484 SEC TION III MUSCULOSKELETAL, SKIN, AND CONNEC TIVE TISSUE PATHOLOGY

Vasculitides
EPIDEMIOLOGY/PRESENTATION NOTES

Large-vessel vasculitis TAKAYASU EL GIGANTE

Giant cell (temporal)
arteritis
Takayasu arteritis
Medium-vessel vasculitis
Females > 50 years old.
Unilateral headache, possible temporal artery
tenderness, jaw claudication.
May lead to irreversible blindness due to
anterior ischemic optic neuropathy.
Associated with polymyalgia rheumatica.
Usually Asian females < 40 years old.
“Pulseless disease” (weak upper extremity
pulses), fever, night sweats, arthritis, myalgias,
skin nodules, ocular disturbances.
NUDOS DE KAWASAKI
Most commonly affects branches of carotid
artery.
Focal granulomatous inflammation A .
ESR. Realizar BIOPSIA
Treat with high-dose corticosteroids prior to
temporal artery biopsy to prevent blindness.
Granulomatous thickening and narrowing of
aortic arch and proximal great vessels B .
ESR.
Treatment: corticosteroids.

Buerger disease Heavy tobacco smoking history, males < 40 Segmental thrombosing vasculitis with vein and
(thromboangiitis years old. nerve involvement.
obliterans) Intermittent claudication. May lead to Treatment: smoking cessation.
Presentación clásica de la gangrene C , autoamputation of digits,
panarteritis nodosa superficial nodular phlebitis.
Raynaud phenomenon is often present.
Kawasaki disease Usually Asian children < 4 years old. CRASH and burn on a Kawasaki.
(mucocutaneous Conjunctival injection, Rash (polymorphous May develop coronary artery aneurysms E ;
lymph node  desquamating), Adenopathy (cervical), thrombosis or rupture can cause death.
syndrome) Strawberry tongue (oral mucositis) D , Hand- Treatment: IV immunoglobulin and aspirin.
foot changes (edema, erythema), fever. > 5 dias sin explicacion Excepcion para darlas en niños

Polyarteritis nodosa
Panarteritis nudosa clasica
Afecta las mesentericas
Small-vessel vasculitis
Behçet syndrome
Cutaneous small-
vessel vasculitis
Angitis cutánea leucocitoclástica
Usually middle-aged males. Typically involves renal and visceral vessels, not
Hepatitis B seropositivity in 30% of patients. pulmonary arteries.
Fever, weight loss, malaise, headache. Different stages of transmural inflammation
GI: abdominal pain, melena. with fibrinoid necrosis. Realizar BIOPSIA
Hypertension, neurologic dysfunction, Innumerable renal microaneurysms F and
cutaneous eruptions, renal damage. spasms on arteriogram (string of pearls
appearance).
Treatment: corticosteroids, cyclophosphamide.
incidence in people of Turkish and eastern Immune complex vasculitis.
Mediterranean descent. LEUCOCITOCLASTICA
CRIOGLOBULINEMICA
Associated with HLA-B51.
Recurrent aphthous ulcers, MICROSCOPICA
genital ulcerations,
uveitis, erythema nodosum. Can be
SCHOLEIN
WEGENER
precipitated by HSV or parvovirus. Flares last
STRAUSSS
1–4 weeks.
Occurs 7-10 days after certain medications Immune complex–mediated leukocytoclastic
(penicillin, cephalosporins, phenytoin, vasculitis; late involvement indicates systemic
allopurinol) or infections (eg, HCV, HIV). vasculitis. ANCA -
Palpable purpura, no visceral involvement.
Se presenta con púrpura palpable y lesiones urticarias que preceden a las lesiones purpúricas observadas más frecuentemente en las piernas.

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 MUSCULOSKELETAL, SKIN, AND CONNEC TIVE TISSUE PATHOLOGY SEC TION III 485

Vasculitides (continued)
EPIDEMIOLOGY/PRESENTATION NOTES

Small-vessel vasculitis (continued)

Eosinophilic Asthma, sinusitis, skin nodules or purpura, Formerly called Churg-Strauss syndrome.
granulomatosis with peripheral neuropathy (eg, wrist/foot drop). Granulomatous, necrotizing vasculitis with
polyangiitis Can also involve heart, GI, kidneys (pauci- eosinophilia G .
Síndrome de Churg-Strauss immune glomerulonephritis). BRONCOESPASMO MPO-ANCA/p-ANCA, IgE level. ANCA +

Granulomatosis with Upper respiratory tract: perforation of nasal Triad:

polyangiitis septum, chronic sinusitis, otitis media, Focal necrotizing vasculitis
Granulomatosis de Wegener mastoiditis. Necrotizing granulomas in lung and upper
Lower respiratory tract: hemoptysis, cough, airway BIOPSIA CON GRANULOMAS
dyspnea. Necrotizing glomerulonephritis
Renal: hematuria, red cell casts. PR3-ANCA/c-ANCA H (anti-proteinase 3). ANCA +

NARIZ EN SILLA DE CXR: large nodular densities.

MONTAR Treatment: corticosteroids in combination with
rituximab or cyclophosphamide. metotrexato
Immunoglobulin A Most common childhood systemic vasculitis. Formerly called Henoch-Schönlein purpura.
vasculitis Often follows URI. Vasculitis 2° to IgA immune complex
Púrpura de Schönlein Henoch Classic triad of Henoch-Schönlein purpura deposition.
Púrpura de Henoch Schönlein Hinge pain (arthralgias) Associated with IgA nephropathy (Berger
Stomach pain (abdominal pain associated disease).
with intussusception) Treatment: supportive care, possibly ANCA -
Palpable purpura on buttocks/legs I corticosteroids.
Microscopic Necrotizing vasculitis commonly involving No granulomas. = sin complejo inmunes
polyangiitis lung, kidneys, and skin with pauci-immune MPO-ANCA/p-ANCA J (anti-
Panarteritis microscópica glomerulonephritis and palpable purpura. myeloperoxidase). ANCA +
Presentation similar to granulomatosis with Treatment: cyclophosphamide, corticosteroids.
polyangiitis but without nasopharyngeal BIOPSIA SIN GRANULOMAS

involvement. Necrosis segmentarias + semilunas

Mixed Often due to viral infections, especially HCV. Cryoglobulins are immunoglobulins that
ANCA -
cryoglobulinemia Triad of palpable purpura, weakness, arthralgias. precipitate in the Cold.
Vasculitis crioglobulinémica esencial
May also have peripheral neuropathy and renal Vasculitis due to mixed IgG and IgM immune
disease (eg, glomerulonephritis). complex deposition.
A B LCC C D E
LM LCX
RSC

LAD

AAo

F G H I J

FAS1_2021_11-Musculo.indd 485 11/8/20 4:11 PM