Hypocomplementemic Urticarial Vasculitis.Case Report.

2. Hypocomplementemic Urticarial
Vasculitis.Case Report.
• Azad A. Haleem1 , Mehvan Sh. Abdulrahman2
,Aziz B Abdulaziz3
• 1.Pediatrician &Lecturer at college of Medicine, University of
Duhok/Kurdistan/Iraq.
2.Pediatric SHO in Heevi pediatrics teaching hospital, Duhok/Kurdistan/Iraq.
3. Pediatrician in Amedi hospital Duhok/Kurdistan/Iraq

4. Case Summary
Elevent years old girl ………
• Presented with frequent attacks of
spontaneous Urticarial rash on the trunk and
extremities when she was three years old,
• firstly diagnosed as a case of allergy treated
by Antihistamine and sort course of steroids
but unfortunately without benefits.

5. • After four months she developed urticarial
rash and painful swelling of the joints in the
upper and lower extremities, for that she
received one course ( one month ) of
prednisolone without knowing the definitive
diagnosis became better but after stopping
prednisolone the condition reappeared again.

6. • At the age of four years they consulted a
doctor who diagnosed her as a case of SLE
depending on clinical features and simple labs
test and the patient received Methotrexate
and NSAID, on treatment the patient became
well for one year.
• Then the family stopped the treatment
suddenly because of family problem and
economic problems.

7. • After one month the patient develop
hematuria, hypertension and renal
impairment admitted to hospital on
conservative management ; Prednisolone,
Amlodipin and Hydroxychloroquine.
• after discharge again the patient received
Methotrexate till the age of 7 years.

8. • After that the patient did not become well
with recurrent attack of urticaria, arthritis,
and lower respiratory tract infections with
frequent admissions to hospital.

9. • At the age of 8.5 years one doctor alter the
diagnosis to Polyarteritis nodosa and put her
on Azathioprine and small dose of
prednisolone, but unfortunately she
deteriorated more and did not respond to
treatment.

10. • At the age of 9 years another doctor changed
the diagnosis toward Churg-strauss disease
and put the patient on Cyclophosphamide but
without benefit.

11. • At the age of 10 years the patient admitted to
Heevi pediatric hospital because of lower
respiratory tract infection and hematuria,
again unfortunately she had renal failure and
bronchiectasis for which she was treated in
ICU , became better after three weeks of
intensive treatment.

12. On further enquiry
• By complete assessment of patient regarding
history, physical examination and farther
investigations , depending on clinical criteria
and lab tests including serum complement
level and renal biopsy.

14. Diagnosis…..

15. Treatment:
• She was treatment with hydroxychloroquine
and Azathioprine with close observation and
follow up.
• Now she is well …

16. Conclusion
• This case illustrates a young female with
Hypocomplementemic Urticarial Vasculitis
treated by many doctors in different places
with variety of medications with wrong
diagnoses.

17. Some theory …..

18. Vasculitis
• What is Vasculitis?
• The term vasculitis means inflammation of
the wall of blood vessels. Various sizes of
blood vessels may be involved.

19. How Common is Vasculitis in Children?
• Vasculitis are rare in the paediatric age group
with varied incidence.
• Although many vasculitides affect both adults
and children, some such as KD occur almost
exclusively in childhood.
• On the other hand, temporal arteritis does
not occur in children.

20. How Common is Vasculitis in Children?
• Only Henoch Schonlein purpura (HSP) and
Kawasaki disease (KD) are more common in
children than in adults, with each one
affecting at least 10–20/100,000 pediatric
patients per year.
• Systemic polyarteritis nodosa (PAN) is also
rare in childhood with an estimated incidence
of one per one million children.

21. How is Vasculitis Classified in Children?
• This schema uses vessel size for classification purposes
• The aorta and its main branches are termed large vessels;
• The first branches of the aorta e.g., renal, mesenteric, coronary
vessels are regarded as mid-sized;
• arterioles, capillaries and venules are regarded as small-sized;
granulomatous and non-granulomatous varieties.

22. New classification of childhood
vasculitides
Predominantly large
vessel vasculitis
Takayasu arteritis
Predominantly medium
sized vessel vasculitis
Childhood polyarteritis nodosa
Cutaneous polyarteritis (C-PAN)
Kawasaki disease
Predominantly small
vessel vasculitis
(A) GRANULOMATOUS
Wegener’s granulomatosis
Churg-Strauss syndrome (CSS)
(B) NON-GRANULOMATOUS
Microscopic polyangiitis
Henoch-Schonlein purpura
Isolated cutaneous leucocytoclastic vasculitis
Hypocomplementemic urticarial vasculitis
Other vasculitides
Behcet disease
Vasculitis secondary to infection (including hepatitis B associated
polyarteritis nodosa), malignancies, drugs, including
hypersensitivity vasculitis
Vasculitis associated with connective tissue diseases
lsolated vasculitis of the central nervous system
Cogan syndrome

23. When Should a Pediatrician Suspect
Vasculitis in a Child?
• As a general rule, it would be appropriate to
suspect vasculitis when confronted with
multi-organ manifestations.
• in the presence of constitutional features such
as fever, weight loss, fatigue, myalgia
• with the presence of inflammatory markers
such as anemia, leukocytosis, thrombocytosis
and a raised erythrocyte sedimentation rate
(ESR).

24. Multisystem manifestations of systemic vasculitis
Organ/system
involved
Manifestation
Skin and mucosa Oral / nasal ulcers ,Exanthema, Livedo reticularis, Purpura,
Panniculitis, Nodules, Edema, Superficial ulcers, Deep ulcers,
Gangrene
Joints and Muscles Arthralgias / it is, Myositis
Kidneys and Genitals Hypertension, Hematuria, Proteinuria, Epidydymoorchitis
GI Tract Abdominal pain /colic, GI bleed, Gut infarction/, perforation,
Intussusception
ENT Epistaxis, Nasal septal perforation, Pan sinusitis /chronic ear
discharge, Sudden onset deafness, vertigo, Subglottic stenosis
Heart Myocardial infarction, cardiomyopathy ,Pericarditis, valvular
incompetence, bruits, Absent pulses,Hypertension, Discrepant
blood pressure, Claudication, Raynauds
Brain and Nerves Stroke, meningo-encepahilitis,cranial nerve palsy,paraparesis,
focal deficits, Mononeuritis multiplex
Eyes Painful red eye, Uveitis, keratoconjunctivitis, Sudden blindness,
Fundoscopic abnormalities e.g. exudates, hemorrhage
Lungs Tracheal stenosis, Hemoptysis, pulmonary hypertension, Nodules,
cavities, infiltrates Asthma , serositis

25. Investigative Approach for a Child with
Vasculitis
• Radiology
• Digital Subtraction Angiography (DSA)
• MR Angiogram (MRA)
• Computerised Tomography (CT) and
Ultrasonography (USG)
• 2 D Echocardiogram
• Serology
• Histology - Biopsy

26. Differential Diagnoses
Infections HIV, Hepatitis B virus, Hepatitis C virus, Hepatitis A virus,
Mycobacterium, Herpes viruses, Infective
endocarditis , Mycotic aneurysms, Protozoa
Inherited disorders Marfan’s syndrome, Ehlers Danlos syndrome type IV, Pseudoxanthoma
elasticum, Neurofibromatosis type I,
Fibromuscular dysplasia, Moyamoya disease
Drugs /toxins Antibiotics (penicillins, aminopenicillins, sulfonamides), thiazides,
warfarin/coumarin derivatives, NSAIDs,
hydantoins
Hypercoagulable
states
Antiphospholipid antibody syndrome
Malignancies Leukemias, lymphomas
Vasospastic
disorders
Reversible cerebral vasoconstriction syndrome, Reversible posterior
leukoencephalopathy syndrome
Multisystemic
inflammatory disorder
Sarcoidosis
Immunodeficiency
disorders
Common variable immunodeficiency, HLA class 1 deficiency
Miscellaneous Segmental arterial mediolysis, Cardiac myxoma, post radiation therapy

27. How are Children with Vasculitis
Managed?
• The management of vasculitis is dependent upon the
diagnosis, the nature of symptoms and the extent of
organ involvement .
• From a quiet “wait and watch” or
• symptomatic therapy to the use of steroids alone or in
combination with immunosuppresive agents such as
cyclophosphamide or azathioprine, decisions are
largely dependent on vessel size and which vascular
bed is involved.
• Large vessel disease (TA) may be approached by a
combination of drug therapy and invasive radiologic
procedures such as dilatation or stenting.

29. Some theory …..

30. Hypocomplementemic urticarial
vasculitis (HUV)
• Hypocomplementemic urticarial
vasculitis (HUV) is a rare form
of vasculitis (small blood vessels) and low
levels of complement proteins in the blood.
• HUV causes recurrent episodes of urticaria
and systemic, multiorgan involvement, causing
arthritic joint pain; pulmonary (lung) disease;
ocular (eye) inflammation; kidney
inflammation; or various other symptoms.

31. Etiology
• Both genetic and environmental factors are
thought to play a role in causing HUV.
• It generally occurs sporadically, but familial cases
have been reported.
• It is thought to develop due to an
abnormal immune system response.
• In some cases HUV may be associated with an
underlying infection or systemic diseases such as
systemic lupus, Sjögren's syndrome, monoclonal
gammopathy, or blood disorders.

32. Clinical features
Organ % Clinical features
Skin 100% Urticarial exanthema, urticarial vasculitis, palpable
purpura, angioedema
Joints 70% Arthralgia and arthritis
Kidneys 50% Proteinuria, hematuria, renal insufficiency, rapid
progressive glomerulonephritis (RPGN)
Gastrointestinal
tract
30% Abdominal pain, nausea, vomiting, diarrhea,
hepatomegaly, splenomegaly, ascites, serositis
Lungs 20% Shortness of breath, coughing, hemoptysis, pleural
effusion, chronic obstructive pulmonary disease
(COPD)
Eyes 10% Episcleritis, uveitis, conjunctivitis
Heart Rare Pericarditis, cardiac valve disease
Nervous system Rare Pseudotumor cerebri, aseptic meningitis, central or
peripheral nerve palsy
Provided by : Human Phenotype Ontology (HPO)

33. Criteria for diagnosis of HUVS
• Major criteria
– Chronic urticarial exanthema (at least 6 months)
– Hypocomplementemia
• Minor criteria
– Dermis (diagnosed by biopsy) Leukocytoclastic vasculitis
– Arthralgia and arthritis
– Uveitis or episcleritis (or conjunctivitis)
– Glomerulonephritis
– Abdominal pain
– Positive C1q antibody.
A diagnosis of HUV requires the presence of the two
major criteria, as well as at least two minor criteria.

34. Treatment
• The appropriate treatment of UV and HUVS is
determined by the severity of the disease.
• Antihistamines represent the cornerstone of treatment
for patients who have UV with only cutaneous lesions,
serving to control the itching.
• Glucocorticoids (GC) are the agents most frequently
employed to combat the inflammation and immune
complex formation.
• Hydroxychloroquine.
• Immunosuppressives are combined with GC, including
methotrexate, azathioprine, cyclophosphamide,
cyclosporin, and mycophenolate mofetil.

35. Prognosis
• HUV varies from person to person.
• The prognosis largely depends on severity of
systemic involvement and is influenced
primarily by the severity of lung (pulmonary),
heart, and kidney (renal) disease.
• Although HUV is uncommon in childhood, the
prognosis is worse for those affected at
younger ages because of more frequent,
severe renal involvement.

36. THANKS FOR YOUR
ATTENTION