Basics of a Blood Vessel & Vasculitis😍

Renal artery stenosis

• Affects the ostium.

• Young patient (without a family history of hypertension)

• 1st investigation is renal Doppler (Image 1)
• has a slow and blunted uptake.
• i.e. pulsus parvus et tardus waveform (Image 2)
• Normal renal artery has a quick upstroke.

• 2nd investigation is CT-angiography or MR-angiography.

• Gold standard procedure is digital subtraction angiography (DSA).

Case scenario

• A 4-year-old child presents with fever and rash for 6 days, along with red eyes and tongue and swelling of hands.

• Age: Typically occurs in children less than 5 years of age.

Diagnostic Criteria (CRASH BURN criteria):

• Fever >39°C for more than 5 days + at least 4 of the following:

• At least four of five criteria: CRASH
• Conjunctivitis (B/L Non Purulent)
• Rash → Polymorphous
• Adenopathy (non tender)
• Strawberry tongue
• Red, fissured lips
• Note: Strawberry tongue also in scarlet fever.
• Hands and feet Erythema that later leads to desquamation → edema

Pathogenesis:

• Associated with anti-endothelial cell antibodies.

Blood Test Findings:

• Thrombocytosis (increased platelet count).

• Leukocytosis, elevated ESR or CRP.

Complications:

• M/c → Myocarditis

• Most dangerous
• Coronary artery aneurysm
• Giant >8mm (>1cm) in diameter
• Risk → resemble infection
• Male sex
• Fever ≥14 days
• Elevated ESR and CRP
• WBC count >12,000/mm³
• Hematocrit (PCV) <35%
• Serum sodium <135 mEq/L

• Can lead to aneurysm or myocardial infarction (heart attack) in child

• Rare but potentially fatal

Treatment

• Aimed at preventing coronary artery aneurysm
• IVIG
• if given within first 10 days, reduces the risk of coronary artery aneurysm
• Corticosteroids
• if persistent fever despite IVIg
• ASPIRIN
• High dose aspirin reduces risk of thrombosis, given until subside fever
• Low dose aspirin is given until ECHO is performed at 6 weeks to exclude aneurysm
• Reye’s syndrome
• Acute Liver Failure
• Microvesicular steatosis

Vasculitis - Henoch-Schönlein Purpura (HSP)

Case Scenario

• A child presents with abdominal pain, hematuria, and palpable purpura over the leg.

• Small vessel vasculitis

• Often preceded by infections/drugs

Diagnostic criteria

• Palpable purpura with presence of 1 or more of:
• Diffuse abdominal pain
• Arthritis or arthralgia
• Any biopsy showing IgA deposition
• Renal involvement

Organs affected (PAARpura):

• P → Purpura (commonly on lower limbs).

• A → Arthralgia.

• A → Abdominal pain.
• Complication - intussusception
• Submucosal hematoma
• Main indication for giving steroid → GI bleed

• R → Renal involvement (e.g., hematuria).
• Kidney is the most common affected organ.
• Glomerulonephritis is seen in 1/3 of patients.
• Proteinuria
• haematuria,
• RBC casts

Biopsy:

• Leukocytoclastic vasculitis with IgA-containing immune complexes

Skin Presentation:

• Palpable purpura (felt)
• Thrombocytopenia is Absent.

• Lower legs and buttocks

• Painful

Treatment:

• Most of the cases are self-limiting.

• if not self-limiting
• DOC: Corticosteroids
• Dapsone
• Colchicine

NOTE:

• Palpable purpura → Vasculitis

• Non palpable purpura → Bleeding, clotting, thrombocytopenia

Granulomatosis with Polyangiitis (GPA)
(formerly Wegener's Granulomatosis)

• Necrotizing vasculitis with granulomas.

• Strawberry gingiva.

• ENT Triad 
• Ears: Otitis media.
• Nose: Nasal septal perforation / Saddle nose
• Throat: Strawberry gums

• Upper respiratory tract (URT) involvement:
• Recurrent sinusitis.
• Epistaxis.

• Lower respiratory tract (LRT) involvement:
• Hemoptysis.
• Cavitations (due to lung blood supply damage).

• Kidney:
• Hematuria.
• A/w
• RPGN Type 3 (with Microscopic polyangitis)
• PAUCI IMMUNE
• Flea bitten kidney

• Note:
• In TB, cavity in upper lobe of lungs.
• In this condition, multiple small cavities due to necrotizing vasculitis.

• Workup:
• Shows both C-ANCA > P-ANCA.
• against cytoplasmic antigen → anti-proteinase 3

• Treatment:
• Cyclophosphamide + Mesna

• Differentiation from Goodpasture syndrome:
• Goodpasture:
• Hemoptysis + hematuria (basement membrane damage in lung and kidney).
• Wegener’s:
• Lung cavitation + URT involvement + hematuria

Cavernous Hemangioma

• M/c benign tumour of liver.

• Association: 
• VHL Syndrome (Von Hippel-Lindau Syndrome).
• Involves chromosome deletion 3P.

• Clinical: 
• Deep-seated (organs like liver).
• Usually asymptomatic.

• Microscopy:
• Shows very large and dilated blood vessels.

• CT:
• Peripheral nodular enhancement.

• No surgical intervention required.

• Mnemonic: Hemand (Hemangioma) oru deep large cave (cavernous) il poi, 3 Pakal (3p) thamasichu → Very hot locationil (VHL)

Pyogenic Granuloma

• Misnomer: 
• Not associated with pus/bacteria (pyogenic); 
• does not show granulomas.

• Other Names:
• Granuloma Gravidarum (partially correct, seen in pregnancy); 
• Pregnancy Tumor (most accurate).

• Clinical: Seen in pregnant ladies (reddish lesion).
• Oral cavity or fingertips.

• Microscopy: Known as Lobular Capillary Hemangioma (LCH).
• Tiny capillaries arranged in lobules/groups.

• Location: Always on nail beds (subungual).

• Symptom: Excruciating pain (pain to the power infinity).

• Origin: Arises from glomus bodies (arteriovenous anastomoses at fingertips).
• Regulate temperature (thermoregulation).

Kaposi Sarcoma

• Cause: Human Herpes Virus 8 (HHV-8).

• Association: Seen in HIV-positive individuals

• Gamma virus

• Most Common Sites:
• First: 
• Seen on legs
• Presents with deep red to bluish papules and nodules
• Second: Lymph node.

• M/c lid malignancy in HIV

• Microscopy:
• Shows spindle cells.

• IHC (Immunohistochemistry): 
• LANA (latency associated nucleic acid) positive.

• Kakkusil pokumbo sing LALA (LANA) and SPIN (Spindle)

Angiosarcoma

• Most Common Organ:
• Most commonly in the liver.

• Most common cause for malignant cancer of heart in adults

• Causes: VAT chemicals:
• V: Vinyl chloride (plastics, polyvinyl chloride).
• A: Arsenic (pesticides).
• T: Thorotrast (thorium-based contrast dye, old radiology use).
• Thorotrast,
• Thorotrast linked to
• HCC,
• cholangiocarcinoma &
• renal cell carcinoma.
• Angiosarcoma (VAT → Plastic)

• Histology:
• Anastomosing vascular channels.
• Highly pleomorphic cells.

• IHC markers:
• von Willebrand factor.
• Factor VIII.
• VEGF.
• CD31 (PCAM)

• Mnemonic: Plastic (Plastic industry) ittu Vaattan (VAT) poyapo Liveril blood cancer vannnu

 

• Systemic vasculitis

• Anti alpha enolase Ab

• Pathergy → Pathetic diagnosis → misunderstood young patient with recurrent oral and genital ulcers

• Misunderstood a young as 51 () yr old nolan (Enolase) in bus ()

Triad of Features

• Relapsing uveitis (mainly posterior)

• Recurrent genital ulcers

• Recurrent oral ulcers

Features (Oro-Oculo-Genital Syndrome)

• Most common diagnostic feature:
• Recurrent oral ulceration.
• Painful, like aphthae, recurrent
• Recurrence at least thrice in any 12 months.

• PLUS TWO OF:
• Genital ulcers:
• Female: Vulva ulcer
• Male: Scrotal ulcer
• not penile ulcer as in STDs like H. ducreyi, syphilis
• Eye lesions:
• Anterior uveitis.
• Posterior uveitis.
• Hypopyon
• Retinal vasculitis
• Skin lesions:
• Erythema nodosum
• Pseudofolliculitis or papulopustular lesions, OR
• Acneform nodules in post-adolescent patients not on corticosteroids.
• Positive pathergy test

Other Systemic Involvement:

• Non-erosive, asymmetric oligoarthritis

• Multi-system: Pulmonary, cardiac, GI, neurological

Pathergy Test/ Pathergy phenomenon:

• Positive because it can cause systemic vasculitis

• Not a diagnostic criteria

• Type 4 Hypersensitivity reaction

• Procedure:
• Sterile needle puncture on forearm with hypodermic cutting edge.

• Normal response:
• bleeding, clotting, mark disappears in 2-3 days.

• Behcet’s response:
• After 48 hours, formation of sterile pustule

Complications:

• Pulmonary artery aneurysm possible.
• Rupture: Lethal complication.
• Note: Pulmonary artery not involved in polyarteritis nodosa.

Management:

• Corticosteroids

• Ocular involvement:
• Azathioprine.

Mnemonic:

• Bus on a path → Pathergy test

• Oro occulo genital → On bus