WEGENER

GRANULOMATOSI
S

M U H A M A D S YA H I L M I

WHAT IS WEGENER GRANULOMATOSIS

Wegeners 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
tissue
It 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.

PATHOGENESIS
The cause of WG is unknown

CLINICAL FEATURE
WG 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
Eye
Orbital pseudotumor, conjunctivitis, keratitis (risk of corneal melt), anterior uveitis,
scleritis, episcleritis
Lung
Nodules, infiltrates, cavitary lesions, alveolar hemorrhage, bronchial lesions
Heart
Occasional valvular lesions, pericarditis
Kidney
Segmental necrotizing glomerulonephritis, renal failure requiring hemodialysis
Skin
Vesicular, palpable purpuric, ulcerative, or hemorrhagic lesions

TREATMENT FOR WEGENER GRANULOMATOSIS

CYCLOPHOSPHAMIDE
The typical daily cyclophosphamide dose is
2 mg/kg/day, and should generally not exceed
200 mg/day.
Dose adjustments are critical for patients with older age (e.g.
greater than
70 years), renal dysfunction

METHOTREXATE
Methotrexate at a starting dose of 0.25 mg/kg/week (typically
1520 mg/week), increased to a maximum of 25 mg/week

GLUCOCORTICOID
dose of prednisone in the range of 0.51.0 mg/kg (up to a
maximum of 80 mg/day)

OBJECTIVE

To Discuss Management approach of

Wegener Granulomatosis
To discuss about the drug related problem and
pharmaceutical care issue of this case

PATIENTS DEMOGRAPHICS
Name

Reg. No.

567112

Age

25

Gender

Female

Race

India

Weight

58kg

Height

150cm

DOA

11/4/14

Allergy

Allergy tp penicilin group and augmentin

Group

Duration of hospitalization

10 days

CHIEF COMPLAINT
Unresolved Painful swelling at ankle Joint

HISTORY OF PRESENTING ILLNESS

Worsening bilateral lower limb edema 2/12,Painful and
proggresively worsening complain of pain on movement
Difficulty in ambulating 2/12
Fever ,SOB and dry cough for 2/7
Loss of appetite 3/7

FAMILY AND SOCIAL HISTORY

Single stay at an orphanage home
Support by herself by working as waiter
Mother had diabetes mellitus and thyroid problem
No smoking or alcohol intake

PAST MEDICAL HISTORY

1)Wegener Granulomatosis
Diagnosed 4 years ago under follow up Rheumatology clinic HSJ
pANCA,cANCA negative
RF positive
2)Bilateral knee osteoarthritis
3)Collapsed nasal bridge and sepstal perforation secondary to
Wegener Granulomatosis
4)Subglotic Stenosis under ENT follow up
5)History of recent admission at Hosp Kulim for UTI

PAST MEDICATION HISTORY

Name/Dose/Strength/Route

Schedule/
Frequency of Use

Indication

Date Start

Date Stop

T. Prednisolone 25mg

OD

Wegener Granulomatosis

2009

On-going

T. Rocalcitriol 0.25mcg

OD

Vitamin D analog

2012

On-going

T Calcium Carbonate 500mg

OD

Calcium Supplement

2012

On-going

T Ranitidine 150mg

BD

Stress Ulcer Prophylaxis

2012

On-going

T.Methotrexate 20mg

Per week

Wegner Granulomatosis

2009

T.Folate 5mg

Per week

Supplement for folate

antagonist

2009

Patient claimed not taking any traditional medications or supplements

Withold in the
ward

Withhold in the
ward

Patient scored 8 over 8 for the morisky medication adherence scale. Patient was well
compliant to her medications

PHYSICAL EXAMINATION/ LABORATORY FOR INITIAL

AND FOLLOW UP
Date
Height (cm)
Weight (kg)
Temp (C)
BP (mmHg)
Pulse (bpm)
RR (bpm)
Peak Flow
pH
Osat (%)
pCO2 (mmHg)
HCO3- (mmHg)
LDL (mmol/L)
HDL (mmol/L)
TG (mmol/L)
TC (mmol/L)
WBC (x109/L)
Hgb (g/dL)
Plt (x109/L)
Chest X-ray
Echocardio

ECG

11/4/14
150
58
37
114/76
90
98
10.3
12.4
226
-

Sinus Rhythm

Date
Na+ (mmol/L)
K+ (mmol/L)
BUN (mmol/L)
SrCr (mol/L)
Urine output

11/4/14
129
2.8
2.7
54
350

I/O
2+
Mg2+
(mmol/L)
2+
Ca2+ (mmol/L)
PO4(mmol/L)
4RBS (mmol/L)
BMI (kg/m2)
LDH (U/L)
CPK (U/L)
INR
PT (sec)
APTT (sec)
TT/FDP
T. Bili (mol/L)
ALT (U/L)
AST (U/L)
ALP (U/L)
T. Protein (g/L)
Alb (g/L)
TSH (mIU/L)
CrCl (ml/min)

0.81
[2.42]
1.15
12.6
396
50
9
37
18
150
66
29
128.91

Pharmacologic Review of System

General

Alert and conscious, rest in bed, no shortness of breath (SOB),

no tachypneic

VS

BP = 114/77 mmHg, PR = 90/min, T = 37.0C, SpO2 = 98%

under room air

NEURO/MENTAL

GCS full (15/15)

CVS

Dual Rhythm No Murmur (DRNM)

CHEST

B/L L2 crepts, no chest pain

HEENT

No ulcer

ABDO

Soft, non-tender, no abdominal pain

HEPATIC

No hepatosplenomegaly

SKIN/MUSCLE

Mildly dehydrated

BLOOD

WBC = 10.3 x 109/L, HB = 12.4 g/100mL, Platelet = 226.0 x

109/L

GIT

Poor oral intake, no nausea and vomiting

KUT

VITAL SIGNS
Date

11/4/14

12/4/1
4

13/4/
14

14/3/
14

15/4/
14

16/4/
14

17/4/
14

18/4/
14

19/4/
14

20/4/
14

21/4/
14

T (oC)

37

37

37

37

37

37

37

37

37

37

37

90

82

84

85

74

90

86

92

84

88

82

BP
(mmHg)
HR
(beat/min)
Input/

Output

Fluid
balance

350

100

400

300

200

-100

100

300

150

650

HAEMATOLOGY : COMPLETE BLOOD COUNT

16/4/14

18/4/1
4

19/4/14

20/4/14

15.5

13.2

13.8

11.9

4.5

4.2

4.4

4.5

4.4

12.4

13

12.2

12

11.9

12.4

36 46 %

37

38.9

37.4

38.8

39.3

38.3

MCV

83 101 fL

88

86.4

88.2

87.6

86.4

87.2

MCH

27 32 pg

28.8

28.8

28.8

28.8

28.8

28.8

31.5 34.5g/dL

33.5

34.4

34

34.5

33.8

34

150 410x103/L

226.0

340

333

292

322

321

Neutrophils

40 80 %

67.4

82.6

63.5

65.4

63

61.0

Lymphocyte

20 40 %

25.8

11.1

29.8

25.4

26.5

28.6

Monocyte

3.4 9.0 %

6.1

6.0

6.1

6.0

6.1

6.0

Eosinophil

0.0 7.0 %

0.6

0.5

0.6

0.5

0.4

0.5

Basophil

0.0 1.5 %

0.1

0.2

0.1

0.1

0.1

0.1

2 7x103/L

6.94

10.3

9.82

9.44

8.48

7.64

Date

Normal range

11/4/14

WBC

4.0-10.0 x103/L

10.3

RBC

3.8-4.8 x106/L

4.3

HGB

12 15 g/dL

HCT

MCHC
Platelets

Neutrophil

13/4/14
12.1

Normal range

11/4

C reactive protein

<5

54.1

Erythrocyte
Sedimentation rate

2-7

38

D-Dimer Latex

>0.2 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
thrombosis (DVT).

thus, elevated levels occur in the presence of

a recent thrombus

deep vein

RENAL PROFILE

Date

Normal range

11/4/14

13/4/14

Na+

136 145 mmol/L

139

137

K+

3.5 5.0 mmol/L

2.8

3.6

Urea

2.5 6.7 mmol/L

2.7

2.3

96

102

54

46

Cl-

98 107

Clcr

50 110 ml/min

Creat

mmol/L

53-115 mol/L

LIVER PROFILE

Date

Normal range

11/4/14

Total Protein

66-87 g/L

66

Albumin

35-50 g/L

27

3-21 mol/L

ALT

0-55 U/L

37

ALP

40 - 150 U/L

150

Total Bilirubin

Basically low albumin chronic

inflammation of the patient had wegener
granulomatosis. And arthritis

OTHER ELECTROLYTE

Date

Normal range

11/4/14

Ca

2.23-2.5mmol/L

2.22

Corr. Ca

2.42

PO4

0.74-1.52mmol/L

1.15

Mg

0.7-1.3mmol/L

0.81

U/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

Date

Normal range

14/4

16/4

18/4

19/4

20/4

21/4

PT

11.9-14.0

14.3

17.8

17.3

17.9

20.8

22.2

INR

2.0-3.0

1.1

1.5

1.4

1.5

1.8

2.0

31.4-45.8

32.9

32.7

32.6

36.8

40.7

39.6

APTT

Coagulation 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

Date

Normal range

11/4

12/4

13/4

14/4

RBS

4-11mmol/L

6.5/6.5.6.
8

6.8/6.7/7.4

7.8

6.5

FBG

<5.5mmol/L

Microbiology Results

Date of
sampling

Source

Result

11/4

Blood

No growth

11/4

Sputum

No growth

11/4

Urine

Grossly mixed 2 types gram rod

18/4

Urine

No significant Bacteriurea

UFEME

Date
Physical /Macroscopic colour
Transparency (Urine)
Sp Gravity
Reaction pH
Protein
Glucose
Bilirubin
Blood
Leucocytes
Nitrite
Urobilinogen
Ketone
RBC
WBC
Epithelial cells
Cast
Bacteria

11/4
Light Yellow
clear
1.019
5.0
Negative
Negative
Negative
Negative
25.0/uL
Negative
Normal
Negative
39.4/uL
52.0/uL
4.0/uL
3.27/uL
2482/uL

DIAGNOSIS
Bilateral pedal edema for inspection (11/4)
Recurrent UTI symptom despite on antibiotic(11/4)
U/L Wegener Granulomatosis and cushing syndrome secondary
to long term corticosteroid(11/4)
Left LL Deep Vein Thrombosis (13/4)

DRUG TREATMENT IN THE WARD

Current Drug Therapy (Oral, Parental, Inhaler and others)
Drug
Prescribe Start
Stop
Name/Dose/Strength/Route Schedule
Date
Date
1.
T Prednisolone 25mg
OD
11/4/14
On
going
2.

T.Rocalcitriol 0.25mcg OD

11/4/14

3.

11/4/14

4.

T Calcium Carbonate OD
500mg
T.Ranitidine 150 mg
BD

5.
6.

IV Lasix
T Lasix 40 mg

11/4//14
12/4/14

BD
BD

11/4/14

On
going
On
going
On
Going
11/4/14
14/4/14

Indication/safety/efficacy
Immunosuppresing
agent for treatment of
wegener
Vitamin D Analog
Calcium Supplement
Stress Ulcer Prophylaxis
To treat edema
To treat edema

DRUG TREATMENT IN THE WARD

7.

T. Cefuroxime 250mg

BD

12/4/14

18/4/14

8.
8.
9.
10.
10.

Mist
Mist KCL
KCL 10ml
10ml
T. Slow K
S/C Clexane 60mg
S/C Clexane 60mg

TDS
TDS
OD
OD

11/4/14
11/4/14
11/4/14
11/4/14

11/4/14
11/4/14
14/4/14
14/4/14

BD
BD

12/4/14
12/4/14

21/3/14
21/3/14

11.
11.

T
T warfarin
warfarin 5mg
5mg

OD
OD

13/4/14
13/4/14

16/4/14
16/4/14

12.
12.

T.
T. Warfarin
Warfarin 5.5
5.5 mg
mg

BD
BD

4/3/14
4/3/14

OnOngoing
going

To cover urinary tract

infection
For
For hypokalemia
hypokalemia
To
correct
To correct hypokalemia
hypokalemia
To treat
To treat
Thrombosis
Thrombosis
To
To treat
treat
thrombosis
thrombosis
To
To treat
treat
thrombosis
thrombosis

deep
deep

vein
vein

deep
deep

vein
vein

deep
deep

vein
vein

DAILY PROGRESS
DAY
DAY 11 (11/4/14)
(11/4/14)
Alert,
conscious,
no
SOB,
Alert, conscious, no SOB, not
not tachypneic,
tachypneic, no
no
nausea
or
vomiting
nausea or vomiting
Lungs:
Lungs: Clear
Clear
1.
1.
2.
2.
3.
3.

4.
4.
5.
5.

Plan
Plan
For
Echo
Inpatient
For Echo Inpatient
Not
Not for
for antibiotic
antibiotic yet
yet to
to observer
observer
temperature
first
temperature first
Continue
Continue patient
patient old
old Medication
Medication
T.Prednisolone
20mg
T.Prednisolone 20mg OD
OD
T.
Rocalcitriol
0.25mcg
T. Rocalcitriol 0.25mcg OD
OD
T.Calcium
Carbonate
500mg
T.Calcium Carbonate 500mg OD
OD
T.
T. Ranitidine
Ranitidine 150mg
150mg BD
BD
Start
Start IV
IV LasIX
LasIX 40mg
40mg BD
BD
Start
Slow
K
11/11
OD
Start Slow K 11/11 OD

1.
2.
3.
4.
5.
6.
7.

DAY 2 (12/4/14)
Alert, conscious, no SOB, not tachypneic
Lungs: Clear
Plan
For Echo Inpatient
Off iv Lasix and started oral Lasix 40mg OD
Cont Slow K
Continue patient old Medication
Started T Cefuroxime 250mg BD
Started S/C Clexane 60mg BD if D
Dimer positive
For US Doppler if D Dimer Possitive

DAY 3 (13/4/14)
Alert, conscious, no SOB, not tachypneic
Lungs: Clear
Plan
1.
For Echo Inpatient CM
2.
COnt oral Lasix 40mg OD
3.
Cont Slow K
4.
Continue patient old Medication
5.
COnt Cefuroxime 250mg BD
6.
S/C Clexane 60mg BD
7.
Started T Warfarin 5mg OD

DAY 4(14/4/14)
Alert, conscious, no SOB, not tachypneic
Lungs: Clear
Plan
1.
For Echo today
2.
Off oral Lasix 40mg OD
3.
Off Slow K
4.
Continue patient old Medication
5.
Started T Cefuroxime 250mg BD
6.
Started S/C Clexane 60mg BD if
D Dimer positive
7.
For US Doppler if D Dimer
Possitive

DAY 7(17/4/14)
Alert, conscious, no SOB, not tachypneic
Lungs: Clear
Plan
1.
Continue Patient Old
Medication
2.
COnt Cefuroxime 250mg BD
3.
S/C Clexane 60mg BD
4.
Increase dose of Warfarin to 5.5mg
OD
5.
Watchout Bleeding tendency

DAY 5 (15/4/14)
Alert, conscious, no SOB, not tachypneic
Lungs: Clear
Plan
1.
Continue Patient Old
Medication
2.
COnt Cefuroxime 250mg BD
3.
S/C Clexane 60mg BD
4.
Cont T Warfarin 5mg OD
5.
Watchout Bleeding tendency
6.
For Echo Last planned by
rheumatologist

DAY 8(17/4/14)
Alert, conscious, no SOB, not tachypneic
Lungs: Clear
Plan
1.
Continue Patient Old
Medication
2.
TO complete Cefuroxime
250mg BD 1/52
3.
S/C Clexane 60mg BD
4.
Cont T.Warfarin 5.5mg OD
5.
Watchout Bleeding tendency S/C
Clexane 60mg BD
6.
T?O room once bed available

DAY 6 (16/4/14)
Alert, conscious, no SOB, not tachypneic
Lungs: Clear
Plan
1.
Continue Patient Old
Medication
2.
COnt Cefuroxime 250mg BD
3.
S/C Clexane 60mg BD
4.
Increase dose of Warfarin to 5.5mg
OD
5.
Watchout Bleeding tendency

DAY 9(17/4/14)
Alert, conscious, no SOB, not tachypneic
Lungs: Clear
Plan
1.
Continue Patient Old
Medication
2.
Off Oral Cefuroxime 250mg
BD
3.
S/C Clexane 60mg BD
4.
Cont dose of Warfarin 5.5mg OD
5.
Watchout Bleeding tendency S/C
Clexane 60mg BD
6.
Insert CBD (to determine whether
hematuria or menses)
COnt Monitor INR

DAY 10(20/4/14)
Alert, conscious, no SOB, not tachypneic
Lungs: Clear
Plan
1.
Continue Patient Old
Medication
2.
S/C Clexane 60mg BD
3.
Cont dose of Warfarin 5.5mg OD
4.
Watchout Bleeding tendency S/C
Clexane 60mg BD
5.
COnt Monitor INR

DAY 11(21/4/14)
Alert, conscious, no SOB, not tachypneic
Lungs: Clear
Plan
1.
Continue Patient Old
Medication
2.
Off S/C Clexane 60mg BD
3.
Cont T Warfarin 5.5 mg OD
4.
Watchout Bleeding tendency
5.
Discharge today INR achive 2-3

DRUG THERAPY PROBLEM LIST (DTPL)

Date
11/4/14

1.

DRP(medication related)
Inappropriate drug

Recommendation
Suggested doctor to give T.Slow K OD for

Potassium level is 2.8mmol/L, patient having hypokalemia

Potassium level low because of loop diuretic, long term of the taste

3 day
Patient refuse to take Mist Kcl Because of

prednisolone and Cushing syndrome

Kdeficit (mmol) = (Knormal lower limit - Kmeasured) x kg

body weight x 0.4

11/4/14
11/4/14

16.3mmol = ( 3.5-2.8) x 58x 0.4

1 tablet of slow k contain 8mmol

2.
2.

Polypharmacy of given T.Slow K IIII OD and Mist KCL

Polypharmacy of given T.Slow K IIII OD and Mist KCL
15ml TDS
15ml TDS

OUTCOME
Patient was prescribed with T.Slow K OD

Suggested doctor to off Mist KCl and

Suggested doctor to off Mist KCl and
Continue tablet Slow K
Continue tablet Slow K
OUTCOME
OUTCOME
Accepted.
Accepted.

DRUG THERAPY PROBLEM LIST (DTPL)

Date
12/4/14

DRP(medication related)
3.

Inappropriate drug of using Heparin and Clexane together

Recommendation
Suggested doctor to stop Heparin and continue
clexane

LMWH and heparin are both used to prevent blood from clotting
inside the body, but are used in different situations.

LMWH produces a more predictable anticoagulant response so

frequent monitoring is not needed to adjust the dose.

OUTCOME
Accepted

LMWHs have the advantage of a longer plasma half-life and a more

predictable dose response than UFH

In addition, heparin has potential to cause a possibly deadly

condition known as Heparin Induced Thrombocytopenia (HIT),
where the body stops producing blood platelets as compared to
clexane which has lower adverse effect of HIT.

12/4/14

4.

Inappropriate Drug when D-Dimer latex >0.2 g/dL and show a Suggested doctor to start S/C Clexane 60mg BD
positive result

OUTCOME
This assay involves measuring D-dimer, which is a fibrin Accepted.
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 thrombus

DRUG THERAPY PROBLEM LIST (DTPL)

14/4/14

5.

In appropriate drug as the pottasium level is back to normal Suggested doctor to stop T slow k
range

OUTCOME
The potassium level is 3.6 mmol/L already in normal range

Accepted

(3.5-5 mmol/L)

14/4/14

6.

Inappropriate monitoring

Patient 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 glucocorticoids

Suggest to doctor to monitor cortisol level

OUTCOME
Not Accepted
Query Diagnosis just physical appearance look
like cushing syndrome ad outcome wrong
diagnosis

DRUG THERAPY PROBLEM LIST (DTPL)

7.

Inadequate

drug

patient

currently

on

long

term Suggest to doctor if on back Methotexate

Methotrexate

Warfarin has drug drug interaction with Methotrexate

Methotrexate may decrease the effect of warfarin that may

cause decrease level of INR in this patient.

dose of warfarin should be increased

OUTCOME
Not Accepted

PHARMACISTS CARE PLAN MONITORING WORKSHEET (PMW

Pharmacotherapeutic
Goal
To achieve normal potassium
level

Monitoring Parameter
Pottasium level (Renal profile)

Desired
Endpoint
(3.5-5.0 mmol/L)

Monitoring
Frequency
Arterial blood gas every hourly
when respiratory acidosis
then daily after respiratory
acidosis resolved

To achieve desired coagulation

profile since patient on
T.Warfarin

Coagulation profile

PT 11.9-14.0
APTT 31.4 - 45.8
INR 2-3

Coagulation profile daily

DISCHARGE MEDICATION
T. Methotrexate20mg weekly
T.Ranitidine 150mg BD
T. Warfarin 5.5mg OD
T.Prednisolone 20mg OD
C. Rocalcitriol 0.25mcg
T Calcium Carbonate 500mg OD

COMMUNCATION
Counselling was given to patient regarding

Indication of warfarin

INR monitoring (target INR level 2-3)

Side effect of warfarin

Drug and food interaction, Drug-Drug interaction

Lifestyle modification

Advice patient to seek Drs Attention if abnormal bleeding

sign occurs

Supplied warfarin booklet and issued CP4 for MTAC warfarin

pharmacist follow up

Time to toke Warfarin