A case of SLE polyserositis & pneumonitis

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A case of SLE polyserositis & pneumonitis

  1. 1. An Interesting case of PUO M 7 UNIT Prof Sundar
  2. 2. About the patient <ul><li>Mrs Mariapalam,61/F from Valliyoor near Nagercoil admitted for evaluation of fever since </li></ul><ul><li>March 2008: </li></ul><ul><li>Fever high grade intermittent followed by sweating </li></ul><ul><li>no evening rise or specific pattern </li></ul><ul><li>no chills or rigors </li></ul><ul><li>body pain+ </li></ul><ul><li>arthralgia involving large joints without swelling,small joint involvement,hand joint involvement or early morning stiffness </li></ul><ul><li>loss of appetite+;no significant weight loss </li></ul><ul><li>She was treated in her native place with only temporary relief </li></ul><ul><li>Breathlessness insiduous onset-exertional,gradually worsened to Class IV with orthopnoea;no PND </li></ul><ul><li>Cough with small quantity mucoid sputum;no hemoptysis </li></ul><ul><li>Negative past history except for a suppurative left axillary adenitis which resolved with treatment </li></ul><ul><li>Negative family history;three healthy siblings;three children who are well;no BOH </li></ul><ul><li>She was brought to Chennai and consulted a physician who hospitalised her and the following investigations were done: </li></ul>
  3. 3. Investigations:2/4/08 <ul><li>Hb 12.6gm/dl CXR cardiomegaly;lung fields clear </li></ul><ul><li>TC 9000 cells/cu mm MP,MF by QBC neg </li></ul><ul><li>DC P70,L27,E03 Widal neg </li></ul><ul><li>ESR 22/45 Lepto Ab IgM Elisa: 18.45 (equivocal) >20+ve </li></ul><ul><li>Urine: SG 1.005 USG Abdomen:Hepatomegaly </li></ul><ul><li>pH 7.0 RK 10.8x4 </li></ul><ul><li>WBC 1+ LK 10.7x4 </li></ul><ul><li>RBC nil No ascites </li></ul><ul><li>Nitrite neg VDRL : Reactive </li></ul><ul><li>Protein neg HIV I&II neg </li></ul><ul><li>Glucose neg RFT&LFT normal </li></ul><ul><li>Ketones neg HBsAg neg </li></ul><ul><li>Urobil,BS,BP neg Blood C/S no growth </li></ul><ul><li>ECG:Sinus tachycardia T inversion in V2-4 </li></ul>
  4. 5. Contd.. <ul><li>Echo: Pericardial effusion 500ml with early tamponade;valves,chambers normal;no RWMA;N LV function </li></ul><ul><li>TFT: T3 71.69ng/dl(N 80-180) </li></ul><ul><li>T4 3.6mgm/dl(N 4.5-11.5) </li></ul><ul><li>TSH 100.960mIu/ml(N 0.35-5.5) </li></ul><ul><li>Cardiologist suggested medical management of effusion. </li></ul><ul><li>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. </li></ul><ul><li>She was followed up as op by same physician with repeat CXR and Mx which were negative </li></ul><ul><li>CECT Abdomen normal </li></ul><ul><li>However, she discontinued ATT and got admitted here on 4/5/08. </li></ul>
  5. 10. On admission at Stanley GH <ul><li>O/E Obese lady </li></ul><ul><li>Febrile </li></ul><ul><li>Dyspnoeic and tachypnoeic </li></ul><ul><li>No cyanosis,clubbing </li></ul><ul><li>No pallor,adenopathy </li></ul><ul><li>Oral ulcers+ </li></ul><ul><li>No skin,hair,nail or eye changes;no bony tenderness or joint swelling/deformities </li></ul><ul><li>JVP not elevated;no pedal edema or facial puffiness </li></ul><ul><li>Tachycardic,BP 120/70,all peripheral pulses+ </li></ul><ul><li>CVS:Heart sounds were normal;no gallop or murmurs </li></ul><ul><li>RS:Trachea in midline;NVBS;Coarse crepitations in Rt interscapular,infrascapular,axillary,infra-axillary areas with diffuse rhonchi </li></ul><ul><li>P/A:No ascites or organomegaly </li></ul><ul><li>CNS:N </li></ul>
  6. 11. Investigations done here:4/5/08 <ul><li>Hb 8.1gm/dl MSAT negative </li></ul><ul><li>TC 8900 cells/cu mm Widal negative </li></ul><ul><li>DC P90 L10 QBC negative </li></ul><ul><li>ESR 20/42 Dengue IgM +ve </li></ul><ul><li>PCV 25% IgG -ve </li></ul><ul><li>Platelets 3.15 lakh RFT & LFT N </li></ul><ul><li>MCV 77.6 </li></ul><ul><li>MCHC 33.6 </li></ul><ul><li>MCH 26.1 </li></ul><ul><li>Peripheral smear: microcytic hypochromic anemia </li></ul><ul><li>Urine analysis: albumin-nil </li></ul><ul><li>pus cells-3-4/hpf </li></ul><ul><li>RBC-nil </li></ul><ul><li>bacteria-nil </li></ul><ul><li>ECG Sinus Tachycardia T inv in V2-4 </li></ul><ul><li>Echo Pericardial effusion;no tamponade </li></ul><ul><li>Cardiologist opinion conservative management </li></ul><ul><li>CXR Cardiac silhouette enlarged with bilateral patchy infiltrates more in right lower zone with obliteration of costo and cardiophrenic angles </li></ul>
  7. 13. Contd..6/5/08 <ul><li>Mx negative </li></ul><ul><li>Sputum 3 samples negative for AFB </li></ul><ul><li>HIV 1&2 negative </li></ul><ul><li>Sputum C/S Staph aureus sensitive to Erythro;yeast cells also grown </li></ul><ul><li>VDRL weakly reactive </li></ul><ul><li>ASO +ve 400 IU/ml </li></ul><ul><li>CRP +ve 96mg/dl </li></ul><ul><li>ANA +ve (IFA using HEp 2 cells & primate liver section) 1:100 (3+) </li></ul><ul><li>Pattern Homogenous S/O SLE/CTD </li></ul><ul><li>RAF 6.1 IU/ml(>14+ve) </li></ul><ul><li>CT Chest:B/L Pleural effusion </li></ul><ul><li>homogenous airspace opacity-posterior segment of right UL </li></ul><ul><li>and superior segment of right lower lobe </li></ul>
  8. 24. Contd.. <ul><li>Provisional Diagnosis: </li></ul><ul><li>SLE </li></ul><ul><li>Serositis-Bilateral pleural&pericardial effusions </li></ul><ul><li>Rt lower lobe consolidation: </li></ul><ul><li>?Infective </li></ul><ul><li>?Acute Lupus Pneumonitis </li></ul>
  9. 25. Treatment <ul><li>Rheumatologist opinion: </li></ul><ul><li>SLE with patchy pneumonitis and serositis </li></ul><ul><li>?Infective ?Lupus pneumonitis </li></ul><ul><li>Antibiotics and Prednisolone </li></ul><ul><li>Repeat Sputum C/S on 14.5.08 grew Staph aureus sensitive to Vancomycin </li></ul><ul><li>Vancomycin started on 18.5.08 </li></ul><ul><li>Patient continued to be febrile and tachypnoeic </li></ul><ul><li>Developed elevated renal parameters on 5 th day and vancomycin stopped </li></ul><ul><li>Dyspnoea worsened and was shifted to IMCW for respiratory support on 21.5.08 </li></ul>
  10. 26. RFT 1.5 2.1 2.6 3.1 2.4 0.6 Creat 132 140 55 121 82 25 Urea 29.5 27.5 24.5 23.5 renal lab 22.5 8.5.08
  11. 27. ABG <ul><li>pH 7.495 </li></ul><ul><li>pCO2 20.6 </li></ul><ul><li>pO2 67.4 </li></ul><ul><li>HCO3 15.5 </li></ul><ul><li>BE(ecf) -7.8 </li></ul><ul><li>O2 sat 95.2% </li></ul><ul><li>Ct CO2 16.1mmol/L </li></ul><ul><li>Na 135 meq/L </li></ul><ul><li>K 4.3 meq/L </li></ul>
  12. 28. At IMCW <ul><li>Anti ds DNA 30.4 U/ml </li></ul><ul><li>(neg <20 U/ml </li></ul><ul><li>pos >20 U/ml) </li></ul><ul><li>Pericardiocentesis was done: </li></ul><ul><li>Sugar 79mg/dl </li></ul><ul><li>Protein 4.4gm/dl </li></ul><ul><li>Cells RBC-120 cells;Lymphocytes-8 cells </li></ul><ul><li>C/S no growth </li></ul><ul><li>Smear neg for AFB </li></ul><ul><li>ADA(fluid) 40.4 U/L </li></ul><ul><li>Serum ADA 69.9 U/L </li></ul><ul><li>Urine C/S grew Pseudomonas </li></ul><ul><li>Patient was treated at IMCW with antibiotics,low dose steroids (oral pred 10mg/d) and fluid management </li></ul><ul><li>Her metabolic parmeters improved;did not require ventilation and shifted back to ward </li></ul>
  13. 29. Contd.. <ul><li>Patient continued to be febrile and tachypnoeic despite antibiotics </li></ul><ul><li>Minimal sputum production;dry cough + </li></ul><ul><li>Repeated induced sputum C/S and AFB neg </li></ul><ul><li>Rheumatologist reviewed and suggested parenteral steroids </li></ul><ul><li>Started on IV Methyl Pred 1gm/d x 5 days </li></ul><ul><li>Patient improved from second dose. </li></ul>
  14. 34. Final Diagnosis <ul><li>LUPUS PNEUMONITIS </li></ul>
  15. 35. ARA criteria for SLE <ul><li>Malar rash </li></ul><ul><li>Discoid rash </li></ul><ul><li>Photosensitivity </li></ul><ul><li>Oral ulcers + </li></ul><ul><li>Arthritis </li></ul><ul><li>Serositis + </li></ul><ul><li>Renal disorders </li></ul><ul><li>Neurological disorder </li></ul><ul><li>Hematological disorder </li></ul><ul><li>Immunological disorder + </li></ul><ul><li>ANA + </li></ul><ul><li>>/=4 documented,present any time in a patient </li></ul><ul><li>95%specific & 75% sensitive </li></ul>
  16. 36. ANA <ul><li>Prevalence in SLE: </li></ul><ul><li>active 95-100% </li></ul><ul><li>inactive 80-100% </li></ul><ul><li>Can be +ve in 5% healthy women & 3% men </li></ul><ul><li>Also +ve in DIL: hydralazine, procainamide, anticonvulsants, INH </li></ul><ul><li>MCTD, RA, PSS, Poly&dermatomyositis </li></ul><ul><li>Sjogren’s, Chr Active Hepatitis, UC </li></ul><ul><li>Negative test r/o SLE </li></ul><ul><li>High titre positivity (1:100) with other criteria favors Dx </li></ul><ul><li>Needs to be confirmed with other tests such as Anti ds DNA </li></ul>
  17. 37. Pleuro pulmonary manifestations of SLE <ul><li>Lupus pneumonitis </li></ul><ul><li>Lymphocytic interstitial pneumonitis </li></ul><ul><li>Pulmonary hemorrhage </li></ul><ul><li>Pulmonary embolism associated with LA </li></ul><ul><li>Pulmonary hypertension </li></ul><ul><li>Pleuritis </li></ul><ul><li>Weakness of diaphragm </li></ul>
  18. 38. Lupus pneumonitis <ul><li>Acute: </li></ul><ul><li>12% of active lupus </li></ul><ul><li>fever,pleuritic pain,dyspnoea,cough, cyanosis </li></ul><ul><li>B/L pulmonary infiltrates and effusion </li></ul><ul><li>HP:alveolar damage,interstitial edema,hyaline membranes </li></ul><ul><li>perivascular lymphocytic & plasma cell infiltrates- clear or persist causing PFT abn </li></ul><ul><li>Chronic: </li></ul><ul><li>Similar to other interstitial lung diseases </li></ul><ul><li>Cough-nonproductive,dyspnoea,basilar rales and abn PFT with persistent infiltrates </li></ul><ul><li>HP:fibrosis,necrosis,plasma cell infiltration with histiocytic desquamation </li></ul><ul><li>IF:immune complex in alveolar wall </li></ul>
  19. 39. Lupus pneumonitis contd.. <ul><li>Diagnosis is one of exclusion </li></ul><ul><li>D/D </li></ul><ul><li>Infective consolidation </li></ul><ul><li>Pulmonary hemorrhage </li></ul><ul><li>Treatment: </li></ul><ul><li>acute-steroids,immunosuppressants if steroid unresponsive </li></ul><ul><li>chronic-asymptomatic:no treatment;poor prognosis if PFT abn </li></ul><ul><li>Prognosis: </li></ul><ul><li>poor; 50% mortality </li></ul><ul><li>sequelae for survivors is severe restrictive lung disease </li></ul>
  20. 40. Acute Lupus pneumonitis
  21. 41. ADA <ul><li>Catalyses deamination of adenosine & deoxyadenosine to inosine & deoxyinosine </li></ul><ul><li>Found in most cells </li></ul><ul><li>2 isoenzymes ADA1 & 2 </li></ul><ul><li>ADA 2 is found in macrophages,monocytes </li></ul><ul><li>Released by organisms within these cells into fluids </li></ul><ul><li>ADA 2 is more diagnostic for TB than total ADA </li></ul><ul><li>False +ve in lymphoma,RA,SLE & adenocarcinoma </li></ul><ul><li>Sensitivity 90-100% </li></ul><ul><li>Specificity 89-100% </li></ul><ul><li>To increase sensitivity of Dx of TB, Pleural fluid ADA>50U/L + L/N ratio > 0.75 </li></ul><ul><li>(Burgess LJ et al:Chest 1996) </li></ul><ul><li>Cut-off for TB pericarditis ADA>40U/L but lymphocytosis must be + </li></ul><ul><li>sensitivity 89% specificity 72% </li></ul><ul><li>IFN gamma >50pg/ml:most useful test </li></ul><ul><li>(Cardiovasc JS Afr 2005 16(3) </li></ul><ul><li>QJM 2006 dec 99(12) </li></ul><ul><li>Acta Trop 2006 Aug 99-meta-analysis </li></ul><ul><li>Rev inst Med Prop Sao Paulo 2007 May jun 49(3) </li></ul><ul><li>Serum ADA levels are markers of disease activity in SLE </li></ul>

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