Wegener Granulomatosis

Wegener GranulomatosisMuhamad SyahilmiWhat Is Wegener GranulomatosisWegeners granulomatosis is a rare disease, in which the blood vessels and other tissues become inflamed. This inflammation damages important organs of the body by limiting blood flow to those organs and destroying normal tissueIt was first described by Klinger in 1933 and by other investigators such as Rossle in 1933, Wegener in 1936 and 1939 and Ringertz in 1947.

PathogenesisThe cause of WG is unknown

Clinical featureWG is most commonly associated with disease of the sinuses, lungs, and kidneys, but it is very much a multisystem disease.

Ear/nose/throat/mouth Nasal septum perforation, saddle-nose deformity, conductive or sensorineural hearing loss, subglottic stenosis, strawberry gums, oral ulcers

EyeOrbital pseudotumor, conjunctivitis, keratitis (risk of corneal melt), anterior uveitis, scleritis, episcleritis

LungNodules, infiltrates, cavitary lesions, alveolar hemorrhage, bronchial lesions

HeartOccasional valvular lesions, pericarditis

KidneySegmental necrotizing glomerulonephritis, renal failure requiring hemodialysis

SkinVesicular, palpable purpuric, ulcerative, or hemorrhagic lesions

Treatment For Wegener Granulomatosis

CyclophosphamideThe typical daily cyclophosphamide dose is2 mg/kg/day, and should generally not exceed200 mg/day. Dose adjustments are critical for patients with older age (e.g. greater than70 years), renal dysfunction MethotrexateMethotrexate at a starting dose of 0.25 mg/kg/week (typically 1520 mg/week), increased to a maximum of 25 mg/weekGlucocorticoiddose of prednisone in the range of 0.51.0 mg/kg (up to a maximum of 80 mg/day)

ObjectiveTo Discuss Management approach of Wegener GranulomatosisTo discuss about the drug related problem and pharmaceutical care issue of this casePatients demographicsNameKReg. No. 567112Age25GenderFemaleRaceIndiaWeight58kgHeight150cmDOA11/4/14AllergyAllergy tp penicilin group and augmentin GroupDuration of hospitalization10 daysChief ComplaintUnresolved Painful swelling at ankle Joint

History of Presenting IllnessWorsening bilateral lower limb edema 2/12,Painful and proggresively worsening complain of pain on movementDifficulty in ambulating 2/12Fever ,SOB and dry cough for 2/7Loss of appetite 3/7Family and social historySingle stay at an orphanage homeSupport by herself by working as waiterMother had diabetes mellitus and thyroid problemNo smoking or alcohol intakePast MEDICAL HISTORY1)Wegener GranulomatosisDiagnosed 4 years ago under follow up Rheumatology clinic HSJpANCA,cANCA negativeRF positive 2)Bilateral knee osteoarthritis3)Collapsed nasal bridge and sepstal perforation secondary to Wegener Granulomatosis4)Subglotic Stenosis under ENT follow up5)History of recent admission at Hosp Kulim for UTI

Past medication historyName/Dose/Strength/RouteSchedule/Frequency of UseIndicationDate StartDate StopT. Prednisolone 25mgODWegener Granulomatosis2009On-goingT. Rocalcitriol 0.25mcgODVitamin D analog2012On-goingT Calcium Carbonate 500mgODCalcium Supplement2012On-goingT Ranitidine 150mgBDStress Ulcer Prophylaxis2012On-goingT.Methotrexate 20mgPer weekWegner Granulomatosis2009Withold in the wardT.Folate 5mgPer weekSupplement for folate antagonist2009Withhold in the wardPatient claimed not taking any traditional medications or supplements

Patient scored 8 over 8 for the morisky medication adherence scale. Patient was well compliant to her medicationsPHYSICAL EXAMINATION/ LABORATORY FOR INITIAL AND FOLLOW UPPharmacologic Review of SystemGeneralAlert and conscious, rest in bed, no shortness of breath (SOB), no tachypneicVSBP = 114/77 mmHg, PR = 90/min, T = 37.0C, SpO2 = 98% under room airNEURO/MENTAL GCS full (15/15)CVSDual Rhythm No Murmur (DRNM)CHESTB/L L2 crepts, no chest painHEENT No ulcerABDO Soft, non-tender, no abdominal painHEPATIC No hepatosplenomegalySKIN/MUSCLEMildly dehydratedBLOODWBC = 10.3 x 109/L, HB = 12.4 g/100mL, Platelet = 226.0 x 109/LGITPoor oral intake, no nausea and vomitingKUT-Vital signsHaematology : complete blood countDateNormal range11/4/1413/4/1416/4/1418/4/1419/4/1420/4/14WBC4.0-10.0 x103/L10.312.1 15.513.213.811.9RBC3.8-4.8 x106/L4.34.54.24.44.54.4HGB12 15 g/dL12.41312.21211.912.4HCT36 46 %3738.937.438.839.338.3MCV83 101 fL8886.488.287.686.487.2MCH27 32 pg28.828.828.828.828.828.8MCHC31.5 34.5g/dL33.534.43434.533.834Platelets150 410x103/L226.0340333292322321Neutrophils40 80 %67.482.663.565.46361.0Lymphocyte20 40 %25.811.129.825.426.528.6Monocyte3.4 9.0 %6.16.06.16.06.16.0Eosinophil0.0 7.0 %0.60.50.60.50.40.5Basophil0.0 1.5 %0.10.20.10.10.10.1Neutrophil 2 7x103/L6.9410.3 9.829.448.487.64Normal range 11/4C reactive protein0.2 g/dL

This assay involves measuring D-dimer, which is a fibrin specific degradation product that detects cross-linked fibrin resulting from endogenous fibrinolysis and hencecan caused deep vein thrombosis (DVT).thus, elevated levels occur in the presence of a recent thrombusRenal Profile

DateNormal range11/4/1413/4/14Na+136 145 mmol/L139137K+3.5 5.0 mmol/L2.83.6Urea2.5 6.7 mmol/L2.72.3Cl-98 107 mmol/L96102Clcr50 110 ml/min5446Creat53-115 mol/LLiver profileDateNormal range11/4/14Total Protein66-87 g/L66Albumin35-50 g/L27 Total Bilirubin3-21 mol/L9ALT0-55 U/L37ALP40 - 150 U/L150Basically low albumin chronic inflammation of the patient had wegener granulomatosis. And arthritisOther electrolyteDateNormal range11/4/14Ca2.23-2.5mmol/L2.22Corr. Ca2.42PO40.74-1.52mmol/L1.15Mg0.7-1.3mmol/L0.81U/S Droppler left Lower Limb done on 13/4/14

Echogenic thrombus noted within left common femoral vein and left proximal area distal superficial femoral vein. Patchy colour Droppler and spectral wavefront demonstrated.

From this information this patient already has Deep Vein Thrombosis of left common femoral vein and left superficial femoral vein.

Coagulation Profile

DateNormal range14/416/418/419/420/421/4PT11.9-14.014.317.817.317.920.822.2INR2.0-3.01.11.51.41.51.82.0APTT31.4-45.832.932.732.636.840.739.6Coagulation profile was taken on 14/4 till 20/4 show that this patient has lower INR, Target range for patient who has DVT is 2 until 3. INR is important because its tell how much is needed for blood to clotting. After doctor increase dose of warfarin INR of this patient achieve target range.Blood Glucose Levels

DateNormal range11/412/413/414/4RBS4-11mmol/L6.5/6.5.6.86.8/6.7/7.47.86.5FBG0.2 g/dL and show a positive resultThis assay involves measuring D-dimer, which is a fibrin specific degradation product that detects cross-linked fibrin resulting from endogenous fibrinolysis and hencecan caused deep vein thrombosis (DVT).thus, elevated levels occur in the presence of a recent thrombusSuggested doctor to start S/C Clexane 60mg BDOUTCOMEAccepted. Drug therapy problem list (dtpl)14/4/14In appropriate drug as the pottasium level is back to normal rangeThe potassium level is 3.6 mmol/L already in normal range (3.5-5 mmol/L)Suggested doctor to stop T slow kOUTCOMEAccepted14/4/14 Inappropriate monitoringPatient was diagnosed with Cushing disease secondary to long term used of prednisolone but cortisol level never been taken before she admitted to the ward.This exogenous Cushing syndrome occurs when the dose of exogenous glucocorticoid exceeds the physiologic needs. This supraphysiologic dosing also leads to suppression of endogenous cortisol production. The suppression occurs at the level of the hypothalamus and pituitary gland, with subsequent atrophy of the part of the adrenal cortex that produces endogenous glucocorticoidsSuggest to doctor to monitor cortisol levelOUTCOMENot AcceptedQuery Diagnosis just physical appearance look like cushing syndrome ad outcome wrong diagnosisDrug therapy problem list (dtpl)Inadequate drug patient currently on long term MethotrexateWarfarin has drug drug interaction with MethotrexateMethotrexate may decrease the effect of warfarin that may cause decrease level of INR in this patient.Suggest to doctor if on back Methotexate dose of warfarin should be increasedOUTCOMENot AcceptedPHARMACISTS CARE PLAN MONITORING WORKSHEET (PMWPharmacotherapeuticGoalMonitoring ParameterDesiredEndpointMonitoringFrequencyTo achieve normal potassium levelPottasium level (Renal profile) (3.5-5.0 mmol/L)Arterial blood gas every hourly when respiratory acidosisthen daily after respiratory acidosis resolvedTo achieve desired coagulation profile since patient on T.WarfarinCoagulation profilePT 11.9-14.0APTT 31.4 - 45.8INR 2-3Coagulation profile dailyDischarge MedicationT. Methotrexate20mg weeklyT.Ranitidine 150mg BDT. Warfarin 5.5mg ODT.Prednisolone 20mg ODC. Rocalcitriol 0.25mcgT Calcium Carbonate 500mg ODCommuncationCounselling was given to patient regardingIndication of warfarinINR monitoring (target INR level 2-3)Side effect of warfarinDrug and food interaction, Drug-Drug interactionLifestyle modificationAdvice patient to seek Drs Attention if abnormal bleeding sign occursSupplied warfarin booklet and issued CP4 for MTAC warfarin pharmacist follow upTime to toke Warfarin