Pulmonary involvement

1. ADULT ONSET STILL’S DISEASE WITH
PULMONARY INVOLVEMENT
A CASE REPORT
Prof.Dr.Jyoti Patnaik
Asst.Prof.Dr.Thitta Mohanty
Dr.Amith Sreedharan
Dr.Niranjan Babu A

2. INTRODUCTION
An inflammatory disorder
Fever,lymphadenopathy,hepatosplenomegaly,
arthritis,myalgia ,skin rash
 Neutrophilic leucocytosis,High ESR
RF and ANA negative & Very High serum ferritin
levels
Respond to immunosuppressive therapy mainly
corticosteroids and disease modifying anti
rheumatic drugs(DMARD).

3. CASE REPORT
• 19 YEAR OLD FEMALE
• High spiking fever,Cough,Myalgia,Skin
rash,Joint pain,Sore throat for 20 days
• Non-toxic,febrile (40.0 C)
• Pallor,hepatomegaly
• Auscultation: bilateral fine inspiratory crackles
in lung bases

4. • Prior to admission ,started on Anti tubercular
therapy based on CT scan features without
any improvement even after 2 weeks.
• started on empirical antibiotics and
antimalarial with no symptomatic relief.

5. LAB PARAMETERS
• TLC: 30,400,N-91%,TPC – 2,50,000
• Hb-10g%
• Urine-Routine/microscopy – WNL
• HIV/HBsAg - Negative
• LFT,RFT – WNL
• THYROID PROFILE – WNL
• MP-ICT,DENGUE IgM,IgG,NS1- Negative
• Hb Electrophoresis- Normal
• Widal – Negative
• RF,ANA – Negative ; SACE- WNL (28U/L)

6. • Blood culture – No growth
• Sputum for AFB –(mucoid sample/IS)-Negative
• Sputum for G/S,C/S – No growth
• USG Thorax,Abdomen& Pelvis : Hepatomegaly
• CXR-PA : B/L LL nodular shadows
• HRCT Thorax: Multiple Centrilobular nodules
B/L Lower lobes
• 2D Echocardiography – Normal study

12. Etiologies of PUO
• Collagen-Vascular-Disease
No diagnostic serology…
Recognize the syndrome otherwise no diagnosis
– Still’s disease (young or adult)
– Giant cell arteritis } → 15% of PUO
– Polymyalgia Rheumatica }
– Behcet’s Disease
– Relapsing polychondritis

13. Pyrexia of Unknown Origin
The majority of disease remaining after an
initial NEGATIVE work-up are:
1. Neoplasm
2. Seronegative Collagen Vascular Disease
3. Increasing Tuberculosis
4. Increasing Drug Addition
5. Elderly with Endocarditis
6. HIV with or without infection or malignancy
7. Implanted prosthetic devices
8. Travel … New Exposure

14. Epidemiology and Etiology
Infections 30 - 40 %
Malignancies 20 – 25 %
Collagen Vascular Disease 10 – 20 %
Miscellaneous 15 – 20 %
Undiagnosed 10 – 15 %
Categories of Illness Causing PUO

15. PUO
• Old Definition:
1. Fever higher than 38.3oC on several occasions.
2. Duration of fever – 3 weeks
3. Uncertain diagnosis after one week of study in
hospital
• New Definition:
– Eliminated the in-hospital evaluation requirements
→ 3 outpatient visits, or 3 days in hospital. …
Ambulatory as well as in hospital

16. YAMAGUCHI’S CRITERIA
MAJOR CRITERIA (4/4)
INTERMITTENT HIGH SPIKING FEVER > 1 WEEK PRESENT
ARTHRALGIA/ARTHRITIS > 2 WEEKS PRESENT
TYPICAL EVANESCENT RASH PRESENT
LEUCOCYTOSIS (>10000) WITH >80% GRANULOCYTES PRESENT
MINOR CRITERIA (3/5)
SORE THROAT PRESENT
SIGNIFICANT LYMPHADENOPATHY ABSENT
HEPATOMEGALY OR SPLENOMEGALY PRESENT
ABNORMAL LIVER FUNCTION ABSENT
ANA , RF Negative PRESENT
EXCLUSION CRITERIA (2/2)
INFECTIONS ---
MALIGNANCIES ---
Diagnosis requires 5 or more criteria, 2 of which must be major

17. FAUTREL CRITERIA
MAJOR CRITERIA (5/6)
Spiking fever ≥ 39° C PRESENT
Arthralgias PRESENT
Transient erythematous rash PRESENT
Sore throat PRESENT
Polymorphonuclear cells ≥ 80% PRESENT
Glycosylated ferritin ≤ 20% ?
MINOR CRITERIA (2/2)
Maculopapular rash PRESENT
Leucocytosis ≥ 10000/mm PRESENT
Diagnosis requires at least 4 or more major criteria or 3 major + 2 minor criteria

18. DISCUSSION
• A young female with AOSD complicated by pulmonary
infiltrates.
• The annual incidence of aosd being 1.6 per million
population
• Equal representation of genders
• Bimodal age distribution of 15-25years and 36-45years.
• AOSD aka WISSLER FANCONI syndrome/ JRA
• Etiology is unknown although genetic predisposition
combined with viral triggers have been proposed

19. • Pulmonary involvement in AOSD is seen in
approximately 50% of cases
• Most common pulmonary manifestations
being pleural effusion(26.4%) transient
pulmonary infiltrates
• Rarely pulmonary fibrosis is also reported.
• Life threatening complications such as ARDS,
diffuse alveolar haemorrhage has also been
reported.

20. • Diagnosed according to YAMAGUCHI’S Criteria
(93.5%SENSITIVE)
• Fulfilled all the major criteria and 4 out of 5 of
the minor criteria.
• Classification criteria by FAUTREL(98.5%
SPECIFICITY),fulfilled 5 out of 6 major criteria and
all the minor criteria.
• Alternative diagnoses such as infection,
malignancy and other rheumatic diseases have
been ruled out.

21. • Fever, increase CRP levels, hyperferritinemia,
hepatosplenomegaly, haematological abnormalities
due to IL-6 and TNF ALPHA.
• The other cytokines involved IL-8 which causes
organomegaly
• IL-18, INTERFERON GAMMA , IL-1 BETA, sIL:2R.
• IL-18 is the most important cytokine involved &
believed to initiate the inflammatory cascade.
• Animal studies → exogenous administration of IL-18
resulted in neutrophil accumulation in lung tissue and
enhanced pulmonary microvascular leakage.

22. • The corner stone in treatment of AOSD is
CORTICOSTEROIDS along with NSAIDS and
DMARDs.
• Studies have shown benefit with TNF ALPHA
INHIBITORS.
• IV IG also reported to have some amount of
success in patients with serositis and
nephropathy.

23. • Macrophage activation syndrome and
Thrombotic thrombocytopenic purpura are life
threatening complications
• Disseminated intravascular coagulopathy(DIC)
-most common cause of death in systemic
JRA/AOSD.

24. THANK YOU