A case profile of SLE

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In the Name of God, Most Gracious, Most Merciful

A CASE PROFILE OF SLE: 

- Dr. Mohammed Sadiq Azam - Dr. N.Samatha Postgraduates M-I, Prof. Md. Siraj’s Unit Department of Internal Medicine Deccan College of Medical Sciences A CASE PROFILE OF SLE

Case 1: 

November 2006 Case 1

History – chief complaints: 

History – chief complaints Mrs. A, 32/F, presented on 14/08/2006 with c/o: FEVER – 15 days (on/off since 6 months) Oral Ulcers – 15 days Loose stools – 15 days Generalized body pains – 15 days Generalised weakness – 15 days Generalised swelling – 15 days

History of presenting illness: 

History of presenting illness Asymptomatic 6 months ago, when she developed fever with chills and rigors for which she took symptomatic treatment – low grade fever +/- ever since. 1 month ago, she developed moderate to high grade fever with chills and rigor with generalized body pains involving all joints (non migratory, not flitting/fleeting, relieved by analgesics). Got admitted at a private nursing home on 4/5/2006 and has been treated for typhoid fever for one week with Inj. Cefotaxime 1g iv BD. No relief in symptoms even with therapy, hence got discharged and went home. One week later she developed swelling all over the body with oral ulcers and persistent low grade temperature with random spikes.

PAST HISTORY: 

PAST HISTORY No past H/O exposure to an adult case of TB, no past documented H/s/o of typhoid, malaria, dengue, chickungunya , exanthematous fevers in past/childhood, no rheumatic history. No h/o rash anywhere on face or body, no h/o photosensitivity. No h/s/o high risk behaviour . No h/o exposure to heavy metals.

Personal / FAMILY history: 

Personal / FAMILY history Mixed diet, sleep decreased, loss of weight / loss of appetite + Bladder and bowel movementts – normal. Recently married (<1yr), no issues, no abortions. Menstrual history: Menarche: 14 yrs, cycles 5/28, regular, normal flow, no pain, no clots. Teacher by occupation at a private school. Illness has affected her school performance (increased sick absenteeism) ALLERGIC to Penicillin. Family History: No similar complaints in family.

On examination:: 

On examination: Moderately built lady, sitting on the bed, is conscious, cooperative, coherent, oriented to time, place and person. Dyspnoeic , Tachypnoeic , Anaemic ++, BPPE ++, Facial puffiness +, Abdomen distended, no generalised LA, I -, Cy -, Cl -. Oral ulcers +, buccal mucosa 2 ulcers on each side, approx 1x2cm size, floor granular, egde sloping, no slough, sub mandibular tender LA + VITALS: BP-100/70mmHg, PR-95bpm, reg , normal vol & character, Temp: 100 F, JVP: raised (8mm) CVS: S1, S2 +, no murmurs/added sounds RS: BAE+, NVBS, B/L basal creps + occasionally P/A: Abd distended, FF + (SD+, FT -), no palpable organomegaly . CNS: NFND, PERRLA+, B/L plantars flexor.

PROVISIONAL DIAGNOSIS: 

PROVISIONAL DIAGNOSIS Pyrexia for evaluation: ? Koch’s ? Connective Tissue Disorder ? Malignancy

INVESTIGATIONS:: 

INVESTIGATIONS: CBP: Hb : 8.5g% , TC- 2,300 cells/cu.mm, DC – N79, L16, M3, E2, Platelet count- 1.09 lakh. Reticulocyte count: 2.5%, ESR- 35/40mm LFT – ALP, AST/ALT – Normal BU/SC – Normal (42/0.9) S.Albumin – 5g%  3.4g% (over 15 days) P/S: Pancytopaenia , BM aspiration: Hypercellular marrow, no abnormal/ immature cells in either. CxR PA: WNL, Mantoux : Negative S.Electrolytes : Na: 110, K: 3.9, Cl : 90, Urine Na:79 meq /l (20-80)

INVESTIGATIONS:: 

INVESTIGATIONS: CUE: Alb +  3 + (over 15 days) RBCs: NIL  5-6 (over 15 days) Pus cells 3-4  plenty (over 15 days) 24h urine protein: 1200mg/24 hrs Urine C/S : Sterile Blood for CRP: Positive Blood for RA Factor: Negative S.Uric acid: 4.5

INVESTIGATIONS:: 

INVESTIGATIONS: USG abdomen: Moderate ascites +, no organomegaly , no lymphadenopathy. Apparent mild thickening of rectal walls, bilateral mild pleural effusion. Ascitic fluid analysis : GM Stain: No organisms seen, AFB: Negative Protein: 1.7 mg/dl, Alb : 0.21mg/dl, Glucose: 91mg/dl ADA: 02 U/L, LDH: Normal Ascitic fluid cytology: only 2 cells – lymphocytes. HbsAg : Negative, HIV: Non Reactive

Going back… Provisional diagnosis: 

Going back… Provisional diagnosis Pyrexia for evaluation: ? Koch’s ? Connective Tissue Disease ? Malignancy

CRITERIA FOR SLE:: 

CRITERIA FOR SLE: Malar Rash Discoid Rash Photosensitivity Oral Ulcers Arthritis Serositis (Effusions/Ascites) Renal disorder (Proteinuria >0.5g/d or >/= 3+ or cellular casts) Neurologic disorder (Seizures, Psychosis with no other cause) Haematologic disorder ( Haemolytic anaemia or leucopaenia (<4000/ uL ) or lymphopaenia or thrombocytopaenia (<100000/ uL ) in the absence of offending drugs) Immunologic disorder (Anti dsDNA , anti- Sm , and/or anti- phospholipid Ab ) Antinuclear antibodies (ANA) positive

INVESTIGATIONS:: 

INVESTIGATIONS: ANA: POSITIVE Anti dsDNA : POSITIVE

final diagnosis: 

final diagnosis Connective Tissue Disorder: Systemic Lupus Erythmatosus

Treatment:: 

Treatment: Started on Steroids, Oral Prednisolone 1mg/kg/day Supportive therapy

FOLLOW UP:: 

FOLLOW UP: Patient developed GTCS involving Right UL/LL 3 days after initiating therapy. CT Brain (Plain): Focal hypodensity in Rt. High parietal lobe posteriorly (?infarct). Suggested review with contrast after 1 week Review scan: Hypodensities in bilateral parieto -occipital regions s/o infarct. Wedge shaped hypodense areas of alterations valus (16-24 Hz) noted in posterior parietal regions bilaterally. s/o Infarct, ? White matter oedema .

FOLLOW UP:: 

FOLLOW UP: MRI Brain with MR Venogram : Bilateral cortical and subcortical T2 hyperintensity in parieto -occipital and frontal lodes which may represent mutiple infarcts (? secondary to vasculitis ).

FOLLOW UP:: 

FOLLOW UP: Uptitrated the steroid Added LMWX, Sodium Valproate 300mg BD with Folic acid 1g OD Supportive therapy. Patient is on regular follow up. Conceived 6 months later, had a FTND, baby healthy, resumed job, has a healthy good QOL now.

Case 2: 

August 2002 Case 2

CASE SNIPPET: 

CASE SNIPPET Mrs. R, 48/F, presented in August 2002 with c/o fever on/off since 4 months, low grade, no chills/rigors. Since 2 weeks she had an associated generalized swelling all over the body and joint pains involving all the joints, no stiffness/no obvious deformity. On admission and evaluation she was found to have pancytopaenia , serositis and albuminuria . Workup revealed a positive ANA and dsDNA . She was advised a renal biopsy but she refused. In view of clinical features and lab investigations she was diagnosed as a case of connective tissue disease – SLE and started on methylprednisolone 1mg/kg/day, supportive therapy and discharged after stabilisation . She was advised regular follow-up.

CASE SNIPPET: 

CASE SNIPPET 5 months later (January 2003), she presented with puffiness of the face and decreased urine output. RFT revealed oliguria (300ml/24h) with nephrotic range proteinuria (4000mg/d) and elevated BU/SC (157/3.5mg/dl). Renal biopsy was done after obtaining an informed consent of the patient and it was consistent with WHO Stage 4 Lupus Nephritis. She was started on pulse therapy with Cyclophosphamide , uptitrated on methylprednisolone and managed with supportive care.

CASE SNIPPET: 

CASE SNIPPET She started showing signs of clinical remission within one week. (Urine output improved to 1060ml/d, 24h urine protein – 200mg/d, BU/SC – 55/1.5mg/dl) 2 weeks after starting therapy, her RFTs stabilised to normal range and she was discharged. Her pulse therapy was continued and cyclophosphamide was gradually tapered off. Steroid was tapered to minimum effective dose. She is currently on Tab.Methylprednisolone 5mg/day. 10 years since initial diagnosis, she is asymptomatic, CBP and RFTs are stable and she has a healthy QOL.

Case 3: 

December 2011 Case 3

CASE SNIPPET: 

CASE SNIPPET Mrs. D, 25/F, was brought to the ER with c/o weakness of all four limbs since 10 days. She had recently been diagnosed with SLE at an outside centre. On admission, her power was 0/5 in both LL and 2/5 in both UL with a sensory level at C5 spinal level. Her Serum potassium was 1.6meq/l. She was tachypnoeic / dyspnoeic and showed signs of respiratory muscle weakness. A provisional diagnosis of SLE associated Transverse myelitis (AIDP) vs. Hypokalemic paralysis was made. She was started on Injectable high dose steroid for the AIDP and Inj. Potassium chloride 60meq/500ml NS to correct the potassium deficit.

CASE SNIPPET: 

CASE SNIPPET 2 days after initiating therapy, her symptoms improved. Power in UL was 4+/5 and in LL was 4/5. Her sensory deficit disappeared. MRI spine was done which was normal. Patient made a complete recovery and was discharged after 1 week. She is currently on follow up for identifying the cause of hypokalemia and evaluating the D/D of AIDP sec to SLE vasculitis vs. Hypokalemic periodic paralysis.

Case 4: 

January 2012 Case 4

Chief complaints: 

Chief complaints Mrs Vijaya , 31 yrs female, a H ouse wife, Presented on 12/12/2011 with C/O Fever : 2 months Malar rash : 45 days Headache : 45 days

History of present illness: 

History of present illness Patient was apparently asymptomatic 2 months back when she developed fever associated with generalised body pains, rash on palms,back and arms, joint pains, anorexia, sleeplessness and headache. Patient presented to a local hospital, treated symptomatically, fever was relieved on medication and was discharged. 10-15 days later she developed malar rash,and persistent fever. Then, she presented to Esra hospital with fever and malar rash.

History of present illness: 

History of present illness Fever was intermittent, low grade, on and off, no chills/rigors, associated joint pains mostly involving large joints[non migratory and non fleeting] From day1 of fever, she developed macular erythema on palms, upperback and extensor aspect of arms and 10-15 days later she developed malar rash,which is slightly raised erythematous rash on cheek and nose, precipitated by sun exposure and non pruritic. No history S/O purpura , no orogenital eruptions or scaly lesions on anyother part of the body.

History of present illness: 

History of present illness No past H/O exposure to C/O TB,mite bite,tick bite,or exposure to rats or cats No history S/O malaria,chikungunya,dengue,typhoid No history of visual disturbances,altered sensorium & no complaints S/O motor or sensory impairment No H/O cough or dyspnoea No history of drug allergy No H/O similar complaints in the past

Personal/family history : 

Personal/family history Mixed diet,decreased appetite,decreased sleep Bowel and bladder habits regular Menstrual history-menarche at 13,cycles regular,4/30,no dysmenorrhea,no clots No similar complaints in the family

General examination: 

General examination Patient is conscious, coherent, cooperative, comfortably sitting on bed, well oriented to time, place & person Weight - 56kgs, Height - 158 cms Pallor +,no icterus no cyanosis,no clubbing,no lymphadenopathy,no pedal edema Normal hair Malar rash on face ,macular erythema on palms and back,non-discoid,non blanchable No orogenital ulcers EYES appear normal O/E URT normal,no congestion or secretions noted

Physical examination: 

Physical examination Vitals ,BP 110/80, PR 80b/m, regular, normal volume,character , all peripheral pulses felt. Temp 100F , RR 16/min RS-BAE+,NVBS,no ADV sounds CVS-S1,S2 heard, no murmurs P/A-soft ,no tenderness,no organomegaly,BS+ CNS-NFND,Plantars-flexor Muskuloskeletal system examination-no swelling or redness or tenderness over large or small joints,no limitation of movements at joints,no pain,no stiffness

Provisional diagnosis: 

Provisional diagnosis P yrexia for evaluation ? Connective tissue disorder ? Granulomatous disease ? Enteric fever ? Malignancy

Investigations: 

Investigations CBP - HGB:10.7gm%,WBC-1,700/cu.mm, plt - 89,000,N69,M5,L10,E2 PS - Normocytic normochromic,leucopenia,lymphopenia CUE - Pus cells-0-1,no rbc,no cystals,no casts LFT - Sr bilirubin-1.08,SGOT-134U/ L,Sr albumin- 4.3gm/dl, ALP-WNL RFT -NORMAL Sr electrolytes - Sr Na-146/k-3.9/Cl-100 Chest xray - Normal ESR -26/55mm

Investigations: 

Investigations VDRL- NR Widal test- Negative D engue NS1 Ag &IgM, IgG -Negative HBs Ag/Anti HAV/Anti HCV /Anti HEV- Negative HIV - NR USG ABD/Pelvis - Normal ANA - POSITIVE(2.975) Anti ds DNA Antibodies-positive(233IU/ml)

CRITERIA FOR SLE: 

CRITERIA FOR SLE 1. MALAR RASH 2. Discoid Rash 3. PHOTOSENSITIVITY 4. Oral ulcers 5. Arthritis 6. Renal disorder 7. HEMATOLOGICAL DISORDER 8. Serositis 9. Neurological disorder 10. IMMUNOLOGICAL (Anti- Sm , and/or anti- phospholipid , Anti dsDNA ) 11. ANTINUCLEAR ANTIBODIES-ANA POSITIVE

Diagnosis: 

Diagnosis SYSTEMIC LUPUS ERYTHEMATOSIS

Management: 

Management Supportive therapy Inj.Solumedrol 1g iv OD At discharge, Tab.Azathioprine , added at 2mg/kg/day, along with oral prednisolone , 40mg bid At follow up, patient cell counts improved, rash faded comparatively, but complained of alopecia,GI symptoms

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