manifestations of vasculitides and pseudovasculitides
TRANSCRIPT
Manifestations of Manifestations of vasculitides and vasculitides and
pseudovasculitidespseudovasculitidesMikhail Valivach, Pavlodar, Mikhail Valivach, Pavlodar,
KazakhstanKazakhstan20152015
Indirect criteriaIndirect criteria Direct signs of vascilitis are histological Direct signs of vascilitis are histological
symptoms of vascular damage due to symptoms of vascular damage due to inflammationinflammation..
Other criteria of vasculitides are indirect Other criteria of vasculitides are indirect and can be also caused by other factors.and can be also caused by other factors.
Due to inconvenience of biopsies in Due to inconvenience of biopsies in different localizations of lesions it is different localizations of lesions it is acceptable to use these indirect criteria acceptable to use these indirect criteria with diagnostic purposeswith diagnostic purposes. .
Indirect criteriaIndirect criteria The most complete list of indirect criteria one can The most complete list of indirect criteria one can
find in Birmingham Vasculitis Activity Scorefind in Birmingham Vasculitis Activity Score - - BVAS BVAS (1994)/ See (1994)/ See http://www.canvasc.ca/pdf/bvasv3.pdf
In this presentation we will use BVAS not for In this presentation we will use BVAS not for vasculitis activity score but only as a list and vasculitis activity score but only as a list and glossary of symptoms.glossary of symptoms.
Many of these symptoms will be provided with Many of these symptoms will be provided with illustrations.illustrations.
One should remember that these symptoms are not One should remember that these symptoms are not specific for vasculitides but can also be found in specific for vasculitides but can also be found in pseudovasculitides (for example, in coagulopathy, pseudovasculitides (for example, in coagulopathy, microembolism, etc) and in other diseases.microembolism, etc) and in other diseases.
General symptoms of General symptoms of vasculitidesvasculitides
MyalgiaMyalgia:: Muscular painMuscular pain Arthralgia or arthritis: Arthralgia or arthritis: Joint pain Joint pain
with or without joint swellowingwith or without joint swellowing HeadacheHeadache:: Newly developed, Newly developed,
unusual or long lastingunusual or long lasting Fever: Fever: Documented increase of Documented increase of
body temperaturebody temperature Weight lossWeight loss:: Two or more kg Two or more kg
Cutaneous symptomsCutaneous symptoms InfarctionInfarction: : Area of tissue necrosis or splinter Area of tissue necrosis or splinter
haemorrhageshaemorrhages PurpuraPurpura:: Petechiae (small red spots), palpable Petechiae (small red spots), palpable
purpura, or ecchymoses (large plaques) in skin purpura, or ecchymoses (large plaques) in skin or oozing (in the absence of trauma) in the or oozing (in the absence of trauma) in the mucous membranes.mucous membranes.
Other skin vasculitis:Other skin vasculitis: Livedo reticularis, Livedo reticularis, subcutaneous nodules, erythema nodosum, etcsubcutaneous nodules, erythema nodosum, etc
UlcerUlcer:: Open sore in a skin surface Open sore in a skin surface GangreneGangrene:: Extensive tissue necrosis (e.g. Extensive tissue necrosis (e.g.
digit)digit)
Livedo reticularisLivedo reticularis
Fixed elevated erythemaFixed elevated erythema
Looks like urticaria but can stay in the same place > 24 hours
Palpable purpuraPalpable purpura
UlcerUlcer
Raynaud's phenomenonRaynaud's phenomenon
GangreneGangrene
MucousMucousmembranesmembranes
Genital ulcersGenital ulcers:: Ulcers localised in Ulcers localised in the genitalia or perineum, excluding the genitalia or perineum, excluding infectionsinfections
Mouth ulcers/granulomataMouth ulcers/granulomata:: Aphthous stomatitis, deep ulcers Aphthous stomatitis, deep ulcers and/or “strawberry” gingival and/or “strawberry” gingival hyperplasia, excluding lupus hyperplasia, excluding lupus erythematosus, and infectionerythematosus, and infection
Aphthous stomatitisAphthous stomatitis
Gingival necrosisGingival necrosis
EyesEyes Significant proptosisSignificant proptosis:: Protrusion of Protrusion of
the eyeball due to significant amounts of the eyeball due to significant amounts of inflammatory in the orbit; ifinflammatory in the orbit; if unilateral, unilateral, there should be a difference of 2 mm there should be a difference of 2 mm between one eye and the other. This may between one eye and the other. This may be associated with diplopia due to be associated with diplopia due to infiltration of extra-ocular muscles. infiltration of extra-ocular muscles. Developing myopia (measured on best Developing myopia (measured on best visual acuity, see later) can also be a visual acuity, see later) can also be a manifestation of proptosismanifestation of proptosis
EyesEyes Red eye conjunctivitisRed eye conjunctivitis: : Inflammation of the conjuctivae Inflammation of the conjuctivae
(exclude infectious causes and excluding uveitis as(exclude infectious causes and excluding uveitis as cause of red eye, also exclude conjunctivitis sicca which cause of red eye, also exclude conjunctivitis sicca which should not be scored as thisshould not be scored as this is not a feature of active is not a feature of active vasculitis); (specialist opinion not usually required).vasculitis); (specialist opinion not usually required).
Red eye (Epi)scleritis:Red eye (Epi)scleritis: Inflammation of the sclerae Inflammation of the sclerae (specialist opinion usually required). Can be heralded by(specialist opinion usually required). Can be heralded by photophobia.photophobia.
Sudden visual lossSudden visual loss: : Sudden loss of vision requiring Sudden loss of vision requiring ophthalmological assessment.ophthalmological assessment.
Blurred visionBlurred vision: : Altered measurement of best visual Altered measurement of best visual acuity from previous or baseline, requiringacuity from previous or baseline, requiring specialist specialist opinion for further evaluation.opinion for further evaluation.
EyesEyes UveitisUveitis:: Inflammation of the uvea (iris, ciliary Inflammation of the uvea (iris, ciliary
body, choroid) confirmed by ophthalmologist.body, choroid) confirmed by ophthalmologist. Retinal exudatesRetinal exudates:: Any area of soft retinal exudates Any area of soft retinal exudates
(exclude hard exudates) seen on ophthalmoscopic(exclude hard exudates) seen on ophthalmoscopic examination.examination.
Retinal haemorrhagesRetinal haemorrhages:: Any area of retinal Any area of retinal haemorrhage seen on ophthalmoscopic examination.haemorrhage seen on ophthalmoscopic examination.
Retinal vasculitisRetinal vasculitis: : Retinal vessel sheathing on Retinal vessel sheathing on examination by specialist or confirmed by retinalexamination by specialist or confirmed by retinal fluoroscein angiographyfluoroscein angiography..
Retinal vessel thrombosisRetinal vessel thrombosis: : Arterial or venous retinal Arterial or venous retinal blood vessel occlusionblood vessel occlusion
ENTENT - - syndromesyndrome Bloody nasal discharge/ nasal crusts/ulcers Bloody nasal discharge/ nasal crusts/ulcers
and/or granulomata: and/or granulomata: Bloody, mucopurulent, Bloody, mucopurulent, nasal secretion, light or dark brown crusts nasal secretion, light or dark brown crusts frequently obstructing the nose, nasal ulcers frequently obstructing the nose, nasal ulcers and/or granulomatous lesions observed by and/or granulomatous lesions observed by rhinoscopyrhinoscopy
Paranasal sinus involvement: Paranasal sinus involvement: Tenderness or Tenderness or pain over paranasal sinuses with pathologic pain over paranasal sinuses with pathologic imaging (CT, MR, x-ray, ultrasound)imaging (CT, MR, x-ray, ultrasound)
Subglottic stenosis: Subglottic stenosis: Stridor and hoarseness Stridor and hoarseness due to inflammation and narrowing of the due to inflammation and narrowing of the subglottic area observed by laryngoscopysubglottic area observed by laryngoscopy
ENTENT - - syndromesyndrome
Conductive hearing loss: Conductive hearing loss: Hearing loss due to Hearing loss due to middle ear involvement confirmed by otoscopy middle ear involvement confirmed by otoscopy and/or tuning fork examination and/or and/or tuning fork examination and/or audiometryaudiometry
Sensorineural hearing loss: Sensorineural hearing loss: Hearing loss due Hearing loss due to auditory nerve or cochlear damage to auditory nerve or cochlear damage confirmed by audiometryconfirmed by audiometry
ChestChest Wheeze: Wheeze: Wheeze on clinical examinationWheeze on clinical examination Nodules or cavities: Nodules or cavities: New lesions, New lesions,
detected by CXRdetected by CXR Pleural effusion/pleurisy: Pleural effusion/pleurisy: Pleural pain Pleural pain
and/or friction rub on clinical and/or friction rub on clinical assessment or new onset of assessment or new onset of radiologically confirmed pleural radiologically confirmed pleural effusion. Other causes (e.g. infection, effusion. Other causes (e.g. infection, malignancy) should be excludedmalignancy) should be excluded
ChestChest Infiltrate: Infiltrate: Detected by CXR or CT scan. Other Detected by CXR or CT scan. Other
causes (infection) should be excludedcauses (infection) should be excluded Endobronchial involvement: Endobronchial involvement: Endobronchial Endobronchial
pseudotumor or ulcerative lesions. Other causes pseudotumor or ulcerative lesions. Other causes such as infection or malignancy should be excluded. such as infection or malignancy should be excluded. NB: smooth stenotic lesions to be included in VDI; NB: smooth stenotic lesions to be included in VDI; subglottic lesions to be recorded in the ENT subglottic lesions to be recorded in the ENT section.section.
Massive haemoptysis/alveolar haemorrhage: Massive haemoptysis/alveolar haemorrhage: Major Major pulmonary bleeding, with shifting pulmonary pulmonary bleeding, with shifting pulmonary infiltrates; other causes of bleeding should be infiltrates; other causes of bleeding should be excluded if possibleexcluded if possible
Pulmonary infiltrates
Nodules and cavities
Cardiovascular Cardiovascular manifestationsmanifestations
Loss of pulses: Loss of pulses: Loss of pulses in any vessel detected Loss of pulses in any vessel detected clinically; this may include loss of pulses leading to clinically; this may include loss of pulses leading to threatened loss of limbthreatened loss of limb
Valvular heart disease: Valvular heart disease: Significant valve Significant valve abnormalities in the aortic mitral or pulmonary abnormalities in the aortic mitral or pulmonary valves detected clinically or echocardiographically valves detected clinically or echocardiographically
Pericarditis: Pericarditis: Pericardial pain &/or friction rub on Pericardial pain &/or friction rub on clinical assessment clinical assessment
Ischaemic cardiac pain: Ischaemic cardiac pain: Typical clinical history of Typical clinical history of cardiac pain leading to myocardial infarction or cardiac pain leading to myocardial infarction or angina. Consider the possibility of more common angina. Consider the possibility of more common causes (eg atherosclerosis)causes (eg atherosclerosis)
Cardiovascular Cardiovascular manifestationsmanifestations
Cardiomyopathy: Cardiomyopathy: Significant Significant impairment of cardiac function due impairment of cardiac function due to poor ventricular wall motion to poor ventricular wall motion confirmed on echocardiographyconfirmed on echocardiography
Congestive cardiac failure: Congestive cardiac failure: Heart Heart failure by history or clinical failure by history or clinical examinationexamination
Abdominal Abdominal manifestationsmanifestations
Peritonism: Peritonism: Acute abdominal pain with Acute abdominal pain with peritonism/peritonitis due to perforation/infarction of small peritonism/peritonitis due to perforation/infarction of small bowel, appendix or gallbladder etc., or acute pancreatitis bowel, appendix or gallbladder etc., or acute pancreatitis confirmed by radiology/surgery/elevated amylaseconfirmed by radiology/surgery/elevated amylase
Bloody diarrhoea: Bloody diarrhoea: Of recent onset; inflammatory bowel Of recent onset; inflammatory bowel disease and infectious causes excluded disease and infectious causes excluded
Ischaemic abdominal pain: Ischaemic abdominal pain: Severe abdominal pain with Severe abdominal pain with typical features of ischaemia confirmed by imaging or at typical features of ischaemia confirmed by imaging or at surgery, with typical appearances of aneursyms or surgery, with typical appearances of aneursyms or abnormal vasculature characteristic of vasculitisabnormal vasculature characteristic of vasculitis
Renal manifestationsRenal manifestations Hypertension: Hypertension: Diastolic BP>95, Diastolic BP>95,
accelerated or not, with or without accelerated or not, with or without retinal changesretinal changes
Proteinuria: Proteinuria: >1+ on urinalysis; >0.2g/24 >1+ on urinalysis; >0.2g/24 hours Infection should be excluded hours Infection should be excluded
Haematuria: Haematuria: 10 or more RBC per hpf 10 or more RBC per hpf ( high power field ), excluding urinary ( high power field ), excluding urinary infection and urinary lithiasis (stone)infection and urinary lithiasis (stone)
CreatinineCreatinine above normal above normal
Nervous systemNervous system Headache: Headache: New, unaccustomed & persistent headacheNew, unaccustomed & persistent headache Meningitis: Meningitis: Severe headache with neck stiffness Severe headache with neck stiffness
ascribed to inflammatory meningitis after excluding ascribed to inflammatory meningitis after excluding infection/bleedinginfection/bleeding
Organic confusion: Organic confusion: Impaired orientation, memory or Impaired orientation, memory or other intellectual function in the absence of metabolic, other intellectual function in the absence of metabolic, psychiatric, pharmacological or toxic causespsychiatric, pharmacological or toxic causes
Seizures Seizures (not hypertensive): Paroxysmal electrical (not hypertensive): Paroxysmal electrical discharges in the brain & producing characteristic discharges in the brain & producing characteristic physical changes including tonic & clonic movements physical changes including tonic & clonic movements & certain behavioural changes& certain behavioural changes
Nervous systemNervous system Stroke: Stroke: Cerebrovascular accident resulting in focal Cerebrovascular accident resulting in focal
neurological signs such as paresis, weakness, etc. A neurological signs such as paresis, weakness, etc. A stroke due to other causes (eg atherosclerosis) should stroke due to other causes (eg atherosclerosis) should be considered & appropriate neurological advice is be considered & appropriate neurological advice is recommended.recommended.
Cord lesion: Cord lesion: Transverse myelitis with lower extremity Transverse myelitis with lower extremity weakness or sensory loss (usually with a detectable weakness or sensory loss (usually with a detectable sensory level) with loss of sphincter control (rectal & sensory level) with loss of sphincter control (rectal & urinary bladder).urinary bladder).
Cranial nerve palsy: Cranial nerve palsy: Facial nerve palsy, recurrent Facial nerve palsy, recurrent nerve palsy, oculomotor nerve palsy etc. excluding nerve palsy, oculomotor nerve palsy etc. excluding sensorineural hearing loss and ophthalmic symptoms sensorineural hearing loss and ophthalmic symptoms due to inflammationdue to inflammation
Nervous systemNervous system Sensory peripheral Neuropathy: Sensory peripheral Neuropathy: Sensory Sensory
neuropathy resulting in glove &/or stocking neuropathy resulting in glove &/or stocking distribution of sensory loss. Other causes should distribution of sensory loss. Other causes should be excluded (e.g. idiopathic, metabolic, vitamin be excluded (e.g. idiopathic, metabolic, vitamin deficiencies, infectious, toxic, hereditary).deficiencies, infectious, toxic, hereditary).
Motor mononeuritis multiplex: Motor mononeuritis multiplex: Simultaneous Simultaneous neuritis of many peripheral nerves, only scored neuritis of many peripheral nerves, only scored if motor involvement. Other causes should be if motor involvement. Other causes should be excluded (diabetes, sarcoidosis, carcinoma, excluded (diabetes, sarcoidosis, carcinoma, amyloidosis).amyloidosis).
Dear colleagues, with questions and Dear colleagues, with questions and suggestions you can address to suggestions you can address to Mikhail Valivach Mikhail Valivach [email protected]
Thank you for attentionThank you for attention!!