Nephrology presentation 28/3/2011
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Transcript of Nephrology presentation 28/3/2011
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NEPHROLOGY PRESENTATION
28/3/2011
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HISTORY
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62 year male from Bethlehem referred with renal failure
1/12 ago: Constitutional complaints No clear focus of infection Raised CRP and lymphopenia Normal renal function
He was treated symptomatically and discharged
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On systemic enquiry - fleeting arthralgia of the large joints - myalgia of the arms and thighs with initiation of movement - he denied any ENT symptoms or skin rash - 35 pack year smoking history, diagnosed with emphysema, but never had any episodes of acute bronchospasm
HOWEVER, ONE MONTH LATER, HIS SYMPTOMS HAD NOT IMPROVED
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Occupation: administrative work, some occasional welding
No previous history of note, i.e. hypertension, DMNo nephrotoxic drugs
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CLINICAL EXAMINATION
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Vitals: BP 105/70, pulse 80/minGen: pale with no edemaCVS: loud P2Resp: hyperinflated with good bilateral air entryAbdo: normalCNS: normalENT: reddish uvula, no ulcersSkin: no evidence of vasculitis / connective tissue disease
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SPECIAL INVESTIGATIONS
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Urine dipstix: 3+ hematuria, 2+ proteinuriaMicroscopy: active sedimentMCS: no evidence of infection24 hour collection: dU protein 0.5g
URINE
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FBC: Hb 8,4 / MCV 88 U+E: urea 11,6 creatinine 299CRP 69 ESR >90HIV (-)Hepatitis B and C (-)Protein electrophoresis normalComplement normal ANA (-)cANCA (proteinase 3) > 100, pANCA (MPO) - negativeAnti-GBM - pending
BLOOD INVESTIGATIONS
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Renal ultrasound showed normal sized kidneysCXR: hyperinflation, but clearShoulder x-ray: evidence of OA
RADIOLOGY
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Segmental necrotizing vaculitisFew crescentsNo granuloma formationNo eosinophillic infiltratesNo immune complexes
HISTOLOGY
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SMALL CELL VASCULITIS AND
THE KIDNEY
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Vasculitis is a clinicopathological process characterized by inflammation of and damage to the blood vessels
Affected vessels vary in size, type and location
Can occur as result of a primary process or secondary to another underlying disease
Given the numerous and varied types of vessels in the kidneys, renal disease is caused by a variety of systemic vasculitides.
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Vasculitis
Large sized vessels Small sized vessels-Takayasu’s - ANCA (+) -Giant cell arteritis - ANCA (-) Medium-sized vessels - Poly-arteritis nodosa - Kawasaki’s disease
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ANCA (+)
Wegener’s granulomatosis
Microscopic poliangiitisRenal limited vasculitis
/ ANCA GNChurg Strauss
syndromeDrug induced
ANCA (-)
Henloch Shönlein purpura
CryglobulinemicConnective tissue
disordersGoodpasture’s diseaseInfection inducedHypersensitivityParaneoplastic
SMALL VESSEL VASCULITIS
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ANCA (+)
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5-10 / 1 000 000Granulomatous inflammation and necrotizing vasculitis
affecting the nasal passages, airways and kidneys.Common presentation include upper airway involvement –
epistaxis, nasal crusting, hemoptysis, ulceration and deafness secondary to serious otitis media.
Inflammation of the retro-orbital tissue van cause proptosis and optic nerve compression
Untreated nasal discharge leads to bone and cartilage destruction
Migratory pulmonary inflitrates and nodules on CXRMostly cANCA(proteinase 3)
WEGENER’S GRANULOMATOSIS
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8 / 1 000 000
Necrotizing vasculitis affecting the capillaries, venules or arterioles
Typically presentation includes a rapidly progressive GN, often associated with alveolar haemorrhage
Cutaneous and GIT involvement
pANCA (+), but can be cANCA (+)
Considered as part of a clinical spectrum that includes Wegener’s and renal limited vasculitis
MICROSCOPIC POLIANGIITIS
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Part of the spectrum including Wegener’s and microscopic poly-angiitis
No systemic involvement
Histology: necrotizing GN
RENAL LIMITED VASCULITIS
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Renal limited vasculitis - vasculitis with necrotizing GN - no systemic symptoms
Microscopic polyangiitis - vasculitis with necrotizing GN - systemic symptoms and involvement
Wegener’s granulomatosis - vasculitis with necrotizing GN - granuloma formation - systemic symptoms and involvement
THE SPECTRUM
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Also known as allergic granulomatosis and angiitis1-3 / 1 000 000Histology similar to WG, but with eosinophillic infiltrates of
the vessel wallsProdromal period for years with allergic rhinitis, nasal
polyposis and late-onset asthmaTriad of skin lesions, asymmetric mononeuritis multiplex and
eosinophillia on a background of resistant asthmaNecrotizing GN Mesenteric vascultitiscANCA or pANCA
CHURG STRAUSS SYNDROME
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ANCA (-)
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Characterized by deposition of IgA-containing immune complexes
Children and young adults
Purpura over the buttocks and lower legs, abdominal symptoms, arthritis following an URTI
GN can occur up to 4/52 after initial symptoms
Biopsy shows IgA deposition on IF
Generally good prognosis but adult presentation, hypertension, renal failure, significant proteinuria can progress to ESRF
HENLOCH SHÖNLEIN PURPURA
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Characterized by the presence of cryoglobulins
Mix of complement and immunoglobulins, that precipitate in the cold
Type 2 (hep C) and 3 ass with vasculitis
Palpable purpura, athralgia, Raynaud’s phenomena, neuropathy
Immune complexes are deposited in the vessel walls
ESSENTIAL CRYGLOBULINEMIC VASCULITIS
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Some authors use this term to include HSP, mixed cryoglobulinemia, allergic vasculitis and serum sickness
Most properly used to refer to vasculitis occurring as a reaction to a known or suspected substance such as a drug.
Major finding is palpable purpura / petechiae – leucocytoclastic vasculitis on biopsy
HYPERSENSITIVITY VASCULITIS
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SLE, RA, relapsing polychondritis, Bechet’s diseaseOrgan involvement is determined by the underlying disease
VASCULITIS SECONDARY TO CONNECTIVE TISSUE DISORDER
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Hepatitis B, CHIV, CMV, EBV, Parvo B19
Clinical presentation can be similar to polyarteritis nodosa or microscopic poylangiitis
Even it is immune complex mediated, it must be distinguished form non-viral associated vasculitides, since the treatment is an anti-viral and not an anti-inflammatory regimen
VASCULTITIS SECONDARY TO VIRAL INFECTIONS
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The presence of a vasculitic disorder should be considered in any patient with an unexplained systemic illness
Patients often present with non-specific symptoms
However, there are a few clinical scenarios where a vascultic disorder should be looked for…
- palpable purpura - pulmonary infiltrates and microscopic hematuria - chronic inflammatory sinusitus - mononeuritis multiplex - unexplained ischaemic events - glomerulonephritis
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Primary - immunosuppressive therapy: steroids cyclophosphamide - Bactrim - plasma exchange in severe cases
Secondary - treat the underlying cause - antivirals - immunosuppression with connective tissue diseases - stop exposure to possible causative drugs
GENERAL MANAGEMENT OF SMALL VESSEL VASCULITIDES
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Glomerulonephritis with renal failurecANCAAsymptomatic “ emphysema” diagnosis, myalgia and arthalgia
No ENT symptomsNo resistant asthmaNo eosinophilliaNo skin involvementNo drug exposure
Renal biopsy: necrotizing GN few crescents no granulomata or eosinophillic infiltrates
BACK TO OUR PATIENT…
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Renal limited vascultitis / ANCA glomerulnephritisMicroscopic polyangiitis
DIFFERENTIAL INCLUDES
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Started on prednisone 1mg/kg/day cyclophosphamide 2mg/kg/day bactrim ca, titralac, vit D
Follow-up in 10/7 for review
MANAGEMENT
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THANK YOU