Many Faces of Systemic Lupus Erythematosus

Prof. Md. Titu Miah Professor of Medicine Dhaka 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 SLE Worldwide Vs DMCH

85 100

90 85

50

20

35

25

45

15

60

70

90

70 70

10

25

40

30

5

0

10

20

30

40

50

60

70

80

90

100

Worldwide DMCH

Number of SLE patients in DMCH 100 in 2015

In SLE clinic 60

In Nephrology 28

In Dermatology 5

In 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 RA

Musculoskeletal cont.

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

4 yr later she develop AVN

SLE Vs Overlap Syndrome O

verla

p sy

ndro

me

SLE

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. Mononeuropathy 2. Polyneuropathy 3. Cranial neuropathy 4. Acute inflammatory demyelinating polyradiculoneuropathy (GBS) 5. Plexopathy 6. Autonomic disorder 7. 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/cmm ESR: 86 mm in 1st hr ANA: Positive Anti ds DNA: Positive

Furtther investigations

Final Diagnosis

CNS LUPUS

A young normotensive nondiabetic patient presented with left sided hemiperesis

Further investigations Lipid profile: normal ANA: positive Anti ds DNA: positive Anti 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/E PlantarResponse: Extensor bilaterally

Hb: 7.92 g/dL WBC: 2.50 x10^9/L ESR: 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

days O/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 hr MT:negative Urine 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: NEGATIVE ANTI DS DNA: NEGATIVE ALL 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:35 WBC:4000/UL Platelet:79,000 Atypical cell: 10% PBF: Leucoerythroblastic blood picture with marked thrombocytopenia

SKIN cont.

Further Investigations Lymphnode biopsy: Jessners Lymphocytic infiltration

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 variety Anti 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 :Positive 24 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 Diagnosis

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 crepitation ECG: AF with first ventricular rate T3: 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: Positive Anti 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