JUVENILE IDIOPATHIC ARTHRITIS IN CHILDREN

JIA IN CHILDREN ESP SHOWING A JDM CASE

1. Moderator- Dr Ritu Rakholia Chairperson – Dr Anil Agarwal Panelist Dr Amit Suyal Dr Ravi Sahota Dr Aditi Jaiman Panel discussion on A case of Juvenile dermatomyositis

2. Case vignette A 6-year-old girl of Indian origin presented to the paediatric outpatient department with a history of erythematous rash over face and trunk associated with swelling not associated with photosensitivity since past 6 months

3. There was also h/o gradually progressive pain and weakness, predominantly affecting the proximal muscles of both upper and lower limbs, for the past four months. Since the past1 month she was unable to sit and for 2-3 days unable to lift her head and swallow ,when they decided to come to STM

4. She also developed ulcerative lesions over chest, back and extremities.

5. Her past Medical and Family History were insignificant. Skin examination revealed swelling and erythematous rashes around eyes, cheeks involving nasolabial folds and earlobes. Rashes and ulcerative lesions were seen over upper trunk shouders and elbows. Hyperpigmented lesions and roughening and cracking of skin was seen on palm over distal metacarpals, proximal and distal interphalangeal joints (mechanics hand). Muscle power in proximal muscles of both upper and lower limbs was 3/5 while distal power was retained. Her neck flexors also showed grade3/5 power deficit. Chest expansion and gag was weak while the rest of the respiratory examination and neuroligical exmiantion was WNL

6. ROUND 1 Ques 1.What is the most likely diagnosis for this condition? Ques 2.How will you investigate this child? Ques 3. What are the differential diagnosis for this condition ?

7. Ans 1 Juvenile dermatomyositis (JDM) is the most common inflammatory myositis in children, distinguished by proximal muscle weakness and a characteristic rash .( peri orbital heliotrope rash (does not show naso labial sparing like in SLE) macular erythematous rash on anterior chest shoulder and upper back- SHAWL SIGN ) , hyper pigmented lesions on hand (mechanics hand) Inflammatory cell infiltrates result in vascular inflammation, the underlying pathology in this disorder.Etiology is multifactorial i.e. genetic predisposition and environmental trigger. The incidence of JDM is approximately 3 cases/1 million children/year. Peak age of onset is 4-10 yr.

8. Ans 2 Investigations Hb-10g/dl, TLC-7800/mm3, DLC-P30L68E03M01, Platelet Count-1.64 lakh /mm3 ESR-30mm CRP-Negative Triglyceride-531 mg/dl LDH- 601 ALT- 91 AST- 430 CPK- 347 U/L Normal (80 U/L)

9. Immunofluorescence ANA -1:80 Speckled Titre Positive Anti DNAase Antibody - Negative Anti Smith Antibody- Positive Anti Jo 1 antibody -Positive MRI Thigh - Diffuse T2W hyperintense signals seen in muscles of all compartment in both legs giving enhancement on post contrast gadolinium scan, likely possibility of inflammatory changes, like dermatomyositis , less likely possibility of polymyositis .

10. Ans 3 D/D SLE Juvenile dermato myositis On the basis of history and clinical examination patient was suspected of having some Rheumatological Disorder most likely Juvenile Dermatomyositis . Diagnosis of dermatomyositis requires the presence of characteristic rash as well as at least 3 signs of muscle inflammation and weakness. The case presented above fulfilled all the components of the diagnostic criteria for JDM. Myositis Muscular dystrophies Congenital myopathies ,

11. Bohan and Peter criteria(revised)for diagnosis of juvenile dermatomyositis Characteristic rash of juvenile dermatomyositis Symmetrical weakness , usually progressive of the limb-girdle muscles Elevation of serum levels of muscle –associated enzymes ( creatinine kinas- LDH, SGOT, Aldolase ) MRI evidence of myopathy.However the original Bohan and Peter criteria was based on positive muscle biopsy and EMG MUSCLE BIOPSY-Necrosis of type I and II fibres , phagocytosis , degenrearion and regeneration of myofibres with variation ,with mononuclear cell infilteration EMG- triad of myopathy -short small amplitude polyphasic motor unit potential , fibrillation potetntial even at rest and bizarre high frequency repetitive discharge.

12. Definite JDM—rash+ 3 other Probable JDM-rash+2 other Possible JDM-Rash +1 other

13. ROUND 2 QUES 1- How will you manage this child? Ques 2- What are the complications expected? Ques 3. How will you monitor this child ?What is the prognosis of this condition?

14. Answer 1 Supportive management- poor gag reflex due to pharangeal muscle weakness- she was given Naso gastric feeding, frequent suction, Physiotherapy to prevent contractures, Emollients and sun protective agents as well as topical antibiotics for ulcers

15. SPECIFIC- Patient was given Immunomodulator drugs. Patient general condition improved gradually. Steroid- she was referred to AIIMS Delhi initially received pulse methyl prednisolone (30mg/kg for 5 doses) later tapered and shifted to oral steroids Steroid sparing agents- Methotrexate (10-15mg/M2 weekly) was started weekly with folic acid and she was given pulse cyclosporin due to swallowing dysfunction and anticipated respiratory involvement. IVIG has also been used in refractory / rapidly progressive disease

16. Answer 2 The mortality rate in JDM has decreased since the advent of corticosteroids, from 33% to currently approximately 1%. Swallowing dysfunction- weakness of palatal and pharyngeal muscles Children with acute and severe weakness are at risk for aspiration pneumonia and respiratory failure and occasionally require nasogastric feeding and mechanical ventilation until weakness improves GIT hemorrhage due to vasculopathy

17. Answer 3 Monitoring –Muscle enzymes(CK normalises first, LDH AST for monitoring),Auto anto bodies Anti Jo1 –severe disease, MRI muscle using spectroscopy using P3 , Nail bed capilloroscopy , inflammatory markers, Drug toxicity

18. Little is known about the long-term consequences of persistent vascular inflammation.Morbidity - Calcinosis – dystrophic calcification over the elbow, knee, digits. Serious morbidity due to ulceration of the deposit, contractures, and pain due to nerve entrapment(poorly controlled disease) Lipodystrophy - late complication due to loss of sub cutaneous fat and visceral fat. It is associated with metabolic complication of hyperglyceamia and derangement in serum lipids. Mortality- sepsis, myocarditis , respiratory insufficiency, pneumonitis , acute gastrointestinal ulceration and bleeding Mono cylclic , Poly cylclic , Chronic continous

19. ROUND 3 Ques 1. Are there any dietary restrictions for my child ? Does the disease affects the growth and development of my child?- Dr Aditi Ques 2. Can I continue the vaccination schedule of my child with Juvenile deramatomyositis ? Dr Amit Suyal Ques 3. Would it affect her schooling and she is quite stressed about his participation in sport activities and ability to live normal life! Does this affect life expectancy? Dr Ravi

20. To summarise ……follow up Rare rheumatological disorder of childhood. Early Diagnosis and management with immunomodulator therapy is important to prevent debilitating consequences and long term complications of the disorder. This child is in inactive disease on medications since 12 months and is attending school.

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