A TRICKY PROBLEM. Presenter-Dr Lakshmi PK

Transcription

1 A TRICKY PROBLEM Presenter-Dr Lakshmi PK

2 Patient particulars 33 years old Male Resident of Andhra Pradesh Occupation-soldier

3 Chief compliants Headache- 03 days Headache-global,throbbing type Associated with vomitting-non projectile, no blood/bile No other localizing symptomn.

4 Examination P-90/min reg, all PP palpable BP-180/110 mm of Hg No pallor, icterus, Cyanosis,clubbing,LNnpathy,Pedal oedema Fundoscopy- bilateral flame shaped hemorrhage s/o hypertensive retinopathy S/E-NAD

5 Investigation-29 SEP 14 Hb TLC PLT 10.1 gm/dl 9700/P65L lakhs Bilirubin 0.6/0.3 OT/PT/SAP 23/20/36 BUN/creatinine 78/12.8 Na/K 131/4.2 Ca/PO4 9.2/4.8 Ur RE/ME ipth Iron/Ferritin/TIBC Blood Sugar 87 25(OH)D3 Prot 4+,6-8 RBCs,8-10 WBCs,8-10 epithelial cells 249.1(14-72pg/dl) 164 mcg/dl, 120ng/ml, 197mcg/dl 17.25( ng/ml)

6 ECG

7 USG KUB RK-8.97 CM LK-8.9CM Increased cortical echogenicity with loss of CMD No HDN/Calculus/Focal lesion

8 Working diagnosis RPGN CGN-CKD

9 Management Started on HD + Supportive care Suspicion of RPGN Methyl prednisolone pulse x 3 Oral Prednisolone Kidney biopsy (after stabilization)

10 ANA canca/panca Anti GBM NEGATIVE NEGATIVE NEGATIVE C3 73.6( mg/dl) C4 dsdna HBsAg/Anti HCV HIV 24.1(15-48mg/dl) Negative Negative Negative 24 hour urinary protein 2451 mg/gm of creatinine

11 Kidney biopsy 12 glomeruli,11 obsolescent Marked IF/TA>50% of cortical area IF-2 obselete glomeruli neg for IgG,IgA,IgM,C3,C1q,K/ʎ Imp-chronic glomerulonephritis

12 Kidney biopsy

13 Kidney biopsy

14 Diagnosis Revised diagnosis: Chronic Glomerulonephritis-CKD stage 5D Plan: Tapered steroids Planned for renal transplant.

15 Course of illness: Asymptomatic for 10 days Dyspnoea & orthopnea Fever -1 day -1 day Examination P-140/min reg BP-160/120 mm of Hg JVP raised SpO2-86% at room air R/R-36/min Chest-bilateral crackles ECG-LVH pattern no new change.

16 INV Hb TLC Plt BUN Creatinine Na/K PBS 12gm/dl 17100/P64L lakhs 65mg/dl 8mg/dl 133/4.2 meq/l No shift to left No toxic granules

17 Inv CXR

18 Possibilities: 1. Pneumonia 2. Fluid overload 3. Combination of both Management: 1. Antibiotics 2. NIV support 3. Intensification of HD Outcome: Dramatic improvement

19 Repeat CXR CXR-PA: at admission CXR-PA: D-3

20 Status: Low grade fever persisted Clinically better/ ambulant Recurrence of similar symptoms after 20 days Exam/ Investigations: BP-160/110 mm of Hg Chest-bilateral crepitations CXR-right sided NHO Echo: Global HK, LVEF- 38%

21 HRCT chest

22 Therapy and outcome Antibiotics-21 days Intensification of HD Rapid resolution of symptoms Low grade fever persisted 4 th episode on 31 Dec 2015 Associated hemoptysis ml 3-4 episodes Nose/ throat/ oral cavity: normal Repeat C3/C4-(normal) ANA-negative ANCA-negative Anti GBM-negative Procalcitonin mg/ml(<0.04) On examination T F P-138/min reg BP-120/78 mm of Hg SpO2-70% at room air 84% with O2 inhalation Chest-bilateral crackles

23 Management Working diagnosis: Pneumonia + fluid overload (Kidney + Cardiac) Strong suspicion of vasculitis (Alveolar Hge) Management: NIV support + Antibiotics + HD Immunomodulation:?? (on going infection & raised procalcitonin) IVIg-5 days (cumulative 2 g/kg)

24 CXR Pre-therapy Post-therapy

25 Issues Issues: Recurrent episodes of chest infiltrates, improving with antibiotics and intensification of HD. Chronic glomerulonephritis- CKD 5D Alveolar hge Evidence of ongoing infection Negative autoimmune markers Plan: Bronchoscopy + TBLB Repeat kidney biopsy

26 Bronchoscopy Haemorrhagic BAL fluid

27 Multiple Thickened Blood Vessels with Interstitial Fibrin deposition

28 interstitial Fibrin deposition

29 Capillaritis and Hemosedirin Laden Macrophages

30 TBLB Organizing pneumonia with pulmonary hemorrhage, interstitial fibrosis and capillaritis Consistent with pulmonary manifestations of collagen vascular disease

31 Recurrence of symptoms MP x 3 TPE + IVIg x 5 Rituximab 375 mg/m2 weekly x 4 No recurrence of symptoms for the last 1 month

32 Pulmonary renal syndrome

33 Diagnosis-Pulmonary renal syndrome Pauci immune vasculitis: IF- neg, normal complements, lung & kidney involvement, Capillaritis, RPGN type picture (now burnt out) Odd point: ANCA negative (Drugs??) Goodpasture's syndrome Anti GBM antibody negative IF of kidney biopsy not supportive

34 DISCUSSION

35 Pulmonary Renal syndromes Definition- combination of diffuse alveolar haemorrhage (DAH) and glomerulonephritis Pulmonary small-vessel vasculitis Renal Inflammation of arterioles, venules and alveolar capillaries (necrotic pulmonary capillaritis). Proliferative glomerulonephritis-fibrinoid necrosis/crescent formation Interstitial infiltration, fibrosis and tubular atrophy Necrotizing granuloma Small vessel vasculitis Papiris et al. Critical Care 2007, 11:213

36 Pulmonary Renal syndromes ANCA asssociated vasculitis-70% 95% ANCA positive Anti GBM antibody associated-20% 92% anti GBM positive 5 10%-without e/o renal activity Zimmerman Cm et al Goodpasture s syndrome with normal renal function. Am J Med 1979;66: Hiroshi Yamaguchi et al Case Reports in Nephrology Volume 2013,

37 Diffuse alveolar hemorrhage in PRS 25% -fatal in first episode of DAH 50% require assisted ventilation. Recurrent episodes have sequalae- Obstructive lung disease Pulmonary fibrosis Brown Kevin et al Proc Am Thorac Soc Vol 3. pp 48 57, 2006

38 Treatment as per disease severity Severe disease defined by - functional impairment of critical organs- renal disease (creatinine- 5.7 mg/dl)/ DAH/ other life-threatening disease Severe disease-plasma exchange followed by maintenance immunosupression Most dialysis dependent Papiris et al. Critical Care 2007, 11:213 Brown Kevin et al Proc Am Thorac Soc Vol 3. pp 48 57, 2006

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