NEPHROLOGY PRESENTATION 28/3/2011. HISTORY 62 year male from Bethlehem referred with renal failure ...
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Transcript of NEPHROLOGY PRESENTATION 28/3/2011. HISTORY 62 year male from Bethlehem referred with renal failure ...
NEPHROLOGY PRESENTATION
28/3/2011
HISTORY
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
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
Occupation: administrative work, some occasional welding
No previous history of note, i.e. hypertension, DMNo nephrotoxic drugs
CLINICAL EXAMINATION
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
SPECIAL INVESTIGATIONS
Urine dipstix: 3+ hematuria, 2+ proteinuriaMicroscopy: active sedimentMCS: no evidence of infection24 hour collection: dU protein 0.5g
URINE
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
Renal ultrasound showed normal sized kidneysCXR: hyperinflation, but clearShoulder x-ray: evidence of OA
RADIOLOGY
Segmental necrotizing vaculitisFew crescentsNo granuloma formationNo eosinophillic infiltratesNo immune complexes
HISTOLOGY
SMALL CELL VASCULITIS AND
THE KIDNEY
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.
Vasculitis
Large sized vessels Small sized vessels-Takayasu’s - ANCA (+) -Giant cell arteritis - ANCA (-) Medium-sized vessels - Poly-arteritis nodosa - Kawasaki’s disease
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
ANCA (+)
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
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
Part of the spectrum including Wegener’s and microscopic poly-angiitis
No systemic involvement
Histology: necrotizing GN
RENAL LIMITED VASCULITIS
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
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
ANCA (-)
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
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
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
SLE, RA, relapsing polychondritis, Bechet’s diseaseOrgan involvement is determined by the underlying disease
VASCULITIS SECONDARY TO CONNECTIVE TISSUE DISORDER
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
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
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
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…
Renal limited vascultitis / ANCA glomerulnephritisMicroscopic polyangiitis
DIFFERENTIAL INCLUDES
Started on prednisone 1mg/kg/day cyclophosphamide 2mg/kg/day bactrim ca, titralac, vit D
Follow-up in 10/7 for review
MANAGEMENT
THANK YOU