Vasculitis
Vasculitis (plural: vasculitides), refers to a heterogeneous group of disorders that are characterized by inflammatory destruction of blood vessels - both arteries and veins are affected. Solitary inflammation of veins (phlebitis) or arteries (arteritis), although both occur in vasculitis, on their own are separate entities. Vasculitis affects both arteries and veins. Vasculitis is primarily due to leukocyte migration and resultant damage.
Classification
Vasculitides can be classified by the size of the blood vessel that they predominantly affect.[1]
Large vessel vasculitis
Takayasu arteritis. Primarily affects the aorta and its main branches. At least 3 out of 6 criteria yields sensitivity and specificity of 90.5 and 97.8%:
- onset < 40 years affects young and middle -aged women (ages 15-45)
- claudication of extremities
- decreased pulsation of one or both brachial arteries
- at least 10 mmHg systolic difference in both arms
- bruit over one or both carotid arteries or abdominal aorta
- arteriographic narrowing of aorta, its primary branches, or large arteries in upper or lower extremities
- Ocular manifestation
- visual loss or field defects
- Retinal hemorrhages
- Neurological abnormalitis
- Treatment: steroids
Giant cell (temporal) arteritis. Chronic vasculitis of both large and medium vessels, primarily affecting cranial branches of the arteries arising from the aortic arch. At least 3 out of 5 criteria yields sensitivity and specificity of 95 and 91%:
- Age at onset ≥ 50 years
- New onset headache with localized tenderness
- Temporal artery tenderness or decreased pulsation
- Elevated ESR ≥ 50 mm/hour Westergren
- Temporal artery biopsy showing vasculitis with mononuclear cell infiltrate or granulomatous inflammation, usually with multinucleated giant cells
Medium vessel vasculitis
Polyarteritis nodosa. Systemic necrotizing vasculitis and aneurysm formation affecting both medium and small arteries. If only small vessels are affected, it is called microscopic polyangiitis, although it is more associated with Wegener's granulomatosis than to classic PAN. At least 3 out of 10 criteria yields sensitivity and specificity of 82 and 87%:
- unexplained weight loss > 4 kg
- livedo reticularis
- testicular pain
- myalgias, weakness
- Abdominal pain,diarrhia,and GI bleeding
- mononeuropathy or polyneuropathy
- new onset diastolic blood pressure > 90 mmHg
- elevated serum BUN (> 40 mg/dL) or serum creatinine (> 1.5 mg/dL)
- hepatitis B infection
- arteriographic abnormalities
- arterial biopsy showing polymorphonuclear cells
Wegener's granulomatosis. Systemic vasculitis of medium and small arteries, including venules and arterioles. Produces granulomatous inflammation of the respiratory tracts and necrotizing, pauci-immune glomerulonephritis. Most common cause of saddle nose deformity in USA (nose flattened due to destruction of nasal septum by granulomatous inflammation). Almost all patients with WG have c-ANCA, but not vice versa. Current treatment of choice is cyclophosphamide. At least 2 out of 4 criteria yields sensitivity and specificity of 88 and 92%.
- nasal or oral inflammation (oral ulcers or purulent/bloody nasal discharge, may be painful)
- abnormal CXR showing nodules, infiltrates, cavities
- microscopic hematuria or RBC casts
- vessel biopsy shows granulomatous inflammation
- Peak incidence: ages 40-60 ,males > females
Kawasaki disease. Usually in children(age<4), it affects large, medium, and small vessels, prominently the coronary arteries. Associated with a mucocutaneous lymph node syndrome. Diagnosis requires fever lasting five days or more with at least 4 out of 5 criteria:
- bilateral conjunctival injection
- injected or fissured lips, injected pharynx, or strawberry tongue
- erythema of palms/soles, edema of hands/feet, periungual desquamation
- polymorphous rash
- cervical lymphadenopathy (at least one node > 1.5 cm)
Isolated CNS vasculitis. Affects medium and small arteries over a diffuse CNS area, without symptomatic extracranial vessel involvement. Patients have CNS symptoms as well as cerebral vasculitis by angiography and leptomeningeal biopsy.
Small vessel vasculitis
There are several vasculitides that affect small vessels.[2]
Churg-Strauss arteritis. Affects medium and small vessels with vascular and extravascular granulomatosis. Classically involves arteries of lungs and skin, but may be generalized. At least 4 criteria yields sensitivity and specificity of 85 and 99.7%.
- asthma (history of wheezeing or presently wheezing)
- eosinophilia > 10% on CBC
- mononeuropathy or polyneuropathy
- migratory or transient pulmonary opacities on chest x-ray (CXR)
- paranasal sinus abnormalities
- vessel biopsy showing eosinophils in extravascular areas
Microscopic polyarteritis/polyangiitis. Affects capillaries, venules, or arterioles. Thought to be part of a group that includes Wegeners since both are associated with ANCA and similar extrapulmonary manifestations. Patients do not usually have symptomatic or histologic respiratory involvement.
Hypersensitivity vasculitis (allergic vasculitis). Usually due to a hypersensitivity reaction to a known drug. Drugs most commonly implicated are penicillin, sulphonamides and thiazide diuretics.[3]There is presence of skin vaculitis with palpable petechiae or purpura. Biopsy of these lesions reveal inflammation of the small vessels, termed leukocytoclastic vasculitis, which is most prominent in postcapillary venules. At least 3 out of 5 criteria yields sensitivity and specificity of 71 and 84%:
- age > 16
- use of possible triggering drug in relation to symptoms
- palpable purpura
- maculopapular rash
- skin biopsy showing neutrophils around vessel
Henoch-Schonlein purpura. Systemic vasculitis due to tissue deposition of IgA-containing immune complexes. Biopsy of lesions shows inflammation of small vessels. It is considered a form of hypersensitivity vasculitis but is distinguished by prominent deposits of IgA. This is the most common vasculitis in children. Presence of 3 or more criteria yielded sensitivity of 87% while less than 2 criteria yielded hypersensitivity vasculitis in 74%:
- palpable purpura (usually of buttocks & legs)
- bowel angina
- GI bleed
- hematuria
- onset < 20 years
- no new medications
Essential cryoglobulinemic vasculitis. Most often due to hepatitis C infection, immune complexes of cryoglobulins --- proteins that consists of immunoglobulins and complement and precipitate in the cold while dissolving upon rewarming --- are deposited in walls of capillaries, venules, or arterioles. Therefore, complement will be low with histology showing vessel inflammation with immune deposits.
Vasculitis secondary to connective tissue disorders. Usually secondary to systemic lupus erythematosus (SLE), rheumatoid arthritis (RA), relapsing polychondritis, Behçet's disease, and other connective tissue disorders.
Vasculitis secondary to viral infection. Usually due to hepatitis B and C, HIV, cytomegalovirus, Epstein-Barr virus, and Parvo B19 virus.
Symptoms and signs
Patients usually present with systemic symptoms with single or multiorgan dysfunction. Common (and nonspecific) complaints include fatigue, weakness, fever, arthralgias, abdominal pain, hypertension, renal insufficiency, and neurologic dysfunction. The following symptoms should raise a strong suspicion of a vasculitis:
- Mononeuritis multiplex. Also known as asymmetric polyneuropathy is highly suggestive of vasculitis, since diabetes is the only other cause of this.
- Palpable purpura. If patients have this in isolation, it is most likely due to cutaneous leukocytoclastic vasculitis. If the purpura is in combination with systemic organ involvement, it is most likely to be Henoch-Schonlein purpura or microscopic polyarteritis.
- Pulmonary-renal syndrome. Patients with hemoptysis and renal involvement are likely to have Wegener's granulomatosis, microscopic polyangiitis, or anti-GBM disease (Goodpasture's syndrome).
Diagnosis
A detailed history is important to elicit any recent medications, any risk of hepatitis infection, or any recent diagnosis with a connective tissue disorder such as systemic lupus erythematosus (SLE). A thorough physical exam is needed as usual.
- Lab tests. Basic lab tests may include a CBC, chem-7 (look for creatinine), muscle enzyme, liver function tests, ESR, hepatitis seroloties, urinalysis, CXR, and EKG. Additional, more specific tests include:
- Antinuclear antibody (ANA) test can detect an underlying connective tissue disorder, especially SLE
- Complement levels that are low can suggest mixed cryoglobulinemia, hepatitis C infection, and SLE, but not most other vasculitides.
- Antineutrophil cytoplasmic antibody (ANCA) may highly suggest Wegener's granulomatosis, microscopic polyangiitis, Churg-Strauss syndrome, or drug-induced vasculitis, but is not diagnostic.
- Electromyography. It is useful if a systemic vasculitis is suspected and neuromuscular symptoms are present.
- Arteriography. Arteriograms are helpful in vasculitis affecting the large and medium vessels but not helpful in small vessel vasculitis. Angiograms of mesenteri or renal arteries in polyarteritis nodosa may show aneurysms, occlusions, and vascular wall abnormalities. Arteriography are not diagnostic in itself if other accessible areas for biopsy are present. However, in Takayasu's arteritis, where the aorta may be involved, it is unlikely a biopsy will be successful and angiography can be diagnostic.
- Tissue biopsy. This is the gold standard of diagnosis when biopsy is taken from the most involved area.
Treatment
Treatment is targeted to the underlying cause. However, most vasculitis in general are treated with steroids (e.g. methylprednisolone) because the underlying cause of the vasculitis is due to hyperactive immunological damage. Immunosuppressants such as cyclophosphamide and azathioprine may also be given.
A systematic review of antineutrophil cytoplasmic antibody (ANCA) positive vasculitis identified best treatments depending on whether the goal is to induce remission or maintenance and depending on severity of the vasculitis.[4]
References
- ↑ Jennette JC, Falk RJ, Andrassy K, et al (1994). "Nomenclature of systemic vasculitides. Proposal of an international consensus conference". Arthritis Rheum. 37 (2): 187–92. PMID 8129773.
- ↑ Jennette JC, Falk RJ (1997). "Small-vessel vasculitis". N. Engl. J. Med. 337 (21): 1512–23. doi:10.1056/NEJM199711203372106. PMID 9366584.
- ↑ Axford J, O'Callaghan C, (eds). 2004. Medicine. Oxford. Blackwell Publishing.
- ↑ Bosch X, Guilabert A, Espinosa G, Mirapeix E (2007). "Treatment of antineutrophil cytoplasmic antibody associated vasculitis: a systematic review". JAMA 298 (6): 655–69. doi:10.1001/jama.298.6.655. PMID 17684188.
External links
Diseases of the skin and appendages by morphology |
|
Growths |
Epidermal
|
verruca, clavus, seborrheic keratosis, acrochordon, molluscum contagiosum, actinic keratosis, squamous cell carcinoma, basal cell carcinoma, merkel cell carcinoma, nevus sebaceous, trichoepithelioma
|
|
Pigmented
|
|
|
Dermal and subcutaneous
|
epidermal inclusion cyst, hemangioma, dermatofibroma, keloid, lipoma, neurofibroma, xanthoma, Kaposi's sarcoma, infantile digital fibromatosis, granular cell tumor, leiomyoma, lymphangioma circumscriptum, myxoid cyst
|
|
|
Rashes |
With epidermal involvement
|
Eczematous
|
essential dermatitis, contact dermatitis, atopic dermatitis, seborrheic dermatitis, stasis dermatitis, lichen simplex chronicus, Darier's disease, glucagonoma syndrome, langerhans cell histiocytosis, lichen sclerosus, pemphigus foliaceus, Wiskott-Aldrich syndrome, Zinc deficiency
|
|
Scaling
|
psoriasis, tinea corporis, tinea cruris, tinea pedis, tinea manuum, tinea faciale, pityriasis rosea, secondary syphillis, mycosis fungoides, systemic lupus erythematosus, pityriasis rubra pilaris, parapsoriasis, icthyosis
|
|
Blistering
|
herpes simplex, herpes zoster, varicella, bullous impetigo, acute contact dermatitis, pemphigus vulgaris, bullous pemphigoid, dermatitis herpetiformis, porphyria cutanea tarda, epidermolysis bullosa simplex
|
|
Papular
|
scabies, insect bite reactions, lichen planus, miliaria, keratosis pilaris, lichen spinulosus, transient acantholytic dermatosis, lichen nitidus, pityriasis lichenoides et varioliformis acuta
|
|
Pustular
|
|
|
Hypopigmented
|
tinea versicolor, vitiligo, pityriasis alba, postinflammatory hypopigmentation, tuberous sclerosis, idiopathic guttate hypomelanosis, leprosy, hypopigmented mycosis fungoides
|
|
|
Without epidermal involvement
|
Red
|
Blanchable Erythema
|
Generalized
|
|
|
Localized
|
cellulitis, abscess, furuncle, erythema nodosum, carcinoid syndrome, carcinoma erysipeloides, fixed drug eruption
|
|
Specialized
|
urticaria, erythema multiforme, erythema migrans, erythema gyratum repens, erythema annulare centrifugum, erythema ab igne
|
|
|
Nonblanchable Purpura
|
Macular
|
thrombocytopenic purpura, actinic purpura
|
|
Papular
|
|
|
|
|
Indurated
|
scleroderma/morphea, granuloma annulare, lichen sclerosis et atrophicus, necrobiosis lipoidica
|
|
|
|
Miscellaneous |
Ulcers
|
|
|
Hair disorders
|
|
|
Nail disorders
|
|
|
Mucous membrane disorders
|
aphthous stomatitis, oral candidiasis, lichen planus, leukoplakia, pemphigus vulgaris, mucous membrane pemphigoid, cicatricial pemphigoid, herpesvirus, coxsackievirus, syphilis, systemic histoplasmosis, squamous cell carcinoma
|
|
|
Cardiovascular disease: vascular disease · Circulatory system pathology (I70-I99, 440-459) |
|
Arteries, arterioles
and capillaries |
Arterial occlusive disease/
peripheral vascular disease
|
Arteriosclerosis: Atherosclerosis · Intermittent claudication · Atheroma · Monckeberg's arteriosclerosis
Stenosis (Renal artery stenosis, Carotid artery stenosis) · Fibromuscular dysplasia · Degos disease · Aortoiliac occlusive disease
Raynaud's phenomenon/Raynaud's disease · Erythromelalgia
|
|
|
Aortic aneurysm/Abdominal aortic aneurysm · Cerebral aneurysm · Coronary artery aneurysm · Intracranial berry aneurysm · Dissection ( Aortic, Carotid, Vertebral) · Pseudoaneurysm
|
|
Other
|
Arteritis (Aortitis) · Buerger's disease
Arteriovenous fistula · Hereditary hemorrhagic telangiectasia
Nevus (Spider angioma, Halo nevus, Cherry angioma)
|
|
|
Veins |
Venous thrombosis/
Phlebitis/
Thrombophlebitis
|
primarily lower limb ( Deep vein thrombosis)
abdomen (May-Thurner syndrome, Portal vein thrombosis, Budd-Chiari syndrome, Renal vein thrombosis)
upper limb/torso (Paget-Schroetter disease, Mondor's disease)
head (Cerebral venous sinus thrombosis)
Post-thrombotic syndrome
|
|
Varicose veins
|
Varicocele · Gastric varices · Portacaval anastomosis ( Hemorrhoid, Esophageal varices, Caput medusae)
|
|
Other
|
Superior vena cava syndrome · Inferior vena cava syndrome · Venous ulcer
|
|
|
Arteries or veins |
|
|
Lymphatic disease |
|
|
Blood pressure |
|
Hypertensive heart disease · Hypertensive nephropathy · Secondary hypertension (Renovascular hypertension) · Pulmonary hypertension · Malignant hypertension
|
|
Hypotension
|
|
|
|
See also ,
|
|
Diseases of the skin and subcutaneous tissue (integumentary system) (L, 680-709) |
|
Infections |
Staphylococcus (Staphylococcal scalded skin syndrome, Impetigo, Boil, Carbuncle) - Cellulitis (Paronychia) - Acute lymphadenitis - Pilonidal cyst - Corynebacterium (Erythrasma) - Pimple (Pustule)
|
|
Bullous disorders |
acantholysis (Pemphigus, Transient acantholytic dermatosis) - Pemphigoid (Bullous, Mucous membrane, Gestational) - Dermatitis herpetiformis
|
|
Inflammatory |
|
Atopic dermatitis - Seborrhoeic dermatitis ( Dandruff, Cradle cap) - Contact dermatitis (Diaper rash, Urushiol-induced contact dermatitis) - Erythroderma - Lichen simplex chronicus/Prurigo nodularis - Itch (Pruritus ani) - Nummular dermatitis - Dyshidrosis - Pityriasis alba
|
|
Papulosquamous disorders
|
Psoriasis (Psoriatic arthritis) - Parapsoriasis (Pityriasis lichenoides et varioliformis acuta, Pityriasis lichenoides chronica, Lymphomatoid papulosis) - other pityriasis (Pityriasis rosea, Pityriasis rubra pilaris) - other lichenoid (Lichen planus, Lichen nitidus)
|
|
|
Dermatographic urticaria - Cold urticaria - Cholinergic urticaria
|
|
Erythema
|
Erythema multiforme/drug eruptions: Stevens-Johnson syndrome - Toxic epidermal necrolysis - Erythema nodosum
Other erythema: Erythema annulare centrifugum - Erythema marginatum - Necrolytic migratory erythema - Erythema toxicum
|
|
|
Radiation-related disorders |
Sunburn - Actinic keratosis - Polymorphous light eruption - Radiodermatitis - Erythema ab igne
|
|
Pigmentation disorder |
hypopigmentation ( Albinism, Vitiligo) - hyperpigmentation (Melasma, Freckle, Café au lait spot, Lentigo/Liver spot, Acanthosis nigricans)
|
|
Other skin |
keratosis/hyperkeratosis (Seborrheic keratosis, Callus) - other epidermal thickening (Ichthyosis acquisita, Palmoplantar keratoderma)
skin ulcer (Pyoderma gangrenosum, Bedsore)
atrophic (Lichen sclerosus, Acrodermatitis chronica atrophicans)
necrobiosis (Granuloma annulare, Necrobiosis lipoidica) - other granuloma (Granuloma faciale, Pyogenic granuloma)
vasculitis (Livedoid vasculitis, Erythema elevatum diutinum)
|
|
Connective tissues |
|
|
Disorders of
skin appendages |
Nail
|
Ingrown nail - Onychogryposis - Beau's lines - Yellow nail syndrome - Leukonychia
|
|
Hair
|
Hair loss
|
Alopecia areata (Alopecia totalis, Alopecia universalis, Ophiasis)
Androgenic alopecia - Hypotrichosis - Telogen effluvium - Traction alopecia - Lichen planopilaris - Trichorrhexis nodosa
|
|
Other
|
Hypertrichosis (Hirsutism)
Acneiform eruption (Acne vulgaris, Chloracne, Blackhead) - Rosacea (Perioral dermatitis, Rhinophyma)
follicular cysts (Epidermoid cyst, Sebaceous cyst, Steatocystoma multiplex)
Pseudofolliculitis barbae - Hidradenitis suppurativa - Folliculitis
|
|
|
Sweat glands
|
eccrine (Miliaria, Anhidrosis) - apocrine (Body odor, Chromhidrosis, Fox-Fordyce disease)
|
|
|
see also congenital, neoplasia
|
|
Systemic CT disorders (M30-M36, 710, 440-448) |
|
Vasculitis/arteritis
(including ANCA-associated vasculitides) |
|
Takayasu's arteritis · Temporal arteritis
|
|
|
|
|
|
Churg-Strauss syndrome · Microscopic polyangiitis · Hypersensitivity vasculitis
|
|
|
Mixed connective tissue disease |
|
|
Other hypersensitivity/autoimmune |
Goodpasture's syndrome · Sjögren's syndrome
|
|
Other |
Behçet's disease · Polymyalgia rheumatica · Eosinophilic fasciitis · Marfan syndrome
|
|
see also soft tissue disorders
|
|
Inflammation |
|
Acute |
|
|
Chronic |
Macrophage · Epithelioid cell · Giant cell · Granuloma
|
|
Processes |
Traditional: Rubor · Calor · Tumor · Dolor (pain) · Functio laesa
Modern: Acute-phase reaction/ Fever · Vasodilation · Increased vascular permeability · Exudate · Leukocyte extravasation · Chemotaxis
|
|
Specific types |
|
CNS ( Encephalitis, Myelitis) · PNS (Neuritis) · Meningitis · eye (Dacryoadenitis, Scleritis, Keratitis, Choroiditis, Retinitis, Chorioretinitis, Blepharitis, Conjunctivitis, Iritis, Uveitis) · ear (Otitis, Labyrinthitis, Mastoiditis)
|
|
|
Carditis ( Endocarditis, Myocarditis, Pericarditis) · Vasculitis (Arteritis, Phlebitis, Capillaritis)
|
|
|
upper ( Sinusitis, Rhinitis, Pharyngitis, Laryngitis) · lower (Tracheitis, Bronchitis, Bronchiolitis, Pneumonitis, Pleuritis) · Mediastinitis
|
|
|
mouth (Stomatitis, Gingivitis, Gingivostomatitis, Glossitis, Tonsillitis, Sialadenitis/Parotitis, Cheilitis, Pulpitis, Gnathitis) · tract (Esophagitis, Gastritis, Gastroenteritis, Enteritis, Colitis, Enterocolitis, Duodenitis, Ileitis, Caecitis, Appendicitis, Proctitis) · accessory ( Hepatitis, Cholangitis, Cholecystitis, Pancreatitis) · Peritonitis
|
|
|
Dermatitis (Folliculitis) · Hidradenitis
|
|
Musculoskeletal
|
Arthritis · Dermatomyositis · soft tissue (Myositis, Synovitis/Tenosynovitis, Bursitis, Enthesitis, Fasciitis, Capsulitis, Epicondylitis, Tendinitis, Panniculitis)
Osteochondritis: Osteitis (Spondylitis, Periostitis) · Chondritis
|
|
|
Nephritis (Glomerulonephritis, Pyelonephritis) · Ureteritis · Cystitis · Urethritis
|
|
|
female: Oophoritis · Salpingitis · Endometritis · Parametritis · Cervicitis · Vaginitis · Vulvitis · Mastitis
male: Orchitis · Epididymitis · Prostatitis · Balanitis · Balanoposthitis
pregnancy/newborn: Chorioamnionitis · Omphalitis
|
|
|