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Case Study
Samar Tharwat Radwan
Lecturer of Internal Medicine
Rheumatology & Immunology Unit
Musculoskeletal Ultrasound –EULAR
Mansoura University
On 29th July ,2020
A 37-year-old female patient presented to the emergency room with :
• High grade fever associated with
• Cough
• Mucoid expectoration
On Examination
• She was febrile (Temp :39°C)
• In respiratory distress (SaO2 :88 %)
• Auscultation revealed few crackles at the chest
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
HRCT Chest
Multiple lung abscesses seen at both upper lung
lobes ,the largest diameter measuring about 5 cm
?Bacterial or fungal infection
The patient was admitted to Chest Department
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
She was received treatment in the form of
• Cilastatin & Imipenem (Tienam ): 500 mg /6 hrs
• Levofloxacin (Tavanic ): 750 mg /24 hrs
• Fluconazole
However, after 1 week of admission
General condition :deteriorated
Rheumatology Consultation was needed
Other manifestations have been appeared
Rheumatological evaluation
Generally unwell
High fever (40°C)
Blood pressure 100/70
Dyspnea
Rheumatological evaluation
Scattered multiple nodules and ulcers
Rheumatological evaluation
Blisters with associated necrosis of the skin
Rheumatological evaluation
Lower limb edema
Rheumatological evaluation
Hemorrhagic bullae
Rheumatological evaluation
• Multiple oral ulces
History
• Recurrent otitis media
(discharging pus 3 months)…Severe hearing loss
• Multiple small nodules at the nose.. Biopsy
• Hoarseness of voice ..Laryngoscopy ..multiple
nodules
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
Autoimmune Profile
• ANA
• Anti-dsDNA
• P-ANCA
• C-ANCA
• C3
• C4
• Anti Ro
• Anti La
All were normal
Nasal mucosa biopsy
Granulomatosis with Polyangiitis
With
Upper Respiratory tract
Against
Kidney
ANCA
Biopsy
Lung abscesses
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
InflammationInfection
For Skin Biopsy (7 days)
Broad spectrum antbiotics
+
High Dose Steroids (60 mg )
Strict follow up
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
0.5 gm Solu medrol for
5 days and then 60 mg
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
High Resolution CT Chest
Regression of lung lesions
Result of skin biopsy
30 days at the hospital
From first presentation
Ready for discharge
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
Full urinary bag :2000 ml
0.5 hr after Cyclophosphamide infusion
Polyuria
• 10 litres / 24 hrs
• Blood pressure :70/50
• Orthostatic hypotension
• Pulse :110 /minute
• CVP :8 (0-14)
Polyuria in GPA
Pituitary involvement
Polyuria after Cyclophosphamide
Workup for Polyuria
• Serum osmolarity
• Urine osmolarity
• Blood glucose
• Serum calcium
• Serum K
• Serum Mg
• Urine analysis
• MRI Brain
No abnormalities detected
??
Explanation :May be granulomatous inflammation at the kidney
4 Days after Cyclophosphamide Infusion
• Massive attack of bleeding per rectum
• HGB decreased:8.6-4 within hours
Digestive Hemorrhage
• Vasculitis
ulcerations of ischemic origin
small intestine
submucous microaneurysmal ruptures
• Stress Ulcers
• Treatment
Endoscopic explorations:
informative
risk of perforations
GIT endoscopy
• Upper :normal
• Lower :erythematous ,edematous and ulcerated mucosa
• Pathological examination was suggestive of vasculitis
Gastrointestinal involvement in GPA is rare
Total of 21 patients developed gastrointestinal symptoms after immunosuppressive therapy
After 10 days of conservative Therapy
• Bleeding diminished
• Urine output decreased 2500 ml per day
Shift to Methotexate (2 months )
12.5 mg SC weekly
The patient is doing very well
Rheumatological Case Study

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