presenting with Edema & facial puffiness.
• Edema & Facial puffiness on the background of prolonged illness – Renal • Nephrotic syn • Immune mediated AGN • Renal failure – Malabsorption – Severe Anemia & hypoprotenemia of chr. Disease
• In this patient we considered CT disease SLE – Arthralgia – Typical rash – Photosensitivity – Oral ulcers• Complicated by – ? Nephrotic syndrome – ? Renal failure
General physical examination• General examination:• Patient is conscious, cooperative, comfortably sitting on bed, well oriented to time, place & person.• Her BMI was 16.06• Her temp 100`F,• PR 98b/m, regular, normal volume, character , all peripheral pulses felt.• BP :170/100 mm Hg.• RR 16/min.• She was Pale & had Malar rash, Facial puffiness, periorbital edema & b/l Pitting pedal edema .• There was no icterus, cyanosis, clubbing, lymphadenopathy or orogenital ulcers .• Her Fundus showed grade 2 htn retinopathy.• There was no skeletal or joint deformity or e/o active arthritis.
Systemic examination• All systemic examinations were normal except for a short systolic murmur in the mitral area.• CVS: apex in the 5th ICS in the MCL ,S1,S2 heard, systolic murmur in the mitral area.• P/A-soft ,no tenderness, no organomegaly, BS+.• CNS-NFND, plantars are b/l flexors.• RS- Clear VBS b/l
SUMMARY• 22 yrs old lady with• Malar rash• facial puffiness & B/L Leg edema• Pallor• Hypertension & Gr II retinopathy• Systolic murmur at apex.
Investigations revealed :• Anemia(hb- 5.1 gm%)• Thrombocytopenia ( 70,000)• Leucopenia (2600)• ESR: 77 (raised)• 24 hrs urinary protein: 5.1 gm• Normal GFR• Hypoalbuminemia( S alb-: 2 gm/dl)• ANA : +• Anti ds DNA : +• Anti histones +• USG ABD/Pelvis – mild hepatosplenomegaly, & b/l mild pleural effusion.• Other investigations were normal.
Diagnosis - SLE• Young lady with Fever, Arthritis, Malar rash, Photosensitivity, oral ulcer• Anemia, Thrombocytopenia, leukopenia• High ESR• Pleural effusion• + ANA & Ds DNA• Nephrotic proteinuria
• Management:• Inj. Methyl prednisolone 1g iv OD for 3 days.• followed by oral prednisolone 40 mg/day in divided doses.• Diuretics, anti- hypertensives, hematinics & blood transfusion.• At discharge, oral prednisolone 40 mg in divided doses was advised to be tappered after 1 month over a peroid of 1 month.
• At discharge: Pt is comfortable, stable, edema & facial puffines had reduced markedly.• P.R- 82 bpm.• B.P- 150/90mm hg – Hb- 7.2 gm% – TLC- 4,400 – DLC-N75,L 18, M 7 – PLT – 92,000. In view of development of renal involvement(NS) , pt was referred to higher centre for renal biopsy and further management with immunosuppressive drugs.
Review on SLE• Systemic lupus erythematosus (SLE) is an autoimmune disease in which organs and cells undergo damage mediated by tissue-binding autoantibodies and immune complexes.• Ninety percent of patients are women of child-bearing years.• Prevalence of SLE is 15–50 per 100,000.
CausesDEFINITE FACTOR : Ultraviolet B lightPROBABLE FACTOR : Sex hormones; F:M ratio is 9:1 ;3:1 in young and old.POSIBLE FACTOR :1)Dietary -high intake of saturated fats2)Infectious agent -Bilateral DNA human retroviruses, endotoxins, bacterial toxins.3)Medication –Hydralazine, Procainamide, INH, Hydantoins, Chlorpromazine, Methyldopa, d-penicillamine, minocycline, TNFα-antibodies.
Triggering agents Abnormal immune regulation T cellsExcessive cytokine release B cells Autoantibody formation Immune complexes build up in the tissues and can cause inflammation, injury to tissues, and pain.
Criteria for diagnosis of lupus A person shall be said to have SLE if any 4 or more of the 11 criteria are present, serially or simultaneously, during any interval of observationS.N Criteria Definition Fixed erythema, flat or raised, over the malar 1 Malar Rash eminences, tending to spare the nasolabial folds Erythematous raised patches with adherent 2 Discoid Rash keratotic scaling and follicular plugging; atrophic scarring may occur in older lesions. Skin rash as a result of unusual reaction of 3 Photosensitivity sunlight. Oral or Nasopharyngeal ulceration, usually 4 Oral Ulcers painless. Nonerosive arthritis involving 2 or more peripheral 5 Arthritis joints, characterized by tenderness, swelling, or effusion.
S.N. Criteria Definition a) Pleuritis –convincing history of pleuritic pain or rubbing heard by a physician or evidence of pleural effision. 6 Serositis OR b) Pericaditis-documented by ECG or rub or evidence of pericardial effusion. a) Persistent proteinuria greater than 0.5 gms. per day or greater than 3+ if quantitation not performed. Renal 7 OR Disorder b) Cellular casts- may be red cell, hemoglobin, granular, tubular, or mixed. a) Seizures –in the absence of offending drugs or known metabolic derangements; e.g.-uremia, Neurologic ketoacidosis, or electrolyte imbalance. 8 Disorder b) Psychosis –in the absence of offending drugs or known metabolic derangements; e.g.-uremia, ketoacidosis, or electrolyte imbalance.
a) Hemolytic Anemia –with reticulocytosis OR b) Leukopenia –less than 4,000/mm Hematolo OR9 gic Disorder c) Lymphopenia –less than1,500/mm OR d) Thrombocytopenia –less than 100,000/mm in the absence of offending drugs Immunolo Anti- dsDNA, Anti-Sm and/ or anti phospholipid10 gic Disorder Antinuclea Abnormal titre of ANA at any point in time in the11 r absence of drugs known to induce ANAs. antibodies
Management1) Musculoskeletal? NSAIDsHydroxychloroquine + steroid -5-10mg/dayMethotrexate 10-20mg/weekPersistent pain in one joint only- ischaemic necrosis of bone2) Cutaneous LupusProtective clothingSunscreen lotionLocal glucocorticoidsHydroxychloroquin 400mg/dayQuinacrine 100mg/dayEtiretinate 1mg/kg/day in divided dosesDapsone, ThalidomideCytotoxic drugs
3) Serositis- • NSAIDs + Steroids 10-15mg/day4) Aggressive TherapyManifestation usually responsive to high dose glucocorticoids Vasculitis Severe dermatitis of SCLE or SLE Polyarthritis Polyserositis – Pericarditis, pleurisy, peritonitis Myocarditis Lupus pneumonitis Glomerulonephritis – proliferative forms Hemolytic anemia Thrombocytopenia Diffuse CNS syndrome – acute confusional state, demyelinating syndromes, intractable headache, serious cognitive defects Myelopathies . Peripheral neuropathies Lupus crisis – high fever and prostration
Cytotoxic Drugs Initial Maint. Drug Advantage Adverse side effect Inci. Dose DoseAzathiopri-- 1-3 1-2 Probably Bone marrow <5 ne mg/kg/ mg/kg/ reduces suppression, Day Day flares, Leukopenia 15requires reduces renal Infection (herps 106-12 scarring, zoster), months reduces Malignancies, <5to work glucocorticoid Infertility, 15Well dose Early menopause 10 Requirement Hepatic damage <5 Nausea 15Cyclophop- 1-3 0.5 As for Bone marrow <5 -hamide mg/kg/ mg/kg/ azathioprine suppression day Day probablyRequires orally or Orally or effective in2-16 8-20mg/ 8-20mg/kg higherweeks to kg IV IV Every proportion ofwork well once a 4-12 Wk + patient month Mesna + mesna
Combination TherapyAzathio- 1.5-2.5 1-2 Possibly more Infections 40-prine mg/kg/ mg/kg/ effective than day day one drug orally Orally plus Cystitis 15Cyclophpspo- 1.5-2.5 1-2 mide mg/kg/ mg/kg/ day day orally orally
Prognosis of SLE• Survival in patients with SLE in the United States, Canada, Europe, and China is approximately 95% at 5 years, 90% at 10 years, and 78% at 20 years.• In the United States, African Americans and Hispanic Americans have a worse prognosis than Caucasians, whereas Africans in Africa and Hispanic Americans with a Puerto Rican origin do not.• Poor prognosis (~50% mortality in 10 years) in most series is associated with (at the time of diagnosis) – high serum creatinine levels [>124 mol/L (>1.4 mg/dL) hypertension – nephrotic syndrome (24-h urine protein excretion >2.6 g) – anemia [hemoglobin <124 g/L (<12.4 g/dL)] – hypoalbuminemia – hypocomplementemia – aPL
• This case is presented to showcase most of the common clinical manifestation of a rare disease.