שיטות קלסיפיקציה חדשות לוסקוליטיס בילדים :

שיטות קלסיפיקציה חדשות לוסקוליטיס בילדים :

A new international classification of childhood vasculitis.

Pediatric nephrology:2006 vol 21:9 pg:1219 -22

Michael J. Dillon . Seza Ozen

הגדרת מחלות וסקוליטיות בילדים נבעה לרוב מההגדרות של מחלות אילו במבוגרים. כמובן שיש הבדל ניכר בין המחלות בילדים ובמבוגרים , מה גם שאין תמימות דעים לגבי צורת הקלסיפיקציה במבוגרים. לפיכך התכנסו בוינה לפני כשנה הרופאים והגופים המובילים בתחום וניסו לייצר קלסיפיקציה חדשה ועדכנית. שיות העבודה היתה לפי - טכניקת דלפי שבה נאספו מגוון גדול מכל ספקטרום הדיעות של רופאים בתחום ועל סמך דעות אילו גובשה ההצעה.

שיטת הקלסיפיקציה שהוצעה:

בכלליות מדובר על גודל כלי הדם הנגועים כבסיס לקלסיפיקציה עם תת-חלוקות לפי הצורך :

I Predominantly large vessel vasculitis

                       Takayasu arteritis 

II Predominantly medium-sized vessel vasculitis

                        Childhood polyarteritis nodosa

                        Cutaneous polyarteritis

                        Kawasaki disease

III Predominantly small vessel vasculitis

                A  Granulomatous

                             Wegeners granulomatosis

                             Churg-Strauss syndrome

                B Non-granulomatous   

                             Microscopic polyangiitis

                             Henoch-Schönlein purpura

                             Isolated cutaneous leukocytoclastic vasculitis

                             Hypocomplementaemic urticarial vasculitis

  IV Other vasculitides 

                         Behçets disease 

                         Vasculitis secondary to infection (including hepatitis B-associated

                         PAN), malignancy and drugs including hypersensitivity vasculitis

                         Isolated vasculitis of the CNS

                         Cogan’s syndrome

                         Unclassified

הועדה הוסיפה מאפיינים עבור כל וסקוליטיס (והן מובאות כלשונן למניעת בלבול):

HSP- at least one of the following four should be present

                   Diffuse abdominal pain

                   Any biopsy showing predominant IgA deposition

                   Arthritis or arthralgia  

                   Renal involvement-any haematuria and/or proteinuria in the presence of

                                                  palpable purpura which was a mandatory criterion

KD – fever persisting for at least 5 days - mandatory criterion

                   And four of the following five being present  

                      Changes in theperipheral extremities or perianal area

                      Polymorphous exanthema

                      Bilateral conjunctival injection

                      Cervical lymphadenopathy

                      Changes in the lips and oral cavity “and/or” injection of the oral and

                                                                                           pharyngeal mucosa.

PAN - presence of a biopsy showing small or mid-size artery necrotizing 

                       vasculitis or angiographic abnormalities (requirement)    

            And two of the following seven criteria

                       Skin involvement (livedo reticularis, tender subcutaneous nodules other

                                                                                                            vasculitic lesions

                       Myalgia or muscle tenderness

                       Systemic hypertension relative to childhood normative data

                        Mononeuropathy or polyneuropathy                 

           Abnormal urine analysis and/or impaired renal function

           Testicular pain or tenderness

           Signs or symptoms suggesting vasculitis of any other major organ

            system (gastrointestinal,cardiac,pulmonary or central nervous system(

Cutaneous polyarteritis -  presence of subcutaneous nodules- painful non-

                                            purpuric lesions with/ without livedo reticularis

                                            but without systemic involvement

)                                            except for myalgia, arthralgia and non-erosive

                                                                                                            Arthritis(.

                                            Skin biopsy- necrotizing,non-granulomatous

                                                                                                          vasculitis

                                           ANCA tests - negative

                                           Association with serologic and microbiologic

                                                            evidence of streptococcal infection.

Microscopic polyangiitis- a necrotizing pauci-immune vasculitis affecting

                                            predominantly small vessels.

                    Often associated with :

                                            High titre of myeloperoxidase (MPO(

                                            ANCA or positive perinuclear ANCA staining

Wegeners granulomatosis -  Three of the following six criteria :

                                                  Abnormal urinalysis

                                                  Granulomatous inflammation on biopsy

                                                  Naso-sinus inflammation

                                                  Subglottic, tracheal or endobronchial stenosis

                                                  Abnormal chest X-ray or

                                                                            computed tomography (CT(

                                                  PR3 ANCA or cytoplasmic ANCA staining

Takayasu arteritis -  at least one of the following should be present :

            Hypertension (related to childhood normative data) in

                                                       the presence of angiographic abnormalities

                                                       conventional,(CT or MR) of the aorta or its  major

                                                       branches (a mandatory criterion)
                                                       Decreased peripheral artery pulse(s) and/or
                                                       claudication of the extremities

                                                       Bruits over the aorta and/or its major branches                                                         

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