a case of sle polyserositis & pneumonitis

An Interesting case of PUO

M 7 UNIT

Prof Sundar

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 lossShe 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 BOHShe was brought to Chennai and consulted a physician who hospitalised her and the following

investigations were done:

Investigations:2/4/08

Hb 12.6gm/dl CXR cardiomegaly;lung fields clearTC 9000 cells/cu mm MP,MF by QBC negDC P70,L27,E03 Widal negESR 22/45 Lepto Ab IgM Elisa: 18.45 (equivocal) >20+veUrine: 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

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 normalHowever, she discontinued ATT and got admitted here on 4/5/08.

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

Investigations done here:4/5/08Hb 8.1gm/dl MSAT negativeTC 8900 cells/cu mm Widal negativeDC P90 L10 QBC negative ESR 20/42 Dengue IgM +vePCV 25% IgG -vePlatelets 3.15 lakh RFT & LFT NMCV 77.6MCHC 33.6MCH 26.1Peripheral smear: microcytic hypochromic anemiaUrine analysis: albumin-nil pus cells-3-4/hpf RBC-nil bacteria-nilECG Sinus Tachycardia T inv in V2-4Echo Pericardial effusion;no tamponadeCardiologist opinion conservative managementCXR Cardiac silhouette enlarged with bilateral patchy infiltrates more in right lower zone with obliteration of costo and

cardiophrenic angles

Contd..6/5/08

Mx negativeSputum 3 samples negative for AFBHIV 1&2 negativeSputum C/S Staph aureus sensitive to Erythro;yeast cells also grownVDRL weakly reactiveASO +ve 400 IU/mlCRP +ve 96mg/dlANA +ve (IFA using HEp 2 cells & primate liver section) 1:100 (3+)Pattern Homogenous S/O SLE/CTDRAF 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

Contd..

Provisional Diagnosis:SLESerositis-Bilateral pleural&pericardial

effusionsRt lower lobe consolidation:?Infective?Acute Lupus Pneumonitis

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

VancomycinVancomycin started on 18.5.08Patient continued to be febrile and tachypnoeicDeveloped elevated renal parameters on 5th day and vancomycin

stoppedDyspnoea worsened and was shifted to IMCW for respiratory support

on 21.5.08

RFT

8.5.08 22.5 23.5 renal lab

24.5 27.5 29.5

Urea 25 82 121 55 140 132

Creat 0.6 2.4 3.1 2.6 2.1 1.5

ABG

pH 7.495pCO2 20.6pO2 67.4HCO3 15.5BE(ecf) -7.8O2 sat 95.2%Ct CO2 16.1mmol/LNa 135 meq/LK 4.3 meq/L

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 PseudomonasPatient was treated at IMCW with antibiotics,low dose steroids (oral pred 10mg/d) and

fluid managementHer metabolic parmeters improved;did not require ventilation and shifted back to ward

Contd..

Patient continued to be febrile and tachypnoeic despite antibiotics

Minimal sputum production;dry cough +Repeated induced sputum C/S and AFB negRheumatologist reviewed and suggested

parenteral steroidsStarted on IV Methyl Pred 1gm/d x 5 daysPatient improved from second dose.

Final Diagnosis

LUPUS PNEUMONITIS

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 patient95%specific & 75% sensitive

ANA

Prevalence in SLE: active 95-100% inactive 80-100%Can be +ve in 5% healthy women & 3% menAlso +ve in DIL: hydralazine, procainamide, anticonvulsants, INH MCTD, RA, PSS, Poly&dermatomyositis Sjogren’s, Chr Active Hepatitis, UCNegative test r/o SLEHigh titre positivity (1:100) with other criteria favors DxNeeds to be confirmed with other tests such as Anti ds DNA

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

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

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

Acute Lupus pneumonitis

ADA Catalyses deamination of adenosine & deoxyadenosine to inosine & deoxyinosine Found in most cells 2 isoenzymes ADA1 & 2 ADA 2 is found in macrophages,monocytesReleased by organisms within these cells into fluidsADA 2 is more diagnostic for TB than total ADA False +ve in lymphoma,RA,SLE & adenocarcinomaSensitivity 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