STILLS DISEASE CASE PRESENTATION

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Khushbu on Jul 24, 2012 Says :

very informative and useful case presentation on Stills disease.

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Slide 1 :

STILL’S DISEASE WITH MACROPHAGE ACTIVATION SYNDROME DR VIKAS

Slide 2 :

HISTORY A 4 yr 3 month old male child, Shreyash, admitted to ABMH on 28/10/11 with C/O High grade fever , on & off, not responding to medications with Tmax of 107Of since 20 days Rash over face and legs during fever and subside as fever decreased.

Slide 3 :

HISTORY… Initially patient was treated with antibiotics and antipyretics at ‘Rachi trust hospital, Rachi’ from 15/10/11 to 23/10/11 as enteric fever but he was not responding. Past history, family history, prev. medication history was not contributory. No history of exposure to Koch’s patient.

Slide 4 :

Clinical examination G/E Child was conscious, active but febrile with temp. of 106of HR: 120/min, RR: 24/min, pallor +nt, inguinal Lymph node +nt. No bony tenderness, no edema, no patechial rash or other rash. S/E Liver : 4cm, nontender, soft to firm, smooth surface, well define soft margins. Liver span: 13cm Spleen : 1cm, nontender, firm. Rest normal.

Slide 5 :

On the day of admission From outside (09/10/11) Optimal, widal test, blood culture were negative. Plan of care To rule out infective focus in the body Start with antibiotic (tazact and netilmycin) Rule out other non infective causes of high grade fever.

Slide 6 :

Investigations On admission (28/10/11): BSL-122 CBC: 8.9/20,000/3,61,000 SGPT- 44 Optimal/dengue/HIV/ -neg Sr Creatinine-0.4 urine routine – NAD CXR: WNL USG Abdo: s/o Hsmegaly CT Brain: NAD Blood culture sent.

Slide 7 :

Investigations & management Even after antibiotics & antipyretics, fever spikes persist. 3-4 spikes/day. (max.106of ). On 30/10/11 Doxycyclin and ciprofloxacin added Work up for connetive tissue disorder and 2DECHO was planed. Bone marrow aspiration/biopsy and culture also kept in mind.

Slide 8 :

Investigations & management… LDH-2526 Uric acid-1.6 Sr ferritin- >1200 ANA/ds-DNA/APLA- Negative 2DECHO: normal. CSF examination : WNL As all investigations were not conclusive and pallor was increasing with no evidence of bleeding, bone marrow aspiration/ biopsy and culture was done on 02/11/11.

Slide 9 :

BM aspiration/biopsy BM aspiration s/o hypercellular marrow with triliniage hemopoiesis with myeloid hyperplasia. BM biopsy showed clots with few bony trabeculae, normocellular marrow with triliniage hemopoiesis. Megakaryocytes were adequate. The blood culture showed growth of Roseomonas gilardii, Sensitive to Tazact, Ciprofloxacin, Doxy, Netilmycin, Taxim, Monocef

Slide 10 :

Clinical course (04/11/11) Over the time fever was not responding & child had daily spike of fever but intensity was decreased. Evanescent rash still persist Cervical lymphadenopathy was noted on 04/11/11 HSmegaly persist Bone marrow culture showed no growth Rheumatologist opinion sought, as features pointing towards CTD.

Slide 11 :

Diagnosis of Still’s disease As child is having High grade fever Evanescent rashes Cervical Lymphadenopathy Mild HSmegaly High WBC count High Ferritin Possibility of macrophage activation syndrome was kept in mind as LDH level was very high.

Slide 12 :

On 06/11/11 Scrotal edema seen (USG s/o hydrocele with scrotal wall edema) Oral methyl prednisolone started. Brucella and Leishmania antibody screen sent; which was negative. BM culture no growth Antibiotics and antipyretics continued.

Slide 13 :

08/11/11 Possibility of “MACROPHAGE ACTIVATION SYNDROME” kept as, Repeated hemogram showed dropping WBC count and Hb.(6.5/3400/3.02) LDH and ferritin level is high BM examination s/o hemophagocytosis So, I.V. methyl prednisolone pulse therapy started and cyclosporin kept in plan if no response.

Slide 14 :

09/11/11 Management of MAS Hematologist opinion sought. Plan for IvIg for 3 days. Oral Cyclosporin started Plan to monitor hemogram daily. Investigations: Triglycerides: 229 S. ferritin: 30,000 PT: 11.6/11.9/1.02 aPTT: 28.3/29.8 Fibrinogen: 225.6

Slide 15 :

Daily trends of hemogram

Slide 16 :

Daily trends of hemogram

Slide 17 :

14/11/11 Child is afebrile since 10/11/11. c/o leg pain Scrotal edema decreased No s/o synovitis, arthritis, DVT Plan : Continue methyl prednisolone till ANC > 1000 – 1500 and Shift out to ward (isolation) when child is stable and remain afebrile.

Slide 18 :

Discharge Child discharged on 18/11/11, as WBC count increasing, child is afebrile, accepting orally well, ferritin drops to 4500

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