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MNJ22-001
A 9-year-old girl Ritu presents with high irregular fever for 3 weeks, each episode associated with erythematous rashes over the trunk and proximal extremities, pain and swelling of right knee and easy fatigability. Has leucocytosis, raised ESR, normal chest skiagram and negative mantoux test:

  1. Give most probable diagnosis.
  2. List other expected systemuc clinical findings.
  3. Give value of serology in diagnosis
  4. Outline steps in management.
  5. List prognostic factors

Answer

  1. JRA—systemuc type

NSAIDs, methotrexate, azathioprine

MNJ22-002
A child has chronic polyarthritis of 4 joints and is ANA positive:

  1. What arthritis is it hkely to be?
  2. What complication should we anhcipate?
  3. In which type of JRA is HLA B,, positive?
  4. A child with JRA presents with fever, leukopenia and hepatosplenomegaly and lymphadenopathy. What is the diagnosis?
  5. What drug 1s indicated 1n treatment of Q,?

Answer

JIA pauciarticular type I

MNJ22-003
A 35-day-old male child came with complaints of rash over the face from last 4 weeks.

On examination, HR was 58/min but child is not in CHF. Face picture is given below:

images/MNJ-P-OSCE 22. Connective tissue disorder_page_0_Picture_22.jpeg
images/MNJ-P-OSCE 22. Connective tissue disorder_page_1_Picture_2.jpeg

  1. What 1s diagnosis?
  2. What is clinical condition in mother you will see?
  3. When you will diagnosis of this earliest in antenatal period? And what test you will do?
  4. What treatment you will offer to mother if condition diagnosis of antenatal?
  5. What 1s risk in next pregnancy?
  6. What is probable diagnosis and what are diagnosis criterias?
  7. What 1s indication for steroid?
  8. Unit head advice at discharge to monitor ure protein. What 1s importance of this?
  9. What antibiotic use for this condition?
  10. Write 2 disease with small vessel vasculitis.

Answer

Neonatal lupus

MNJ22-004
A 4-year-old male child presents with complaints of fever for 5 days with all feature shown in picture. Antibiotic was given but no response. Coronary angiogram was done:

Image: Dr Manoj Sharma

images/MNJ-P-OSCE 22. Connective tissue disorder_page_1_Picture_15.jpeg
images/MNJ-P-OSCE 22. Connective tissue disorder_page_1_Picture_16.jpeg

  1. Whats probable diagnosis?
  2. Diagnostic criteria?
  3. What are ECHO criteria for this disease?
  4. What are DDs? At least 4
  5. What is treatment?
  6. What 1s follow-up plan for a child with single coronary artery involvement?

Answer

Kawasaki disease

Risk stratification of Kawasaki disease (AHA 2004)
Table 22.1:
Risk level Pharmacological
therapy		 Physical activity
diagnostic testing		 Follow-up Invasive testing
I.
artery
involvement)		 (Nocoronary None beyond Ist
6-5 weeks		 Norestrictions beyond
Ist 6-8 weeks		 Cardiovascular
risk assessment,
counseling at
5-year intervals		 None
(Transient
Il.
coronary
artery ectasia
disappears
within 1st
6-8 weeks)		 None beyond Ist
6-8 weeks		 Norestrictions beyond
Ist 6-8 weeks		 Cardiovascular
risk assessment,
counseling at 3—
5-year intervals		 None
(1 small-
Tif.
medium
coronary
artery
aneurysm /
major coro-
nary artery)		 Low-dose aspirin
(3-5 mg/kg
until aneurysm
regression
documented		 For patients <11-year-old)
no restriction beyond 1st follow-up with
aspirin /d), at least 6-8 weeks; patients
11—20-year-old, physical
activity guided by
biennial stress test,
evaluation of myocardial counseling;
perfusion scan; contact
or high-impact sports
discouraged for patients of myocardial
taking antiplatelet agents		 Annual cardiology Angiography, if
echocardiogram + suggests ischemia
ECG, combined
with cardiovascular
risk assessment,
biennial stress
test/evaluation
perfusion scan		 noninvasive test
(1 large or
IV.
giantcoro-
nary artery
aneurysm,
or multiple
orcomplex
same coro-
nary artery,
truction)		 Long-term anti-
platelet therapy and)
warfarin (target INR) because of risk of
2.0-2.5) or low-
heparin (target:
0.5-1.0 U/ml)
should be com-
without obs- bined in giant
aneurysms		 Contact or high-impact
sports should be avoided}
bleeding; other physical evaluation of
molecular-weight activity recommendations! myocardial perfu- indicated;
guided by stress test/
aneurysms in Antifactor Xa level evaluation of myocardial
perfusion scan outcome		 Biannual follow-up]
with ECHO + ECG;)
annual stress test/ sooner if
sion scan		 Ist angiography
at 6-12 mo or
clinically
repeated
angiography if
noninvasive test,
clinical, or
laboratory
findings suggest
ischemia
(Coronary
V.
artery obs-
truction)		 Long-term low-
farin or LMWH
reduce myocardial
O, consumption		 Contact or high-impact
dose aspirin; war- sports should be avoided)
other physical activity
if giant aneurysm recommendations guided)
persists; consider by stress test/myocardial of myocardial
use of f-blockers to} perfusion scan outcome perfusion scan		 Biannual follow-up)
with ECHO and
ECG; annual stress address
test/evaluation		 Angiography
recommended to
therapeutic
options

images/MNJ-P-OSCE 22. Connective tissue disorder_page_6_Picture_2.jpeg

MNJ22-005
A 7-year-old male child presents with complaints of knee joint swelling for 2 weeks duration with limitation of movement, there is history of gastroenteritis 2 weeks back, CBC— Normal, X-ray knee—normal:

  1. What 1s probable diagnosis?
  2. Whats HLA associated with this? And how many % patient associated with this?
  3. What 1s causative agents 4 bacteria and 2 virus?
  4. What is prognosis?

Answer

  1. Post-infectious reactive arthritis

MNJ22-006
A 13-year-old female presents with fever for 10 days, swelling of feet and pain abdomen. One episode of seizure present 2 days back, on examination, free fluid is present in abdominal cavity with pallor and rash over face given below:

images/MNJ-P-OSCE 22. Connective tissue disorder_page_2_Picture_2.jpeg

  1. What investigation will you order to confirm the diagnosis?
  2. What are 4 drugs that lead to this disease?
  3. What are treatment options?
  4. What HLA is associated with this?

Answer

  1. SLE

MNJ22-007
True/False

  1. Congenital deficiency of C1 q, C2, C4 associated with SLE
  2. Positive lupus anticoagulant/anticardiolipin AB are also associated with SLE.
  3. ANA is good screening test for SLE because it is associated with 95% cases of SLE.
  4. MC causes of death is infection, GN, neuropsychiatric disease
  5. Hip joint never involves in oligoarticular arthritis.

Answer

  1. True, 2. True, 3 False, 4 True, 5. True 6. True See Nelson for all explanation.

MNJ22-008
Match the following drugs according to their action:

  1. Methotrexate A. Anti CD20

2 Adaluzimab B: Interleukin 1 receptor antagonist

  1. Abatacept C. DMARD

4 Rituximab D Anti-tumor necrosis factor alpha

5 Anakınra E: Anti-cytotoxic T-lymphocyte associated antigen 4 Ig

Answer

  1. C, 2. D, 3. E, 4. A, 5. B

MNJ22-009
A 4-year-old child presents with complaints of fatigue and weakness during walking, child also develop red rash around eyes and hand. He has history of gastroenteritis 4 weeks back:

images/MNJ-P-OSCE 22. Connective tissue disorder_page_2_Picture_23.jpeg

  1. What is probable diagnosis?
  2. What is sign shown in this picture? And what is presents over face?
  3. What serum enzyme level you will advice for diagnosis?
  4. What antibody present in serum?
  5. What drug use for these skin lesion with dose and side effect?

Answer

  1. Juvenile dermatomyositis

MNJ22-010
A 9-year-old female child presents with complaints of fever, malaise, weight loss, headache and cloudication for last 2 months, on examination, child has high BP and low pulse. Angiography done that is shown below:

images/MNJ-P-OSCE 22. Connective tissue disorder_page_3_Picture_10.jpeg

  1. Whats probable diagnosis?
  2. What is most common artery involve in this?
  3. What is criteria for diagnosis?
  4. What 1s prognosis?

Answer

  1. Takayasu arteritis or pulseless disease.