Case discussion

Dr W.A.P.S.R.WeerarathnaRegistrar in Medicine

THJ

.

• 72 yrs old lady from Jaffna presented with a H/O intermittent fever for 3/12,multiple joint pains with difficulty in walking for last 1/12.

• Fever-mild to moderate,continuous fever with episodes of defeverance of few days+, no associated rashes with febrile episodes, no h/o sorethroat

• She has a noticable LOW of about 10kg during the coures of her illness.she claims that she has moderate LOA.

• Initially she developed pain in left KJ & over several weeks she experienced pain over right KJ,pelvic & shoulder girdles then she had difficulty in walking.

• She experienced a back pain & involment of small joints of hands including left WJ where her ADL were affected.

• No H/O morning stiffness,

• No H/O headache-genaralized or localized,nosugestive H/O claudication including arms,legs or jaw.

• Though she had moderate fatigue there is no H/O musle aches,soreness or stiffness.

• She denies a H/O drenching night sweats,bonepains or pathalogical fractures or lower limb weakness.

• There is no sugestive H/O any haemorragicdiastheses including easy bruising,

• Fever was not associated with concomittentbody rashes involving the trunk or extremities,no H/O photosensitivity, alopecia or oral ulcers.

• No H/O chronic cough ,haemoptysis,pasthistroy or contact histroy of PTB.

• She denies a H/O alterd bowel habbitsincluding abdominal pain,bleedingPR, tenesmus ,passage of mucus or chronic dysentry.

• Fever was not associated with gen.bodypruritus,passage of dark urine or pale stools.

• UOP had been normal with no H/O haematuria,pyuria or incontinance.

• No H/O PV bleeding or passage of any offensive PV discharges.

• She had not been to any malarial endemic area in the recent past.

• No contact H/O domestic pets,birds or cattle & she was not engaged In farming.

• She usually doesn’t consume food from outside & she is a vegetarian.

• With this Hx she got admitted to CNTH Ragama where she was investigated extensively with laboratory investigations & she was subjected to several invasive procedures .

• According to her knowledge most of the investigations were found to be normal.

• Despite being subjected to a battery of various investigations and continous theraphy along with blood transfusions she remained symptomatic.

• PMH- no H/O DM,HTN,IHD,BA,anyrhumatological diseases

• PSH-underwent B/L LRT 20 yesrs back

• DH-not been on any regular medications

• Allergy HX-no food,drug allergies

• FH-no significant illnesses run among family members

• SH-mother of 10 children,lives with her daughter &has a good family support,due to her illness she is unable to attend her ADL including toileting & taking meals with her own.

• EXAMINATION-• She is wasted,BMI-20.1Kg/m moderate

pallor+,not icteric, febrile to touh,• No clubbing ,no body rashes involving the

peripheris or over the trunk including any haemorrhagic manefestations or eschar marks.

• No peripheral stigmata of IE.• ENT-normal• No oral ulcers,significant alopecia or no evidence

of photosensitivity• B/L temporal arterial pulse palpable with no scalp

tenderness.• No bone tenderness,deformities or evidence of

pathological fractures

• Tendeness was elicited over B/L shoulder & pelvic girdles,KJ & left WJ

• B/L KJ effusions were detected with positive patellar tap,

• No dactylitis or small joint tenderness in hands,no rheumatoid nodules

• Single group of firm L/S posterior cervical LN were palpable, B/L axillary LN were palpable.

• Mild hirsutism detected

• Fundoscopy- normal despite R/S hard exudates near the optic disc-9 o’clock position.

• Examination of the breast & thyroid gland were normal

• AS- mild hepatomegaly ,2 fingers breath below the costal margin,firm,spleen not palpable & kidneys were not ballotable,noascites detected.

• CVS- BP 140/80 mmhg, PR 96/min, regular, Grade 2 PSM + best heard over the apex

• RS-unremarkable

• CNS-gait difficult to elicit, tone & power normal,reflexes not exagerated,no focal neurological deficites.

summary

• 72 years old lady presented with a H/O continous fever with polyarthralgia & girdle pains with impaired ADL, marked LOA,LOW for 3/12.O/E moderate pallor,mild hirsutism, L/S posterior cervical LN & B/L axillary LN were enlarged.AS-mild,firm hepatomegaly no other organomegaly. L/S WJ arthritis with B/L KJ effusions with no rheumatoid nodules or skin manefestations.fundus-R/S hard exudates+ with no background Hx of DM or HTN.she has a Gd 2 MR without periphrral stigmata of IE. she remaines symptomatic despite extensinsive Ix & therapeutic interventions.

investigations

• FBC-DATE 10/28 11/25 01/09 01/23 01/28 01/31 02/10

Hb 6.9 7.5 7.4 6.8 6.9 8.4 7.6

RBC 3.76 2.72 3.09 7.81 2.78 3.27 3.16

PCV 22.3 22.2 24.1 21.1 21.8 26.0 23.7

MCV 80.6 82.07 78.0 75.2 78.4 79.5 75.0

MCH 26.55 27.2 23.9 24.1 24.8 26.0 24.1

MCHC 32.9 32.9 30.7 32.1 31.7 32.7 32,1

PLT 523000

450000

634000

605000

538000

539000

179000

WBC 15800 14300 16300 18300 12300 24100 10600

N 87 86 87 86 76.4 87.3 80.4

L 10 13 12 13 1.2 5.3 8.2

E 2 1 1 2 0.3 1.9 1.6

• BP-

• RBC-normochromic normoctic with moderate rouleaux formation.

• WBC-normal total count with a reactivemonocytosis.some neutrophiles show hypolobulation.

• PLT-mild thrombocytosis with clumps.

• BMA-• Site-R posterior superior iliac spine• Bone consistancy-osteoporosis• Cellularity-normocllular marrow fragments & cell traits• Erythropoisis-mildly hypocellular with normoblastic

maturation

• Granulopoisis-mildly hypercellular with occational giant metamyelocytes.blasts< 3

• Megakaryopoisis-NL in no.&morphology• Plasma cells-3%of nucleated marrow cells are normal

plasma cells• Lymphocytes-3%of nucleated marrow cells are

normallymphocytes

• Histeocytes-histeocytic activity is increased

• ESR-

DATE 15/10 11/02 12/03 01/12 28/01

LEVEL 104 90 154 116 120

• CRP-

DATE 10/24 01/23

LEVEL 49 148 108 30 88.2 114

• LFTs-DATE 10/22 12/05 1/29 N/R

T.PRO 6.46 6.87 6.6

S.ALB 2.95 2.80 3.2

S.GLOB 3.56 4.07 3.4

T.BILI 0.6

ALP 193 204 346 (38-126)

AST 20 28.93 41 (3-42)

ALT 24.59 39.17 19 (14-50)

G-GT 89 157 (11-50)

A/G 0.94

• CXR-

• 2D ECHO CARDIOGRAME/ TOE• EDD-3.8

• ESD-2.7

• NO RWMA,EF=60%,AMVL-myxomatous

• Mild MVP,trivial MR,Novegetations seen,AVnormal,No AS/AR

• No significant PHT

• Nopedunculated masses seen.

• TOE-NO evidence of vavular destruction/IE seen.

• USS ABDOMEN-• LIVER-AP diameter=10.1cm

• Gb/cbd=NORMAL

• SPLEEN/PANCREAS=NORMAL

• KIDNEYS=R/S-9.5 ,L/S-9.5 CM

• BLADDER=partially filled

• PARAAORTIC LN=NORMAL

• INTRA ABDOMINAL MASSES-NILL

• CONCLUSION-NORMAL STUDY

• S.FERRITIN-NR=20-400(>50 years)

DATE 12/07 1/29

LEVEL 4427 5994

• S.LDH-NR-(230-460)

DATE 11/26

LEVEL 444

• S.CPK-NR-(26-140 U/L) Females

• 55.0 U/L (normal)

• RF- NEGATIVE (<8 IU/ML)

DATE 10/18 12/23

LEVEL <8 <8

• ANTI CCP Ab-negative=<25/positive=>25

• <25 units/ml

X-RAY B/L HANDS-AP

• SPEP-prominent alpha-2 band,no abnormal monoclonal bands,?acute infection

T.PRO 70.0 (60-85)

ALPHA-1 GLOB. 3 (1-4)

ALPHA-2 15 (5-11)

BETA GLOB. 7 (6-12)

GAMMA GLOB. 10 (5-15)

ALBUMIN 35 (30-50)

A/G 1 (1-2)

• ANF- NEGATIVE

• BF MP- NEGATIVE

CT-ABDOMEN-

MILD HEPATOMEGALY,REGULAR OUTLINE,NO FOCAL LEASIONS.SLEEN,PANCREAS,GB,BOTH KIDNES-NORMAL.

FEW PROMINENT PARAAORTIC LN-COELIAC/SUP.MESCENTRIC/RENAL GROUPS.

NO BOWEL MASSES,NO ASCITES,NO BONE LEASIONS.CALCIFIED UTERINE

FIBROID+

SUGEST-FOLLOWUP SCAN.

• REPEAT CT SCAN-ABD-contrast-

• No enlarged para aortic,aoroto garval nodes,

• Liver,spleen,supra renal,kidneys are NORMAL

• NO evidence of bowel obstruction

• NO ascires,NO pleural effusions

• Bones NORMAL.

• IMPRESSION-NO SIGNIFICANT ABNOMALITIES SEEN.

• AXILLARY LN BIOPSY-

• Microscopy-vague follicular archtecturedisplaying a polymorphus population of lymphoid celles including scattered polymorphs.

• There is no evidence of primary haematolymphoid malignancy or metastatic disease.

• AXILLARY LN Bx-REACTIVE LYMPH NODES.

• SPUTUM FOR AFF *3-NEGATIVE

• TB-PCR-NEGATIVE

• TB CULTURE BONE MARROW-NEGATIVE (Lowenstein Jenson medium)

• MANTOUX TEST- NEGATIVE

• LIPID PROFILE-

TC 163

HDL-C 33

TG 94

LDL-C 111.2

VLDL-C 18.8

TC/HDL-C ratio 4.94

• UFR-• App-clear

• PRO-Nill

• SUGAR-Nill

• PC-Occational

• RBC-Occ

• CAST-Nill

• EC-+

• Bacteria-Nill

• UC/ABST- NEGATIVE (2 times)

• RETRO VIRAL SCREEN -NEGATIVE

• BU-

DATE 10/22 11/12 12/01 01/25

LEVEL 22.23 34 46 33

• SE-

DATE 10/23 10/31 11/16 12/12 O1/20

S.Na+ 134 129 134 148 135

S.K+ 4.6 4.1 3.5 3.7 4.1

• S CREATININE-

DATE 10/22 11/28 12/11 01/21

LEVEL 0.67 0.84 O.9 0.8

• CLOTTING PROFILE-

• PT/INR-1.3-1.2

• APTT-NORMAL

• BT/CT=NORMAL

• X RAR L/S KT-AP view

• BLOOD CULTURE/ABST-

NEGATIVE (2 TIMES)

• OTHER INVESTIGATIONS-

DATE

S.Calcium 11.2 9.5

S.Posphate 4.1 5.3

S.Mg 2.4 2.3

• BRUCELLA Ab levels-

• QHT-

• Patient was started on a trial of steroides(Prednesolon 60mg daily & since she was responding the fever seems to be setteling,she was discharged.

• She will be reviewed in the clinic in 2 weeks time with repeat S.Ferritin,CRP,FBC.

• Dx ? Adult onset Still’s disease