Many Faces of Systemic Lupus ErythematosusProf. Md. Titu MiahProfessor of MedicineDhaka Medical College & Hospital

”

“ The Very Appearance of SLE might be

DECEIVING!NO TWO LUPUS PATIENTS HAVE EXACTLY THE SAME MANIFESTATIONS AND ONE PERSON DOES NOT USUALLY HAVE ALL THE SYMPTOMS.

. So a VERY HIGH INDEX OF CLINICAL SUSPICION should be there during

diagnosis of SLE

SLE might present as“Catch Me if U Can!”

Movie: Catch Me If u Can

Actor: Leonardo de Caprio

Main Role: Con Artist

Substitue Role: Lawyer,Doctor , Pilot, Detective

Epidemiology

In Asia: Prevalence rates 30—50/100,000 population. Incidence rates 0.9/100,000 to 3.1% per annum. In U.S.A: Incidence: 5 cases per 100,000 population. Race, sex and age-related demographics:

The prevalence of SLE is highest in women aged 14 to 64 years. Black women have a higher rate of SLE followed by Asian women and then White women.

.

Clinical presentation among the SLE patients can be Diverse, Highly variable !

Ranging from

INDOLENT to

FULMINANT

Prevalance of SLEWorldwide Vs DMCH

0

10

20

30

40

50

60

70

80

90

100

85

100

9085

50

20

35

25

45

15

60

70

90

70 70

10

25

40

30

5

Worldwide DMCH

Number of SLE patients in DMCH 100 in 2015In SLE clinic 60In Nephrology 28In Dermatology 5In Pediatrics 8

A prospective study was done from January 2002 to December 2006 in Mymensingh Medical College and Hospital.

Number of SLE patients : 33 Objective: To observe the clinical

profile and outcome of the patients

Classical TRIAD of SLE

FEVER

JOINT PAIN

RASH

Constitutional

Fatigue, the most common constitutional symptom associated with SLE.

Can be due to active SLE, medications, lifestyle habits, or concomitant fibromyalgia or affective disorders.

Fever may reflect active SLE, infection, and reactions to medications (drug fever)

Musculoskeletal

Joint pain is the most common clinical presentation.

In contrast to Rheumatoid Arthritis SLE arthritis may be Asymmetrical

Pain is disproportionate to swelling.

Increased Prevalence of Avascular necrosis in the patients with SLE.

SLE

Musculoskeletal cont.

A 32yrs Lady with SLE since 1998 was on hydroxychloroquine and steroid.

4 yr later she develop AVN

SLE Vs Overlap Syndrome

Overlap syndromeSLE

Systemic sclerosis

Myositis

SLE

MYOSITIS

SYSTEMIC SCLEROSIS

Central Nervous System

Patients with SLE can have several Neuropsychiatric symptoms represent a subcategory termed NPSLE

According to 10 high quality prospective studies including 2049 SLE patients the prevalence of NPSLE manifestations among them was 56%, were CNS manifestations were 90%.

 Brey RL, Holliday SL, Saklad AR, Navarrete MG, Hermosillo-Romo D, Stallworth CL. Neuropsychiatric syndromes in lupus: prevalence using standardized definitions. Neurology. 2002; 58:1214–20.

Neuropsychiatric Lupus

Central Nervous System 1. Headache 2. Seizure disorders 3. Cerebrovascular

disease 4. Demyelinating

syndrome 5. Myelopathy 6. Movement disorder

7. Aseptic meningitis 8. Cognitive dysfunction 9. Mood disorder 10.Anxiety disorder 11.Psychosis 12.Acute confusional

state

Neuropsychiatric Lupus

Peripheral nervous system:

1. Mononeuropathy2. Polyneuropathy3. Cranial neuropathy4. Acute inflammatory demyelinating polyradiculoneuropathy (GBS)5. Plexopathy6. Autonomic disorder7. Myasthenia gravis

30 yr old patient complaining of severe headache for 15 days

Fundoscopy

CT Scan of Head

Superior Sagital Sinus Thrombosis

MRV Showing

NORMAL

CBC: Hb% 9.1 g/dl WBC: 6580/cmm Platelet: 27,8000/cmmESR: 86 mm in 1st hrANA: PositiveAnti ds DNA: Positive

Furtther investigations

Final Diagnosis

CNS LUPUS

A young normotensive nondiabetic patient presented with left sided hemiperesis

Further investigationsLipid profile: normalANA: positiveAnti ds DNA: positiveAnti phospholipid Ab: positive

Patients with lupus had higher risk for all stroke subtypes

except in subarachnoid hemorrhage

• Mrs Y 23yrs old was presented with-Convulsion for 3 days.-Pain in multiple joints for4months.-Fever for 6 months She had history of

hallucination.

O/EPlantarResponse:Extensor bilaterally

Hb: 7.92 g/dLWBC: 2.50 x10^9/LESR: 88 mm in 1st hour

ANA screening: +ve (42.5 U/mL)Anti-dsDNA: 145.0 IU/ml

Furtther investigations

Diagnosis

CNS LUPUS

CNS Lupus: A D/D of Multiple Sclerosis

A young girl named Tanzila presented with blurring of vision for 15 days .

Fundoscopy revealed:

Diagnosis? Lupus Retinopathy

Pulmonary

SLE may lead to multiple pulmonary complications such as pleurisy, pleural effusion, DPLD, pneumonitis, pulmonary hypertension.

Hemoptysis may herald diffuse alveolar hemorrhage, a rare, acute,life-threatening pulmonary complication of SLE.

Pulmonary cont.

Mrs x 65 yrs old was admitted with the complaints of- Fever for one month Chronic dry cough for last

15 daysO/E: Anemia: Present Lung: fine basal

crepitation Chest Xray

Pulmonary cont.

CBC:• Hb:11.0 g/dl• WBC:9000• Platelet:335000• ESR:101 mm in 1st hrMT:negativeUrine R/M/E:• Rbc: 1-2/HPF• PC:2-3/HPF• Albumin:Trace

Diffuse Parenchymal Lung Disease Due To SLE

Diagnosis?

Further investigations

Pulmonary cont.

Mrs. X, presented with - fever for 1 month -cough for 1 month.

Clinically she had features of consolidation.She had non resolving pneumonia for 3 month. All other relevant investigations were normal apart from neutropenia and then her ANA and Anti Ds DNA revealed high titre subsequently she was diagnosed as a case of SLE.

Skin Changes in SLE

Malar Rash Rash In Trunk Or

Extremities Urticaria Bullae Maculopapular Lesions Ulcerations Raynauds

Male presented with

-Fever for 15 days-Erythomatous rash

involving chest for same duration

ANA: NEGATIVEANTI DS DNA: NEGATIVEALL OTHER INVESTIGATIONS REVEALED NORMAL.

Subsequently it was diagnosed as case of SLE on histopathology which showed liquefactive degeneration of basal layer of epidermis.

6 month later he was diagnosed as acase of DPLD with SLE

• Patient presented with ulcer in hand for one month.

DIFFERENTIAL DIAGNOSIS?

Skin cont.

3 month later she was diagnosed as a case DLE

Mr. X 82 yrs old was admitted with the complaints of Multiple painless

nodular leison all over his body for 4 month.

O/E:• Anemia : +• Lymphnode :

generalized lymphadenopathy.

• P/A/E: no organo megaly• USG of W/A: Fatty

change of liver• Urine R/M/E: Normal

Hb:11.4gm/dl ESR:35WBC:4000/ULPlatelet:79,000Atypical cell: 10%PBF: Leucoerythroblastic blood picture with marked thrombocytopenia

SKIN cont.

Further InvestigationsLymphnode biopsy: Jessners Lymphocyticinfiltration

Diagnosis?

SKIN cont.

Investigations S. ALT:45 U/L, S. AST:90 U/L CBC: Hb-10gm/dl WBC10.6×10³/mm ESR: 100 mm in 1st hour Dengue NS1 antigen : negative.

A 23years female medical student of DMC presented with• High grade continued fever for

6 days• Headache for same duration

O/E:• Temp:102 F• Bp:80/60• Pulse:114/min• Anemia:+

After treating with antibiotic no remission of fever.subsequently she develop lymphadenopathy involving ant and post cervical chain,left supra clavicle and both inguinal region.

.

FNAC of lymphnode:Focal aggregation of epitheloid cells. Features suggestive of granulomatous inflammation.

Biopsy of Lymph node:Acute necrotizing lymphadenopathy (Kikuchi’s disease)

.

DIAGNOSIS ?

• Several authors reported association between SLE and Kikuchi disease

• Kikuchi disease has been diagnosed before,during, and after diagnosis of SLE was made in same patients.

• Histological appearance of lymphnodes of both disease are similar.

• Kikuchi disease may represent a forme fruste SLE.

Further investigations:ANA: Strongly positive Speckled varietyAnti Ds DNA: positive

Gastrointestinal

Occasional abdominal pain in active SLE may be directly related to active lupus

-including peritonitis, pancreatitis, mesenteric vasculitis,and bowel infarction.

Jaundice due to autoimmune hepatobilliary disease

Miss Joba,13 Years of age, was admitted with the complaints of

1) Swelling of whole body for 3weeks.2) Rashes over whole body – same duration.3) H/0 Burst Abdomen with peritonitis followed by appendectomy 3 wks back.

Gastrointestinal

Gastrointestinal

O/E:• Anemia : ++• Odema : +++• Multiple purpuric, non palpable, non

tender rashes on the back of the body. Excoriating lesions over abdomen and both upperlimb

P/A/E:• wound dehiscence present• Ascites and Hepatospleenomegaly• Vulval Swelling

Gastrointestinal

• Hb:12.5 g/dl• ESR :105mm/1st hr• TC: 12.5×109• Platelet :70×109 Urine R/M/E

• RBC: Plenty• Pus cell: 4-5• Protien:+++Anti Ds DNA :Positive ANA :Positive24 hrs UTP:29.91 g/24hrs

D- Dimer > 4.00mg/ml(↑)FDP > 120 ug/ml(↑)CRP 7.45mg/L

USG of W/A :1) Hepatosplenomegaly.2) Suggestive of acute renal parenchymal disease.3) Bilateral mild pleural effusion.4) Huge ascites.

Gastrointestinal

Final Diagnosi

s

SLE with Lupus Nephritis with septicemia with wound dehiscence following appendectomy

A young women presented with sudden severe Abdominal Pain.

There is diffuse circumferential wall thickening with diffuse oedema involving entire small intestine resulting in double halo or target sign

Further investigations

Lupus Mesentric Vasculitis

• ANA: Positive• Anti Phospholipid Ab :

Positive• Anti ds DNA: Positive

Diagnosis

Gastrointestinal

A lady of 33 year presented with: Sudden onset abdominal distension for 7 days. Abdominal discomfort . Respiratory distress.On examination• She was mildly

icteric• Shifting dullness

+ve• Splenomegaly

USG shows hepatic vein thrombosis, with arrow pointing to the thrombus

Diagnosis

Budd Chiari Syndrome Resulting From Hyper Viscosity Caused By Anti Phospholipid Syndrome Secondary To SLE

• ANA: Positive• Anti Phospholipid

Antibody: Positive• Anti ds DNA: Positive

Further investigations

SLE during Pregnancy

Fertility When and how to time

pregnancy Obstetric issues -Pre eclampsia -Lupus nephritis -Thrombosis

• Prevalence of Pre eclampsia is 13% in SLE Vs 6-8% in normal condition.

• Risk factor include -pre-existing hypertension, -nephritis and -presence of antiphospholipid antibodies (APL)

Differences between, Pre-eclampsia and Renal flare in a

patient with SLE

SLE in CHILDREN:Malar rash, ulcers, mucocutaneous involvement, proteinuria,urinary cast, seizures, haemolytic anemia, thrombocytopenia, fever and lymphadenopathy are more commonly in childhood onset SLE

A female patient presented with -respiratory distress for one month -palpitation for same duration

O/E: Irregularly irregular pulse lungs: basal crepitationECG: AF with first ventricular rateT3: Raised ; T4: raised; CRP: Raised Diagnosed as a case

of Autoiimune thyroidits with myocarditis

Association with other Autoimmune disease?

After 3 month further investigations revealed:ANA: PositiveAnti Ds DNA: Positive

Take Home Message

Even in difficult situation to diagnose SLE, most

physicians need high index of suspicion, special intuition, obsession to finally bring a

differential diagnosis in appropiate clinical scenario in every discipline. So no matter

whatever might be the faces or presentation SLE should be

Red handed

No matter how steep the journey is, destiny is not beyond your reach…

“Yes! We can and We will

….”

THANK YOU