This case study describes a 37-year-old female patient who presented with fever, cough, and respiratory distress. Imaging showed multiple lung abscesses. She did not respond to antibiotic treatment and her condition deteriorated, developing skin lesions. A rheumatology consultation determined she had granulomatosis with polyangiitis (GPA) based on biopsy results. She received high-dose steroids and cyclophosphamide, with improvement. However, she developed polyuria after cyclophosphamide and bleeding after further treatment, which were complications of her GPA involving other organs. Her condition continued improving with conservative management.

1. Case Study
Samar Tharwat Radwan
Lecturer of Internal Medicine
Rheumatology & Immunology Unit
Musculoskeletal Ultrasound –EULAR
Mansoura University

2. On 29th July ,2020
A 37-year-old female patient presented to the emergency room with :
• High grade fever associated with
• Cough
• Mucoid expectoration

3. On Examination
• She was febrile (Temp :39°C)
• In respiratory distress (SaO2 :88 %)
• Auscultation revealed few crackles at the chest

4. Investigations
• WBCs: 26.7 × 109/L
• Neutrophils : 23.7 × 109/L(88.7)
• Lymphocytes : 2.7 × 109/L (10.2 %)
• Hemoglobin : 8.7 g/dL
• Platelets : 576 × 109/L

5. HRCT Chest
Multiple lung abscesses seen at both upper lung
lobes ,the largest diameter measuring about 5 cm
?Bacterial or fungal infection

6. The patient was admitted to Chest Department

7. Work Up
• Blood and sputum :culture ,sensitivity, gram stain
• Tuberculin test
• ACE level
• COVID 19 PCR swab test
• Echocardiography
• Abdominal US
• Procalcitonin
All were negative

8. She was received treatment in the form of
• Cilastatin & Imipenem (Tienam ): 500 mg /6 hrs
• Levofloxacin (Tavanic ): 750 mg /24 hrs
• Fluconazole

9. However, after 1 week of admission
General condition :deteriorated

10. Rheumatology Consultation was needed
Other manifestations have been appeared

11. Rheumatological evaluation
Generally unwell
High fever (40°C)
Blood pressure 100/70
Dyspnea

12. Rheumatological evaluation
Scattered multiple nodules and ulcers

13. Rheumatological evaluation
Blisters with associated necrosis of the skin

14. Rheumatological evaluation
Lower limb edema

15. Rheumatological evaluation
Hemorrhagic bullae

16. Rheumatological evaluation
• Multiple oral ulces

17. History
• Recurrent otitis media
(discharging pus 3 months)…Severe hearing loss
• Multiple small nodules at the nose.. Biopsy
• Hoarseness of voice ..Laryngoscopy ..multiple
nodules

18. Investigations
• WBCs:35
• Neutrophils : 80%
• CRP:170
• ESR :180
• Virology :negative
• Cr: 0.5 mg/dl
• ALT:14 IU/L
• AST: 21 IU/L
• Bilirubin:0.34
• Protein /creatinine ratio:0.33
mg/mg

19. Autoimmune Profile
• ANA
• Anti-dsDNA
• P-ANCA
• C-ANCA
• C3
• C4
• Anti Ro
• Anti La
All were normal

20. Nasal mucosa biopsy

21. Granulomatosis with Polyangiitis
With
Upper Respiratory tract
Against
Kidney
ANCA
Biopsy
Lung abscesses

22. Abscess in GPA
WBCs
(× 109/l)
Treatment
17.9 Aspiration, Antibiotics,prednisolone And
Cyclophosphamide
23 Prednisone 1mg/Kg /Pulse Cyclophosphamide (1
G/Monthly)
Mycophenolate Mofetil And Corticosteroids
12.7 Antibiotics, Corticosteroids And Cyclophosphamide
13.2 Cyclophosphamide, Prednisolone, And Cotrimoxazole

23. InflammationInfection

24. For Skin Biopsy (7 days)

25. Broad spectrum antbiotics
+
High Dose Steroids (60 mg )
Strict follow up

26. 2 Days Follow Up
35.5
36
36.5
37
37.5
38
38.5
39
39.5
40
40.5
6 hrs 12 hrs 24 hrs 30 hrs 36 hrs 42 hrs 48 hrs
Temperature
Temperature °C
SaO : 84-88 %
WBCs:38-37-38 × 109/l

27. 0.5 gm Solu medrol for
5 days and then 60 mg

28. Follow up data
Day 1 Day 3 Day 5 Day 7 Day 9 Day 11 Day 13 Day 15
General Wellbeing
ImprovingVoice
Hearing
Temperature 37.5 37 38 37.2 37.8 37 37 36.9
SaO2 88 90 90 92 96 96 96 97
WBCs 33 30 21.3 24.7 28.6 26.5 26 28.2
Platelets 655 314 139 615 763 108 215 693
HGB (g/dl) 8.5 9.4 10.5 10.6 9.11 8.9 8 8.5
ESR 170 100 90 50 85 30 40 30
CRP 170 110 100 90 57 55 30 28

29. High Resolution CT Chest
Regression of lung lesions

30. Result of skin biopsy

31. 30 days at the hospital
From first presentation
Ready for discharge

32. Another immunosuppression need to be added
0.5 gm Cyclophosphamide
(WBCs:28.000)
• In 500 cc normal saline over 2 hrs ..
• Preceded by 500 cc normal saline
• Followed by 500 cc normal saline

33. Full urinary bag :2000 ml
0.5 hr after Cyclophosphamide infusion

34. Polyuria
• 10 litres / 24 hrs
• Blood pressure :70/50
• Orthostatic hypotension
• Pulse :110 /minute
• CVP :8 (0-14)

35. Polyuria in GPA
Pituitary involvement

36. Polyuria after Cyclophosphamide

37. Workup for Polyuria
• Serum osmolarity
• Urine osmolarity
• Blood glucose
• Serum calcium
• Serum K
• Serum Mg
• Urine analysis
• MRI Brain

38. No abnormalities detected
??
Explanation :May be granulomatous inflammation at the kidney

39. 4 Days after Cyclophosphamide Infusion
• Massive attack of bleeding per rectum
• HGB decreased:8.6-4 within hours

40. Digestive Hemorrhage
• Vasculitis
ulcerations of ischemic origin
small intestine
submucous microaneurysmal ruptures
• Stress Ulcers
• Treatment
Endoscopic explorations:
informative
risk of perforations

41. GIT endoscopy
• Upper :normal
• Lower :erythematous ,edematous and ulcerated mucosa
• Pathological examination was suggestive of vasculitis

43. Gastrointestinal involvement in GPA is rare
Total of 21 patients developed gastrointestinal symptoms after immunosuppressive therapy

44. After 10 days of conservative Therapy
• Bleeding diminished
• Urine output decreased 2500 ml per day

45. Shift to Methotexate (2 months )
12.5 mg SC weekly

46. The patient is doing very well