A case of SLE polyserositis & pneumonitis

1. An Interesting case
of PUO
M 7 UNIT
Prof Sundar

2. About the patient
Mrs Mariapalam,61/F from Valliyoor near Nagercoil admitted for evaluation of fever since
March 2008:
Fever high grade intermittent followed by sweating
no evening rise or specific pattern
no chills or rigors
body pain+
arthralgia involving large joints without swelling,small joint involvement,hand joint involvement or
early morning stiffness
loss of appetite+;no significant weight loss
She was treated in her native place with only temporary relief
Breathlessness insiduous onset-exertional,gradually worsened to Class IV with orthopnoea;no
PND
Cough with small quantity mucoid sputum;no hemoptysis
Negative past history except for a suppurative left axillary adenitis which resolved with treatment
Negative family history;three healthy siblings;three children who are well;no BOH
She was brought to Chennai and consulted a physician who hospitalised her and the following
investigations were done:

3. Investigations:2/4/08
Hb 12.6gm/dl CXR cardiomegaly;lung fields clear
TC 9000 cells/cu mm MP,MF by QBC neg
DC P70,L27,E03 Widal neg
ESR 22/45 Lepto Ab IgM Elisa: 18.45 (equivocal) >20+ve
Urine: SG 1.005 USG Abdomen:Hepatomegaly
pH 7.0 RK 10.8x4
WBC 1+ LK 10.7x4
RBC nil No ascites
Nitrite neg VDRL : Reactive
Protein neg HIV I&II neg
Glucose neg RFT&LFT normal
Ketones neg HBsAg neg
Urobil,BS,BP neg Blood C/S no growth
ECG:Sinus tachycardia T inversion in V2-4

5. Contd..
Echo: Pericardial effusion 500ml with early
tamponade;valves,chambers normal;no RWMA;N LV function
TFT: T3 71.69ng/dl(N 80-180)
T4 3.6mgm/dl(N 4.5-11.5)
TSH 100.960mIu/ml(N 0.35-5.5)
Cardiologist suggested medical management of effusion.
Patient was started on ATT: AKT4 kit daily regime on 4/4/08 along with
Thyroxine 100mcg and discharged with a provisional diagnosis of
Tuberculous Pericardial effusion,and Hypothyroidism.
She was followed up as op by same physician with repeat CXR and Mx
which were negative
CECT Abdomen normal
However, she discontinued ATT and got admitted here on 4/5/08.

10. On admission at Stanley GH
O/E Obese lady
Febrile
Dyspnoeic and tachypnoeic
No cyanosis,clubbing
No pallor,adenopathy
Oral ulcers+
No skin,hair,nail or eye changes;no bony tenderness or joint swelling/deformities
JVP not elevated;no pedal edema or facial puffiness
Tachycardic,BP 120/70,all peripheral pulses+
CVS:Heart sounds were normal;no gallop or murmurs
RS:Trachea in midline;NVBS;Coarse crepitations in Rt
interscapular,infrascapular,axillary,infra-axillary areas with diffuse rhonchi
P/A:No ascites or organomegaly
CNS:N

11. Investigations done here:4/5/08
Hb 8.1gm/dl MSAT negative
TC 8900 cells/cu mm Widal negative
DC P90 L10 QBC negative
ESR 20/42 Dengue IgM +ve
PCV 25% IgG -ve
Platelets 3.15 lakh RFT & LFT N
MCV 77.6
MCHC 33.6
MCH 26.1
Peripheral smear: microcytic hypochromic anemia
Urine analysis: albumin-nil
pus cells-3-4/hpf
RBC-nil
bacteria-nil
ECG Sinus Tachycardia T inv in V2-4
Echo Pericardial effusion;no tamponade
Cardiologist opinion conservative management
CXR Cardiac silhouette enlarged with bilateral patchy infiltrates more in right lower zone with obliteration of costo and
cardiophrenic angles

13. Contd..6/5/08
Mx negative
Sputum 3 samples negative for AFB
HIV 1&2 negative
Sputum C/S Staph aureus sensitive to Erythro;yeast cells also grown
VDRL weakly reactive
ASO +ve 400 IU/ml
CRP +ve 96mg/dl
ANA +ve (IFA using HEp 2 cells & primate liver section) 1:100 (3+)
Pattern Homogenous S/O SLE/CTD
RAF 6.1 IU/ml(>14+ve)
CT Chest:B/L Pleural effusion
homogenous airspace opacity-posterior segment of right UL
and superior segment of right lower lobe

24. Contd..
Provisional Diagnosis:
SLE
Serositis-Bilateral pleural&pericardial
effusions
Rt lower lobe consolidation:
?Infective
?Acute Lupus Pneumonitis

25. Treatment
Rheumatologist opinion:
SLE with patchy pneumonitis and serositis
?Infective ?Lupus pneumonitis
Antibiotics and Prednisolone
Repeat Sputum C/S on 14.5.08 grew Staph aureus sensitive to
Vancomycin
Vancomycin started on 18.5.08
Patient continued to be febrile and tachypnoeic
Developed elevated renal parameters on 5
th
day and vancomycin
stopped
Dyspnoea worsened and was shifted to IMCW for respiratory support
on 21.5.08

26. RFT
1.52.12.63.12.40.6Creat
132140551218225Urea
29.527.524.523.5
renal
lab
22.58.5.08

27. ABG
pH 7.495
pCO2 20.6
pO2 67.4
HCO3 15.5
BE(ecf) -7.8
O2 sat 95.2%
Ct CO2 16.1mmol/L
Na 135 meq/L
K 4.3 meq/L

28. At IMCW
Anti ds DNA 30.4 U/ml
(neg <20 U/ml
pos >20 U/ml)
Pericardiocentesis was done:
Sugar 79mg/dl
Protein 4.4gm/dl
Cells RBC-120 cells;Lymphocytes-8 cells
C/S no growth
Smear neg for AFB
ADA(fluid) 40.4 U/L
Serum ADA 69.9 U/L
Urine C/S grew Pseudomonas
Patient was treated at IMCW with antibiotics,low dose steroids (oral pred 10mg/d) and
fluid management
Her metabolic parmeters improved;did not require ventilation and shifted back to ward

29. Contd..
Patient continued to be febrile and tachypnoeic
despite antibiotics
Minimal sputum production;dry cough +
Repeated induced sputum C/S and AFB neg
Rheumatologist reviewed and suggested
parenteral steroids
Started on IV Methyl Pred 1gm/d x 5 days
Patient improved from second dose.

34. Final Diagnosis
LUPUS PNEUMONITIS

35. ARA criteria for SLE
Malar rash
Discoid rash
Photosensitivity
Oral ulcers +
Arthritis
Serositis +
Renal disorders
Neurological disorder
Hematological disorder
Immunological disorder +
ANA +
>/=4 documented,present any time in a patient
95%specific & 75% sensitive

36. ANA
Prevalence in SLE:
active 95-100%
inactive 80-100%
Can be +ve in 5% healthy women & 3% men
Also +ve in DIL: hydralazine, procainamide, anticonvulsants, INH
MCTD, RA, PSS, Poly&dermatomyositis
Sjogren’s, Chr Active Hepatitis, UC
Negative test r/o SLE
High titre positivity (1:100) with other criteria favors Dx
Needs to be confirmed with other tests such as Anti ds DNA

37. Pleuro pulmonary manifestations of
SLE
Lupus pneumonitis
Lymphocytic interstitial pneumonitis
Pulmonary hemorrhage
Pulmonary embolism associated with LA
Pulmonary hypertension
Pleuritis
Weakness of diaphragm

38. Lupus pneumonitis
Acute:
12% of active lupus
fever,pleuritic pain,dyspnoea,cough, cyanosis
B/L pulmonary infiltrates and effusion
HP:alveolar damage,interstitial edema,hyaline membranes
perivascular lymphocytic & plasma cell infiltrates- clear or persist causing
PFT abn
Chronic:
Similar to other interstitial lung diseases
Cough-nonproductive,dyspnoea,basilar rales and abn PFT with
persistent infiltrates
HP:fibrosis,necrosis,plasma cell infiltration with histiocytic desquamation
IF:immune complex in alveolar wall

39. Lupus pneumonitis contd..
Diagnosis is one of exclusion
D/D
Infective consolidation
Pulmonary hemorrhage
Treatment:
acute-steroids,immunosuppressants if steroid unresponsive
chronic-asymptomatic:no treatment;poor prognosis if PFT abn
Prognosis:
poor; 50% mortality
sequelae for survivors is severe restrictive lung disease

40. Acute Lupus pneumonitis

41. ADA
Catalyses deamination of adenosine & deoxyadenosine to inosine & deoxyinosine
Found in most cells
2 isoenzymes ADA1 & 2
ADA 2 is found in macrophages,monocytes
Released by organisms within these cells into fluids
ADA 2 is more diagnostic for TB than total ADA
False +ve in lymphoma,RA,SLE & adenocarcinoma
Sensitivity 90-100%
Specificity 89-100%
To increase sensitivity of Dx of TB, Pleural fluid ADA>50U/L + L/N ratio > 0.75
(Burgess LJ et al:Chest 1996)
Cut-off for TB pericarditis ADA>40U/L but lymphocytosis must be +
sensitivity 89% specificity 72%
IFN gamma >50pg/ml:most useful test
(Cardiovasc JS Afr 2005 16(3)
QJM 2006 dec 99(12)
Acta Trop 2006 Aug 99-meta-analysis
Rev inst Med Prop Sao Paulo 2007 May jun 49(3)
Serum ADA levels are markers of disease activity in SLE