Wegner's granulomatosis with atypical presentation

2. 
Case Presentation
By
Dr Hania Afzal
R3 internal Medicine

3. 
 Biodata
 Name: Waseem
 Age: 27y/o
 Occupation: technician in PAF
 Education: BS Mathematics
 Resident: Sargodha

4. 
 Presenting Complain:
 Skin rash ….. 5 years
 Joint Pain …. 1 year
 Cough ……. 4 weeks
 Hemoptysis .. 1 week

5. 
 HOPC
According to my patient, he was in his usual state of health 5 years
back when he started developing wounds/ rash even after minor
scratch or itch on skin (more on lower limbs). But it was not
hindering him from routine chores and was not associated with
fever or any other complain. But 1 year ago he developed joint
pains and swellings which shifted from 1 joint to other, each joined
remained involved for 1 to 2 days and 2 to 3 joints remained
involved at a time. Large joints were involved more especially hip
and knee joints. Meanwhile his skin wounds changed into rash
involving bilateral legs below knee. Rash was pruritic in nature and
pus and blood oozed out of each lesion.

6. 
 HOPC
His rash and joint pains were associated with low grade fever,
undocumented weight loss and malaise, more at night time.
Patient was put on antipsychotics but it did not help. Later on
some GP prescribed him tab deltacortil 3x BD for 1 week,
then 2 x BD for next week. This treatment improved his joint
pains, he remained asymptomatic and gained weight in next 6
months but had mild joint pains occasionally associated with
taking protein rich diet. His rash started involving upper
limbs too then 5 months back he got skin biopsy which
revealed Reactive Perforating Collagenosis.

7. 
HOPC
Few weeks back he again developed similar joint pains,
associated with low grade fever, worsening rash (involving
thighs & buttocks) and malaise. He took some aurvedic
treatment for 3 weeks which initially improved his condition
but when he withdrew this medicine, his condition worsened
and he started developing dry cough which gradually
worsened and he noticed hemoptysis 1 week before
presentation. Blood was dark red to brown in color, ½ table
spoon in quantity early morning and mild bleed throughout
the day. He has lost 3 kg weight in 1 month during this
episode.

8. 
 Past Medical Hx
He has hx of some STD leading to genital ulcers 5 months
after marriage
 Past Surgical Hx
Not significant
 Drug Hx
Deltacortil, aurvedic medications at different intervals
 Vaccination Hx
 Fully vaccinated with EPI vaccines in childhood
 Received three doses of covid vaccine

9. 
 Family Hx
His mother has RA since young
His maternal uncles and aunts had pul TB. Last exposure was
in 2009
 Personal Hx
 Normal appetite
 normal sleep wake cycle
 Normal bowel habits
 Married for 6 years and has 2 alive, healthy children

10. 
 No hx of blood transfusions,
 No hx addictions
 He has history of dust allergy(sneezing and Upper
respiratory symptoms on dust exposure)
 Australian parrots as pets at home

11. 
 Travel Hx: Nil
 Socioeconomic Hx:
He lives in a joint family with his parents, a brother & his
family. He, his brother and their father are earners, have own
home in city with basic facilities available
 Systemic Inquiry:
Insignificant

12. 
 Clinical examination
A young male patient, average height and built sitting on
hospital bed, well oriented in time, place and person (GCS
15/15) with vitals of
 BP: 120/80 mmHg, Pulse: 80 /min, SaO2: 95% on air
RR: 10 /min, Temp: afebrile,
GPE

13. 
Jaundice: -ive
Palor: -ive
Clubbing: -ive
Koilonychia: -ive
Edema: -ive
Neck Swelling: -ive
Lymph Nodes: -ive

14. 
He has no joint swelling or tenderness at the moment
and has vesicular rash on lower limbs which is black in
color, painful, blood oozes out of active lesion and
healed lesions leave a black colored scar.
Skin and joints

15. 
Respiratory
He has bilateral clear chest with no added sounds
Abdomen
Soft, non tender
CVS
Audible 1st and 2nd heart sound
CNS
GCS 15/15
CNS,Abdomen & CVS

16. 
 Sarcoidosis
 Granulomatosis with polyangitis
 Eosinophilic granulomatosis
 Polyarteritis nodosa
DDs

17. 
 Hb: 13.4,
 TLC: 10.9 with 5% eosinophils
 Plt: 303
 ASO titres: negative
 LFTs, RFTs, S/E are normal
 Urine R/E normal
 Sputum for AFB & fungal hyphae negative
 Montoux test positive 16mm induration
 Gene xpert MTB trace detected
Investigations

18. 
 ESR 30
 CRP positive
 s.IgE: 228
 cANCA positive with 40 titres
 ANA, RA negative
Investigations

19. 

20. 

21. 

22. 

23. 
 Normal 2D Echo

24. 
 USG Abd + KUB unremarkable
 Bronchial washings were negative for AFB, revealed
gram negative rods (pseudomonas aeruginosa)
 Bronchial biopsy revealed medium vessel vasculitis

25. 

26. 
 ATT for latent TB
Myrin p fort 4x OD
Tab Vita 6 x OD
Modified d/t raised ALT to ethambutol, amikacin and moxifloxacin
 Tab Nuberol fort x SOS
 Cap Risek
 Tab Deltacortil
 Inj Ristova/rituximab is in plan after 3 months of completion of
ATT
Management

27. 
Granulomatosis with
polyangitis

28. 
ANCA associated
 vasculitis of small to medium vessels (arteries, arterioles,
capillaries)
 Necrotizing granulomatous lesion of upper and lower
respiratory tract
 Glomerulonephritis and
 Other organ manifestations
What it is…..

29. 
 With an incidence of
12 cases per million per year
 In 4th and 5th decades of life
 Affects both genders equally
 Prognosis
Generalized disease is inevitably fatal with less than 1 year
survival after diagnosis

30. 
Unknown etiology but possibly triggered by
 Environmental exposures including
 RTIs
 living in northern latitudes
 farming
 allergies
 exposure to solvents or silica
 Host factors including
 Genetic factors (α-1 antitrypsin, CTLA4, PTPN22*620W,
DPB1*0401, Fcϒ receptor IIIb on neutrophils and
macrophages)
 Cellular immune responses
Etiology

31. 
Develops over 4-12 months
 Upper respiratory tract symptoms (90% pts)
 Nasal congestion, crusting, ulceration, bleeding and
perforation of nasal septum may lead to ‘saddle nose
deformity’
 sinusitis
 otitis media
 mastoiditis
 Inflammation of gums
 Stridor due to subglottic stenosis
Features

32. 
 Lower respiratory tract symptoms (40-80%)
 cough
 dyspnea
 hemoptysis
 Migratory oligoarthritis (predilection for large joints)
 Ocular disease
 unilateral proptosis (orbital pseudotumor)
 Red eye (scleritis, episcleritis, ant uveitis, peripheral
ulcerative keratitis)
Features

33. 
 Skin disorder
 Purpura
 other lesions
 Dysesthesia
 Renal involvement (3/4th of cases)
subclinical until advanced disease
will develop in majority of untreated patients
 Fever, weight loss and malaise
 Newly acquired HTN (rarely)
 Venous thrombotic events
Features

34. 
 CBC
Anemia
Mild leukocytosis
 ESR, CRP
 ANA (0-15%)
 RF (50%)
 ANCA (93-96%)
PR3-ANCA/c-ANCA(>90% specificity, >95% sensitivity)
MPO-ANCA(10-25% pts)
 Urine RE
Proteinurea
red cells/red cell casts
Diagnosis

35. 
 HRCT chest
Infiltrates, nodules, masses, cavities
 CT PNS
extensive sinusitis
bony sinus erosion

36. 
 Biopsy/Histopath
 Lung biopsy
Vasculitis, granulomatous inflammation/granulomas,
geographic necrosis, acute and chronic inflammation
 Nasal biopsy
Chronic inflammation
 Kidney biopsy
Pauci-immune glomerulonephritis
segmental necrotizing glomerulonephritis with multiple
crescents(characteristic but not diagnostic

37. 
 Early treatment is crucial
 in preventing end-organ complications
 and preserving life
 Remission Induction
Depends on severity of disease
 Mild(no significant kidney dysfunction/immediately life threatening
disease)
 Severe(life or organ threatening disease/RPGN/pul hemorrhage)
 Remission Maintenance
Management

38. 
 Severe disease
 Corticosteroids plus Rituximab
375mg/m2 IV once a week for 4 weeks of Rituximab with 1mg/kg
PO prednisolone daily
 Coricosteroids plus Cyclophosphamide
 Remission induced in > 90% pts
 Prednisolone (1mg/kg/day P/O) plus cyclophosphamide
(2mg/kg/day P/O dose adjustment required for >70y/o and with
kidney disease).
 Intermittent high dose IV cyclophosphamide is associated with
higher relapse risk compared to daily oral
 To avoid toxicity give cyclophosphamide for 3-6 months
Remission Induction

39. 
 Non severe disease
Methotrexate plus glucocorticoids
Remission Induction

40. 
 Azathioprine 2mg/kg/day P/O
 Methotrexate 20-25mg/wk P/O or IM
 Rituximab (500mg IV repeated in 14 days then 6monthly)
 Mycophenolate mofetil
Rituximab is better in remission maintenance and overall
survival than other drugs
One trial showed that relapse after 28months was 5% with
rituximab and 29% with azathioprine
Remission Maintenance

41. 
 Relapse with severe disease manifestation
o Who are not receiving Rituximab for maintenance give
Rituximab
o Who receive rituximab switch to cyclophosphamide rather
than giving additional dose
Relapse management

42. 
 Switch to other therapy rather than combining both
 Add IVIG to current therapy
Refractory disease

43. 