Note: the following material is for personal use only see below for
Download 3.95 Mb.
|
- Navigate this page:
- Vasculitis Associations
Sarcoidosismore common in women, African-Americans Presentation: various protean manifestations / adults: CNS, lungs > heart >> renal Lungs: restrictive lung disease, pleurisy (with effusion) / actually does not produce rales (too much fibrosis) Liver: hepatomegaly (20-30%) Skin: both acute and chronic changes / lupus pernio (violaceous indurated lesions with a predilection for the nose, ears, lips, and face), skin plaques [dermis], maculopapular/papules (red-brown, waxy), subcutaneous nodules, and erythema nodosum, vitiligo (hypo or hyperpigmented), alopecia, old cars Ddx (for skin changes): Tb, berylliosis, leprosy, leischmaniasis, syphilis, deep fungal infection / other panniculitis (Behçet’s, superficial thrombophlebitis, cutaneous vasculitides) CNS (5%): can present with only CNS problems (peripheral neuropathy, aseptic meningitis) or focal (cranial nerves; usu. bilateral VII, hypothalamus, pituitary) Ddx (for CNS): cancer with mets, fungal or Tb, lymphoma, Langerhans histiocytosis, other Joints: knees, ankles, elbows, wrists, small joints of the hands / swollen, warm, tender, painful Complications: Lungs: hilar lymphadenopathy, pleural effusion Eye: variety of conditions / uveitis / others (20% incidence) ENT: parotitis / nasal involvement CNS: Bell’s palsy, diabetes insipidus (posterior pituitary > anterior), cranial nerves, basal meninges, hypothalamus, seizures, etc. / elevated ACE in CSF (66%), mononuclear pleocytosis / leptomeningeal enhancement [MRI] Heart: restrictive cardiomyopathy Liver: very common, but usually no symptoms, can be good biopsy site Renal: very uncommon Diagnosis: can be diagnosis of exclusion when biopsies inconclusive, often first recognized from CXR in asymptomatic patients (60-70% will have some abnormality on chest CT) Biopsy (of involved lesions): widespread non-caseating granulomas with Schaumann and asteroid bodies / may look like Tb / any one of following has 50-80% sensitivity (all three combined have 99% sensitivity) / note: granulomas in scalene, liver nodes are not (by themselves) sufficient for diagnosis (because granulomas are so frequent in these nodes) Transbronchial biopsy (TBLB) Transbronchial needle aspiration (TBNA) BAL showing lymphocyte predominance ( > 12%), high CD4:CD8 ratio (should not have high neutrophils or eosinophils at same time; ratio > 3.5 has 90% specificity, 50% sensitivity) Labs: Hypercalcemia (10%) (elevated 1-hydroxylase produces 1,25-OH D3) (hypercalciuria in 33%) serum ACE elevated in 66% (many false positives including Mycobacteria and malignancy) lysozyme elevated d-dimer (correlates with disease activity) Course: often asymptomatic and self-limiting Children under 5: skin rash, eyes (uveitis), arthritis (worse prognosis) Older children: lungs (usu. bilateral, hilar lymphadenopathy), lymph nodes, eyes
acute sarcoidosis / usually with symmetric, periarticular ankle inflammation / may have erythema nodosum Systemic vasculitides Complement levels all have normal complement levels except variable in PAN, leukocytoclastic, connective tissue disease, endocarditis / decreased in urticarial vasculitis Vasculitis AssociationsPAN and hairy cell leukemia Wegener’s and Hodgkin’s disease Granulomatous angiitis of CNS and lymphoma GCA and lymphoma HSP and lymphoma grouped by vessel-size Large giant-cell arteritis Takayasu’s arteritis primary CNS vasculitis Medium (with or w/out involvement of small) PAN Kawasaki’s Churg-Strauss Wegener’s Buerger’s? Small leukocytoclastic (HSP, cryoglobulinemia, infectious) connective tissue diseases
microscopic PAN urticarial vasculitis
APAS
endocarditis (bacterial/marantic) other embolic cholesterol embolism drugs (amphetamines and rarely cocaine) Infectious Vasculitis Ddx Bacterial agents Acute septic meningitis agents Mycobacteria Spirochetes Treponema, Borrelia sp., Leptospira
Brucella species Bartonella henselae
Mycoplasma Viral agents HSV, VZV, CMV, EBV, B19, HBV, HCV, HIV, HTLV More: hantavirus, California encephalitis virus, EEE encephalitis virus, influenza, rubella Fungus
Aspergillus, Coccidoides, Candida Mucormycetes Parasites Cysticercosis CNS vasculitis Ddx: reversible cerebral vasoconstriction syndromes (RCVS) PAN – classic, microscopic/HBV, HIV Wegener’s Takayasu’s hypersensitivity angiitis – drug-induced, HSP neoplasia (many) infection (see below) CTD: SLE, RA, GCA primary angiitis of CNS (PACNS) – CNS angiography, brain biopsy
Acute septic meningitis agents Mycobacteria (5%) Spirochetes Treponema, Borrelia sp., Leptospira (Weil syndrome) Brucella species Bartonella henselae Rickettsiae Mycoplasma Viral agents HSV, VZV, CMV, HBV, HCV, HIV, HTLV Fungus
Aspergillus, Coccidoides, Candida Mucormycetes Parasites Cysticercosis BACTERIAL AGENTS
Neonates get arteritis/thrombophlebitis (larger, +/- hemorrhagic infarcts, +/- secondary abscess formation) / venous thrombosis and hemorrhagic necrosis (associated with Pseudomonas, Proteus, Enterobacter, and Serratia) Septic venous sinus thrombosis/thrombophlebitis (up to 5%, usu. < 1 or 2 weeks) Note: sinus, middle ear, skull infection can beget cerebral vasculitis without detectable meningitis / also, basilar infection can causes vasculitis of ascending arteries Peripheral Nervous System Lyme disease multifocal axonal radiculoneuropathy HIV-1 multiple mononeuropathies CMV
HCV cryoglobulinemia HSV, VZV sensory ganglia, radicular syndrome Giant Cell Arteritis (GCA) (temporal arteritis) Not uncommon (1 in 5000) / usually > 50 yrs / women > men (2:1) / whites, Scandinavians / HLA-DRB1*04 and DRB1*01 Presentation: gradual > abrupt / 15% fever / headache (66%, unilateral >> bilateral, dull and boring, superimposed sharp pain), jaw claudication (50%), temporary blindness, fever, weight loss, myalgias/arthralgia, malaise, cough/hoarseness (10%), neuropathies (10%), TIA, polymyalgia rheumatica (50%) Complications: blindness (can occur early on, retinal changes like edema of optic disk, cotton-wool patches, small hemorrhages, usu. occur after blindness, usu. from nerve ischemia), eye exam can be helpful / thoracic aortic aneurysm (17x) normal incidence Associations: lymphoma (vasculitis may precede lymphoma) / Pathology: mainly external carotids and vertebral / can hit central retinal artery (but intracranial arteries are NOT involved) Diagnosis: biopsy temporal artery, try to get affected site (if cannot localize on exam, then get larger piece, 3-5 cm), can do bilateral biopsy to increase yield, yield of biopsy decreases with each day of steroids but can still be positive even at 1-2 wks post-steroid (lymphocytes, plasma cells, giant cells) / high ESR (over 50, often > 100, can be expected to decrease within days of treatment, ~20% have normal ESR) / CRP is more sensitive / NOTE: careful exam may reveal findings prior to onset of symptoms Treatment: prednisone (1 mg/kg qd 1-2 months then taper by 5-10% q 1-2 wks) / usually see improvement within days (if not, question diagnosis) / can use 100 mg qd for optic nerve involvement / can begin cyclophosphamide (maybe other DMARDS) if necessary / can begin to think about tapering after 1-2 months (10-20% every 2 weeks), but must continue to treat for a long time (1-4 yrs to reduce recurrence) / qod therapy thought to be less effective but open question of whether some patients can start at lower doses (20-30 mg qd) / ½ of patients have serious complication of extended steroid use ASA also thought to decrease risk of occlusions Recurrence: 30-50% have spontaneous exacerbations independent of steroid regimen / most often, recurrence involves PMR Sx and can be treated by increasing steroids by 2 to 5 mg/qd Takayasu’s Arteritis Granulomatous inflammation of large arteries / young women (>Asian) / complications may develop over months to years / affects aorta and branches (subclavian), pulmonary arteries (up to 50%), renal artery Presentation: weak pulse in upper extremities (arm claudication), ocular disturbances, HTN (renal artery) / Raynaud’s / systemic: fever, weight loss
Labs: increased ESR Kawasaki’s Mostly children (4-5/years) / medium-sized arteries 5 diagnostic criteria: more than 5 days of fever, bilateral conjunctival injections, oral mucosa and pharynx (infected and dry fissured lips, strawberry tongue), peripheral extremities (edema, erythema, desquamation – rash, primarily truncal), cervical adenopathy Complications: hydrops of gallbladder, more - Treatment: restrict activity 3-4 wks Anti-inflammatory Immunoglobulin 2 gm/kg single / 400 mg/kg/day x 4 days MMR should be delayed 6 months Aspirin – 80-100 mg/kg/day QID until afebrile Anti-platelet agents Treat for 3 months, but indefinitely and add more agents? if coronary involvement ANCA Associated Vasculitides (AAV) Polyarteritis Nodosum (PAN) rare / occlusion (infarct) of medium to small arteries (NOT capillaries) / type III hypersensitivity Presentation: usually present with constitutional symptoms Associations: HBV, hairy cell leukemia Findings: cotton-wool patches, pericarditis, myocarditis, palpable purpura aneurysm (hemorrhage) Diagnosis: 3 or more of 10 criteria (sensitivity 80%, specificity 85%)
Download 3.95 Mb. Share with your friends:
|