Many Faces of Systemic Lupus Erythematosus. Prof. Md. Titu Miah Professor of Medicine Dhaka Medical College & Hospital

Transcription

1 Many Faces of Systemic Lupus Erythematosus Prof. Md. Titu Miah Professor of Medicine Dhaka Medical College & Hospital

2 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

3 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

4 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.

5 Clinical presentation among the SLE patients can be Diverse, Highly variable! Ranging from INDOLENT to FULMINANT

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

7 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

8 Classical TRIAD of SLE FEVER RASH JOINT PAIN

9 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)

10 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

11 Musculoskeletal cont. A 32yrs Lady with SLE since 1998 was on hydroxychloroquine and steroid. 4 yr later she develop AVN

12 SLE MYOSITIS SYSTEMIC SCLEROSIS Overlap syndrome SLE Systemic sclerosis Myositis SLE Vs Overlap Syndrome

13 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:

14 Neuropsychiatric Lupus Central Nervous System 1. Headache 7. Aseptic meningitis 2. Seizure disorders 8. Cognitive dysfunction 3. Cerebrovascular disease 9. Mood disorder 4. Demyelinating syndrome 10.Anxiety disorder 5. Myelopathy 11.Psychosis 6. Movement disorder 12.Acute confusional state

15 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

16 30 yr old patient complaining of severe headache for 15 days Fundoscopy

17 CT Scan of Head MRV Showing NORMAL Superior Sagital Sinus Thrombosis

18 Furtther investigations CBC: Hb% 9.1 g/dl WBC: 6580/cmm Platelet: 27,8000/cmm ESR: 86 mm in 1 st hr ANA: Positive Anti ds DNA: Positive CNS LUPUS Final Diagnosis

19 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

20 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

21 Furtther investigations Hb: 7.92 g/dl WBC: 2.50 x10^9/l ESR: 88 mm in 1 st hour ANA screening: +ve (42.5 U/mL) Anti-dsDNA: IU/ml CNS LUPUS Diagnosis

22 CNS Lupus: A D/D of Multiple Sclerosis

23 A young girl named Tanzila presented with blurring of vision for 15 days. Fundoscopy revealed:

24 Diagnosis? Lupus Retinopathy

25 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,lifethreatening pulmonary complication of SLE.

26 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

27 CBC: Hb:11.0 g/dl WBC:9000 Platelet: ESR:101 mm in 1 st hr MT:negative Urine R/M/E: Rbc: 1-2/HPF PC:2-3/HPF Albumin:Trace Pulmonary cont.

28 Further investigations Diagnosis? Diffuse Parenchymal Lung Disease Due To SLE

29 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.

30 Skin Changes in SLE Malar Rash Rash In Trunk Or Extremities Urticaria Bullae Maculopapular Lesions Ulcerations Raynauds

31 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.

32 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

33 Patient presented with ulcer in hand for one month. DIFFERENTIAL DIAGNOSIS?

34 3 month later she was diagnosed as a case DLE Skin cont.

35 Mr. X 82 yrs old was admitted with the complaints of Multiple painless nodular leison all over his body for 4 month.

36 SKIN cont. 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

37 Further Investigations Lymphnode biopsy: Jessners Lymphocytic infiltration Diagnosis?

38 SKIN cont. 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:+ Investigations S. ALT:45 U/L, S. AST:90 U/L CBC: Hb-10gm/dl WBC ³/mm ESR: 100 mm in 1st hour Dengue NS1 antigen : negative.

39 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)

40 Further. investigations: ANA: Strongly positive Speckled variety Anti Ds DNA: positive 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.

41 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

42 Gastrointestinal 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

43 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

44 Gastrointestinal Hb:12.5 g/dl ESR :105mm/1 st hr TC: Platelet : USG of W/A : 1) Hepatosplenomegaly. 2) Suggestive of acute renal parenchymal disease. 3) Bilateral mild pleural effusion. 4) Huge ascites. D- Dimer > 4.00mg/ml( ) FDP > 120 ug/ml( ) CRP 7.45mg/L Urine R/M/E RBC: Plenty Pus cell: 4-5 Protien:+++ Anti Ds DNA :Positive ANA :Positive 24 hrs UTP:29.91 g/24hrs

45 Final Diagnosis SLE with Lupus Nephritis with septicemia with wound dehiscence following appendectomy

46 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

47 Further investigations ANA: Positive Anti Phospholipid Ab : Positive Anti ds DNA: Positive Diagnosis Lupus Mesentric Vasculitis

48 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

49 Further investigations ANA: Positive Anti Phospholipid Antibody: Positive Anti ds DNA: Positive Diagnosis Budd Chiari Syndrome Resulting From Hyper Viscosity Caused By Anti Phospholipid Syndrome Secondary To SLE

50 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)

51 Differences between, Pre-eclampsia and Renal flare in a patient with SLE

52 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

53 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 After 3 month further investigations revealed: ANA: Positive Anti Ds DNA: Positive Association with other Autoimmune disease?

54 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

55 Yes! We can and We will. No matter how steep the journey is, destiny is not beyond your reach

56 THANK YOU