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Wegener’s Granulomatosis- 3 case studies

Wegener’s Granulomatosis- 3 case studies. Jill Firth Rheumatology Nurse Specialist St. Luke’s Hospital, Bradford. Case 1. 22 year old Asian male Presented 4 years ago with : ENT symptoms dating back to 1993 Recurrent epistaxis / nasal blockage Weight loss Fever

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Wegener’s Granulomatosis- 3 case studies

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  1. Wegener’s Granulomatosis-3 case studies Jill Firth Rheumatology Nurse Specialist St. Luke’s Hospital, Bradford

  2. Case 1 • 22 year old Asian male • Presented 4 years ago with: • ENT symptoms dating back to 1993 • Recurrent epistaxis / nasal blockage • Weight loss • Fever • Bilateral lacrimal,parotid & orbital swelling • Decreasing visual acuity • Opacities on CXR

  3. Investigations: • CRP > 200 • C-ANCA +ve / PR3 +ve • Histology of pulmonary nodule showed necrotising vasculitis and granuloma formation

  4. Management: • 60mg prednisolone • Cyclophosphamide 15mg/kg (after banking sperm) • Received 20 pulses (around exams) • Converted to MTX 17.5mg weekly (ALT) • Weaned off steroids (weight gain + acne)

  5. Relapsed • 40mg of prednisolone + MTX 20mg weekly • Improved, then: • Decreased visual acuity (proptosis, ptosis) • 3rd 4th & 6th nerve palsy • CT brain – large enhancing mass in intraconal position L orbit

  6. Progress • Responded to further course of cyclo • On 10mg prednisolone & MTX 22.5mg • Well & enjoying a business placement • A success story?

  7. Case 2 • 52 year old male employed by BT • Married to a teacher • Unwell since October 2000 • Presented to ENT in March 2001

  8. Presentation • Weight loss • Night sweats • Bilateral neck swelling • Gingivitis & Rhinosinusitis • Referred to Haematologists with ? Lymphoma

  9. Investigations • Biopsy of submandibular lumps showed acute sialadenitis with vasculitis • C-ANCA + PR3 +ve • Raised CRP • Patchy lesion on CXR.

  10. ENT Team • Diagnosed Wegener’s • Advised if no renal involvement refer directly to Rheumatology. • On-going care – later grommets inserted

  11. Management • Commenced on 40 mg Prednisolone & pulsed cyc 15 mg / kg. • 14 pulses then converted to MTX 15mg weekly • Prednisolone reduced to 5mg daily

  12. Progress • Doing well • ANCA - ve • Socially isolated • Ongoing support by CNS • Referred DEAS

  13. Relapsed • June 2002: • Arthralgia, sinusitis, L blepharitis • Anxious/confused, drowsy, slurred speech, spasms R arm

  14. Neurology Referral • LP normal • CT & MRI: area (L) thalamus consistent with infarct & non-specific appearances, possibly vasculitic process.

  15. Slow Progress • Recommenced Cyclo & Pred 40 mg • Poor short-term memory & expressive dysphasia. • Nasal swab – Staph – Septrin • Paroxetine discontinued.

  16. Now • On increasing dose of Azathioprine • Very depressed – Amitriptylline – improving • Monthly review by CNS – • Socially isolated • Ref DEAS

  17. Case 3 • 15 year old female • GP presentation 02/02/03 with: • 1/12 sore throat • 2/52 migratory polyarthritis • Early morning stiffness in knees and arms • No rash or murmur

  18. Clinical Features • ? Rheumatic Fever, FBC/E/ESR/ASOT/Monospot • Penicillin and Ibuprofen • Urgent Paeds OPA: • Recent episode of conjunctivitis noted • ? JCA

  19. Results • ESR 36, CRP 107 • RhF 168 (<40) • ASOT 127 (0-100) • ANA + 1/40, mildly raised • dsDNA normal • Xrays normal

  20. Diagnosis • 24/02/03 Seropositive Polyarticular Juvenile Chronic Arthritis • Referred to Ophthalmology, Physiotherapy and Paediatric Rheumatologist

  21. But… • 26/02/03 Emergency admission: D&V / Abdo pain / Fever / Purpura Hb 6.9 WBC 41.6 neut 36.5 AXR dilated small bowel • Transferred to ITU Transfused 2 units iv Abx

  22. ITU • 27/02/03: Surgical opinion - Improved, not for surgery ? HSP / Crohns • Haematuria, Melaena Hb 4.7, DCT Neg, PT Elevated 4 units blood, Vit K, FFP • Dr Helliwell consulted - for MP

  23. Transfer to Ward • 28/02/03: Melaena stopped. Hb 8.4 • Started Methylprednisolone • cANCA – PR3 > 100 Diagnosed with Wegener’s Granulomatosis • Improved - Hb 10.5 • School /Child & Adolescent Psychology services involved

  24. Deterioration • 07/03/03: Abdo pain and distension, Vomiting • AXR - SBO • Laparotomy: SB Resection, Ileostomy and mucous fistula. To ITU • Histology: SB vasculitis, ischaemic necrosis, multiple perfs. c/w HSP

  25. Slow Progress • 10/03/03: TPN • 20/03/03: E coli sepsis • 07/04/03: PCI>4000, haematuria Ur 24.4 Cr 119 Renal biopsy • 16/04/03: TPN ceased • 17/04/03: Transfer F4 • 20/04/03: Fever / Abdo pain CT - ?Scar abscess Rx ABx

  26. Cytotoxic Treatment • 23/04/03: Improved. Started Cyclophosphamide pulses 2/52ly • 07/05/03: Blood in stoma OGD normal • 21/05/03: Hickman line infection Rx removed, Abx • 04/06/03: Home!

  27. Unusual Presentation #2 • 14/07/03: Sore throat / Headache / Mouth ulcers.Rx Penicillin, to A&E • Swollen tongue, DIB Not c/w anaphylaxis • ENT - Tracheostomy No glottic oedema, normal larynx Improved on ITU, then ward

  28. Relapse • 19/07/03: 3x Tonic-clonic seizures over 45 mins, terminated with diazepam • CT - ??Early infarct L parietal area • LP - normal. Apyrexial • ? Relapse of Wegener’s • Continue with Cyclophosphamide on F4

  29. Slow Progress • 29/07/03: Rt. groin cellulitis. Spiking Temps. • ? Abscess. Rx Abx, settled • 05/08/03: Lower abdo pain. No bleeding, not peritonitic • ?Abdo vasculitis • ?Clot colic (Cyclo/Vasculitis)

  30. Investigations • USS - Normal • Ur / Cr - Normal • Cystoscopy - Normal • Abdo CT - Normal • 16/08/03: Pain decreased • 18/08/03: cANCA - PR3 >100 • 20/08/03: Home!

  31. Current Situation • Reasonably well, thin but losing weight – • High output ileostomy ? reversal • On monthly Cyclophosphamide pulses • Prednisolone 10mg daily • Omeprazole 40mg daily • Loperamide 2mg QDS • Calcichew D3 forte 1 bd

  32. But... • cANCA-PR3 still raised (39) despite cytotoxics • Very young presentation • Malignant course of disease so far • Lifelong surveillance • Prognosis poor...

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