CASE PRESENTATION unknown serositis and multiple other things

Unsolved but Curious case of Benjamin button

1. CASE DISCUSSION DR MALIKA THARANI PG TRAINEE MEDICAL 1

2. HISTORY 45 years old male ,Married k/c of CIDP? , works in garment factory, resident of orangi town, shifted from neurology ward presented with c/o Pedal edema for 1 month Facial swelling for 4 days Abdominal distension for 3 days Rash on both legs for 2 days

3. HOPC A/c to my patient he was in usual state of health till one month back when he noticed swelling of his both feet which gradually increase to involve the shins ,it was not associated with fever, pain or redness. for 4 days he noticed swelling of the face and abdominal swelling which has increased progressively and associated with generalized abdominal pain that is mild to moderate in intensity ,dull aching in nature, not radiating anywhere, with no aggravating or relieving factors, not associated with nausea or vomiting. He developed rash on both legs for 2 days which are small, reddish ,painless on both shins, not associated with itching. no such rashes on any other site and no bleeding from any other site.

4. HOPC There is no current or previous hx of melena, hematemesis, altered sensorium. No hx of chest pain, shortness of breath, orthopnea ,PND.NO hx of hematuria, frothy urine, burning micturition, urinary frequency, urgency or Dec urine output.

5. SYSTEMIC INQUIRY General: generalized weakness and Fatigue is present Abd: unremarkable. CVS: unremarkable Resp: unremarkable CNS: weakness and numbness of both legs is present with no tingling or paresthesia, no hx of vertigo, dizziness, headache, blackouts, seizures or diplopia GU: unremarkable MSK: no hx of joint pain, oral ulcers, photosensitivity, dry eyes, dry mouth, dysphagia, Raynaud's phenomenon or psychosis Endocrine: no hx of heat or cold intolerance, palpitations, tremors or dryness of skin.

6. PERSONAL HX No hx of weigh loss Appetite is normal Sleep is normal Bladders habits are normal Bowel habits are normal He is Gutka addict

7. PAST HX Pt has hx of b/l lower limb weakness 2.5 years back which was gradual progressive and ascending .he become unable to walk and even do his daily activities, he left his job due to this weakness, no urinary or fecal incontinence at that time. it extended to involved the upper limbs within the period of 1 year and at that time MRI cervical and dorsal spine (screening) was done which showed disc degeneration at C5 C6 C7 D7 D8 levels, EMG-NCVs were done which showed CHRONIC SENSORY MOTOR AXONAL POLYNEUROPATHY, 5 sessions of plasmapheresis were done at that time and he was started on Imuran and steroids( which he took for 5 months and then left) after this treatment his weakness improved, he become able to carry out his daily activities but still walk with support. This weakness again increased for 2 months and then the patient presented in neurology

8. PAST HX Pt has hx hemorrhoids for that he took hakeemi medications for 1 month. There is no past surgical or blood transfusion history.

9. FAMILY HX NO hx of tuberculosis in family No chronic illness in family

10. NUTRIONAL HX Good intake of green vegetables and chicken Takes red meat once a week

11. SOCIOECONOMIC HX Lives in well ventilated house No pets at home

12. EXAMINATION

13. GENERAL PHYSICAL EXAMINATION Middle aged male with normal built and average height lying on bed comfortably, he is well oriented to time place and person with vitals of BP 130/70mm oh hg Pulse 87beats/min regular and good volume Temp A/F RR 20 breaths/min RBS 100mg/dl So2 96% on RA

14. GENERAL PHYSICAL EXAMINATION SUBVITALS Anemia present Jaundice absent Clubbing absent Koilonychias/leukonychia absent Dehydration absent Edema present ( B/l up to shins and periorbital puffiness ) Thyroid Not palpable Lymph nodes Not palpable JVP Not raised

15. GENERAL PHYSICAL EXAMINATION SUBVITALS Oral ulcers Not present Palmar erythema Not present Spider nevi Not present Flapping Tremors Not present Petechial rash on distal half of both shins ( small 2-3mm in diameter non blanching)

16. ABDOMINAL EXAMINATION INSPECTION: Abdomen was distended, moving with respiration. Umbilicus was central with everted margins. No visible pulsations, stria, scars or prominent veins. PALPATION: Abdomen was tense , non tender, no visceromegaly appreciated. PERCUSSION: fluid thrill was positive. ASCULTATION: Gut sounds audible. No renal hepatic or aortic bruit present

17. CENTRAL NERVOUS SYSTEM EXAMINATION GCS 15/15 SOMI absent Pupils BERL Higher Mental functions intact Speech normal Cerebellar function and Cranial nerves intact Gait – unable to walk/walk with support Fundus normal

18. CENTRAL NERVOUS SYSTEM EXAMINATION SENSORY SYSTEM Proprioception was impaired in lower limbs Vibration was impaired in lower limbs Pinprick was impaired in lower limbs ( glove and stocking type of sensory loss)

19. CENTRAL NERVOUS SYSTEM EXAMINATION MOTOR SYSTEM UPPER LIMB LOWER LIMB RIGHT LEFT RIGHT LEFT BULK N N BULK N N TONE N N TONE DEC DEC POWER +4/5 +4/5 POWER (proximal) +4/5 +4/5 DISTAL +3/5 +3/5 ANKLE 1/5 1/5 REFLEXES A A REFLEXES A A PLANTARS MUTE MUTE

20. RESPIRATORY SYSTEM EXAMINATION INSPECTION: Normal shaped chest with abdominothoracic respiration. No prominent striae, pulsations or scar marks, moving equally with respiration. PALPATION: Trachea central. Apex beat palpated in 5th ICS medial to midclavicular line. Normal chest expansion. PERCUSSION : Resonant percussion note throughout lung fields except on the b/l mid to lower zones ( DULL on b/l mid to lower lower zones) AUSCULTATION : Coarse crepitations on b/l mid zones with increased vocal resonance. Absent breath sounds at both lower zones with decreased vocal resonance.

21. CARDIOVASCULAR EXAMINATION PERIPHERAL PULSES: Palpable and good volume. INSPECTION: No scar marks, pigmentation, pulsations or prominent vessels PALPATION: Apex beat palpated in 5th intercostal space medial to midclavicular line, normal in character. No parasternal heave or thrill present. AUSCULTATION: S1 + S2 audible. No Murmur Appreciated

22. CASE SUMMARY 45 years old male ,Married k/c of CIDP? , works in garment factory, resident of orangi town, shifted from neurology ward presented with c/o Pedal edema for 1 month, Facial swelling for 4 days, Abdominal distension for 3 days, Rash on both legs for 2 days. generalized weakness , Fatigue. Weakness and numbness of both legs. He is gutka addict. Pt has past hx of b/l lower and upper limb weakness 2.5 years back .got plasmapheresis done one year back, and took steroids and Imuran for 6 months then left, increasing weakness for 2 months. Pt has hx hemorrhoids for that he took Hakimi medications for 1 month.

23. CASE SUMMARY Pt is anemic with periorbital puffiness and bilateral pitting edema and petechie on both shins. Abdomen was distended with everted umbilicus, tense , fluid thrill was positive. On CNS exam tone and power was decreased in both lower limbs, reflexes were absent in all four limbs , planters bilaterally mute, proprioception and vibration, pinprick were impaired , glove and stocking type of sensory impairment. On chest exam pt has bilateral coarse crepitation on mid zones with increased VR consistent with consolidation and absent breath sounds with dec VR on lower zones consistent with pleural effusion.

24. DIFFERENTIAL DIAGNOSIS Disseminated tuberculosis Nephrotic syndrome Serositis sec to connective tissue disease Decompensated liver disease Hypothyroidism Congestive cardiac failure

25. CIDP may occurs with these conditions Chronic hepatitis Diabetes Infection with the bacterium Campylobacter jejune HIV/AIDS Immune system disorders due to cancer Inflammatory bowel disease Systemic lupus erythematosus Cancer of the lymph system Overactive thyroid Side effects of medicines to treat cancer or HIV paraprotenemias

26. INVESTIGATIONS

27. HB 8.3 HCT 27.9 MCV 93 WBC 4.0 N 37.5 L 50.8 E 1.3 Monocytes 10.1 B 0.3 plat 68 PERIPHEAL FILM ANISOCYTOSIS, POIKILOCYTOSIS, POLYCHROMASIA ? CAUSE PLATELETS LOW ON FILM OCCASIONAL ATYPICAL LYMPHOCYTES SEEN? VIRAL INFECTION ESR 115 CRP 64.6 Retic count 2.80%

28. NUTRIONAL PROFILE S.iron 28 mcg/dl Ferritin 108 ng/ml TIBC 233 mcg/dl T.sat 12% Vit B12 >2000

29. BUN 13 Cr 1 Na 135 k 3.2 CL 108 Ca 7.9 Mg 1.8 phos 3.6 Corrected Ca 9.4 T.bili 1.1 SGPT 13 ALK ph 142 T.pro 7.1 Albumin 2.1 globulin 5 A/G ratio 0.42 PT 14.2 APTT 34.2 INR 1.36

30. Dengue Ns1 antigen was not detected MP Mp(ICT) were not seen HbsAg and AntiHCV on ICT and CMIA are Non-reactive Hep B core antibody negative HIV Non-reactive

31. URINE DR Color yellow Appearance slightly turbid PH 5 Sp gravity 1.021 Protein 0.25g/l (+1) Glucose negative Ketones negative Red cells >20 Pus cells>20 Casts nil Multiple UDR repeated shows protein trace

32. CULTURES UCS on 24 th may shows growth of E.coli. Multiple UCS and BLOOD C/S shows NO GROWH. HbA1C 4.7

33. Urinary ACR

34. CHEST XRAY

35. CHEST XRAY

36. U/S ABDOMEN Liver is Enlarged in size 17.5cm with irregular margins and coarse echotexture.NO focal mass. Intrahepatic biliary ducts ae not dilated. Portal vein normal 0.8cm,nomal flow, normal phasicity and velocity. Thick walled gallbladder. Normal Pancreas Spleen enlarged measuring 14cm.homogenous parenchyma, no focal mass, splenic vein normal. Gross abdominopelvic ascites . Bilateral minimal pleural effusion . Repeated ultrasound after one month shows thick walled urinary blader with internal echoes suggestive of cystitis . otherwise same findings

37. ASCITIC FLUID STUDIES SAAG 0.54

38. GASTIC ASPIRATE FOR AFB AND GENE XPERT NOT DETECTED

39. THYROID PROFILE TSH 6.22 ( 0.4-4.5) fT3 2.07 (2.1-4.4) fT4 1.06 ( 0.8 – 2.7) Anti thyroid Peroxidase antibodies Negative

40. ECHO ECG :NSR

41. ANA PROFILE ANA POSITIVE Spindle pole pattern (SLE , Sjogren syndrome) 1/160 (titer) ASMA NEGATIVE AMA NEGATIVE ANTI DsDNA (igG) NEGATIVE Serum C3 and C4 within normal ranges Rheumatoid factor Negative

42. ENA PROFILE

43. EMG-NCVs Chronic sensorimotor demyelinating polyneuropathy

44. CT CHEST AND ABDOMEN WITH CONTRAST CONCLUSION Gross ascites with omental thickening and bilateral minimal non tapable pleural effusion with basal collapse consolidation likely due to infective etiology raising the possibility of tuberculosis. Ascitic tap for D/R and gene expert is advised for confirmation. Bony changes in right glenoid cavity are likely due to aggressive etiology could be osteomyelitis in suspected case of tuberculosis. Would recommend MRI for proper evaluation and characterization of the lesion.

45. ASCITIC FLUID STUDIES AFB SMEAR and GENE XPERT not detected AFB Culture is negative.(prelim report) C/S shows No growth. ADA levels : 20 ( normal upto 30) Cytology shows Proteinaceous background along with lymphocytes, histiocytes and some Reactive mesothelial cells.

46. Tumor markers CA 19-9 19.32 ( <37) CEA 1.81 (<3) AFP 1.34 (<8.2)

47. MESENTIC LYMPH NODE OMENTAL BIOPSY

48. FINAL DIANOSIS

49. TREATMENT GIVEN IN WARD Inj Lasix 40mg iv BD initially then SOS Inj Neurobion iv OD for 7 days Tab folic Acid 5mg OD Inj tanzo 4.5 gm iv TDS Inj flagyl 500mh iv TDS Inj N/S @60cc/ hr Tab HCQ 200mg BD started one week back

50. Table 18.22-1. Classification criteria for primary Sjögren syndrome according to ACR/EULAR guidelines Clinical inclusion criteria  (ocular and oral symptoms; ≥1 positive response to the following questions): 1) Have you had daily, persistent, troublesome dry eyes for >3 months? 2) Do you have a recurrent sensation of sand or gravel in your eyes? 3) Do you use tear substitutes >3 times a day? 4) Have you had a daily feeling of dry mouth for >3 months? 5) Do you frequently drink liquids to aid in swallowing dry food? Or suspicion of Sjögren syndrome based on ESSDAI a Clinical exclusion criteria : History of head and neck radiation therapy, active HCV infection (confirmed by PCR), AIDS, sarcoidosis, amyloidosis, graft-versus-host disease, IgG4-related disease Classification criteria  (histopathology, autoantibodies, ocular signs) Points Diagnosis of focal lymphocytic sialadenitis in a labial salivary gland b  with a focus score count >1 foci/4 mm 2 3 Anti-Ro/SSA antibody positive 3 Ocular staining score c  ≥5 or van Bijsterveld score d  ≥4 in ≥1 eye 1 Schirmer test ≤5 mm/5 minutes in ≥1 eye 1 Unstimulated whole saliva flow rate ≤0.1 mL/min f 1 Interpretation : Patients with a total score ≥4 points meet the criteria for primary Sjogren syndrome (sensitivity, 96%; specificity, 95%)

51. Workup for sjogren syndrome CBC ESR ANA Anti-Ro/Anti-La RF Schirmer test SPEP Staining (Rose Bengal and lissamine green staining) Salivary testing (sialometry) Protein profiling ( tear proteomics) Sialography and scintigraphy Minor salivary gland biopsy with histology

52. PH 7.512 PCo2 27.6 PO2 88.7 So4 98.4 Hco3 24.3 ABE -0.7 Na 133 k 4.4 Cl 104 A.G 5 ABGs

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