690 the Increased Frequency of Henoch Shoenlein Purpura in Familial Mediterranean Fever

Abstract

obscured because these terms a r e often used interchangeably. A l l cases of PC and OC with a positive blood,tissue a s p i r a t e , o r abscess culture seen a t Children's o r Eye and Ear Hospital i n the past 10years were analyzed. Results (including median age): TOTAL H.influenzae B(H1B) S.pneumoniae(SP) Other bacteria -PC 61 57% (15 mos) 18% (12 mos) 25% (6 y r s ) OC 10 0% 0% 100% (11 yrs) PC caused by HIB and SP, characterized by bacteremia, fever and p e r i o r b i t a l swelling, discoloration, induration and pain, is a d i s t i n c t clinicalentityoccurringinyoung chi1dren;sinus radiographs frequently demonstrated "cloudy" sinuses,but interpretation of the radiographswas complicated by the small sizeand i n d i s t i n c t bonymarginsof thesinuses o f i n f a n t s , andby concurrent upper respiratory infection and overlying s o f t t i s s u e swelling. Ophthalmoplegia and proptosis, never present i n cases of PC, always accompanied OCas did radiographic evidence of s i n u s i t i s . OC due t o HIBhasnot been documented i n t h i s study or i n the l i t e r a t u r e . PS secondary t o s i n u s i t i s i s characterized by non-tender, noninduratededema due t o venous congestion, not b a c t e r i a l c e l l u l itis. Careful d i f f e r e n t i a t i o n of PC,OC and PS secondary t o sinus i t i s is necessary f o r the selection of appropriate therapy. Familial Mediterranean fever (FMF) i s characterized by periodic attacks of fever and s e r o s i t i s , particularly p e r i t o n i t i s and synovitis. The disease i s transmitted by an autosomal recessive gene and primarily a f f e c t s Sephardic Jews, Arabs, Turks and Armenians. Duing the period [1970][1971][1972][1973][1974][1975][1976][1977][1978][1979] we observed seven children with FMF who developed HenochShoenlein purpura (HSP). During t h i s period 0.16% of 2140 pediatric admissions per year were due t o HSP. From a pediatric FMF population of 70 patients an average of 23 (33%) were admitted annually and one (4.3%) had HSP each year. Pertinent c l i n i c a l features a t t h e time of HSP -age range 3-13 years (mean 7 years) males: fernales 5:2. All patients had non thrombocytopenic purpura, a r t h r i t i s or arthlagia and fever. Five patients (70%) had diffuse abdominal pain and two boys had t e s t i c u l a r pain and swelling. Four patients (56%) had hematuria and one protei nuria, only. The erythrocyte sedimentation r a t e ranged from 50-150 mn/hr (mean 88) and fibrinogen 208-660 mg/dl (mean 373 mg/dl). The duration of hospitalization ranged from 13-150 days (mean 51 days). The association of FMF and HSP, two diseases of unknown etiology may provide new avenues of study in these diseases.PULMONARY PARAGONIMIASIS I N INDOCHINESE REFUGEE CHIL-691 DREN. 5.T. Shulman, R. Yogev, K. Burton and K . Boyer (spon. by Henry Nadler). Children's Memorial Hosp., Northwestern Univ., and Michael Reese Hosp., Chicago.The high prevalence of tuberculosis i n recent Indochinese refugees of a l l ages i s well documented, with 100-fold grea...

Coexistence of vasculitides with Familial Mediterranean Fever

“…Given that, most of the FMF-associated vasculitis cases had MEFV gene mutations, contributing role of these mutations for vasculitis development may be considered in the pathogenesis. In line with this view, our literature search showed that most of the FMF patients reported in literature with coexistent HSV or PAN had [7,9,10,13,14,65,66] Medium vessel vasculitic diseases Polyarteritis nodosa [7, 8, 11-13, 15, 70, 71] Large vessel vasculitic diseases Unreported Unclassified vasculitic conditions Protracted febrile myalgia [16,60,[72][73][74] Behçet's disease [17,18,79] Cutaneous vasculitis [87,88] either homozygous or compound heterozygous M694V mutations. However, since many FMF patients carrying these mutations do not have associated vasculitis, we admit that this coexistence cannot solely be explained by the presence of MEFV gene mutations.…”

“…Schlesinger et al [10] also reported this association from Israel in 1985. Then, Ozdogan et al [7] from Turkey retrospectively reviewed the charts of their series of 207 FMF patients and found out 15 patients with HSP, corresponding to the frequency of 7.2%.…”

“…Following isolated case reports of HSP or PAN associated with FMF published from 1982 to 1989 [10][11][12], Ozdogan et al [7] from Turkey performed the first retrospective analysis of the frequencies of both HSP and PAN in FMF in 1997. Then, other studies reporting the increased frequencies of HSP or PAN in both pediatric and adult FMF patients appeared in the literature [13,14].…”

Recurrent Hereditary Polyserositis or Familial Mediterranean Fever: An Overview

“…Joint involvement in children can masquerade as various other pediatric arthritides [26,45]. In 10 to 70% of cases, dermatological abnormalities are present; these consist most commonly of erysipelas-like lesions [6,12,46,47] which involve the legs, ankles, and dorsum of the feet. Other manifestations are nodular rashes, Schonlein-Henoch purpura [5,46,47], urticaria [6,47], bullous lesions [48], and a vasculitis which is usually associated with an immune-complex nephropathy [49].…”

“…In 10 to 70% of cases, dermatological abnormalities are present; these consist most commonly of erysipelas-like lesions [6,12,46,47] which involve the legs, ankles, and dorsum of the feet. Other manifestations are nodular rashes, Schonlein-Henoch purpura [5,46,47], urticaria [6,47], bullous lesions [48], and a vasculitis which is usually associated with an immune-complex nephropathy [49]. In addition, splenomegaly is present in about 30% of cases overall [11][12][13] and, like the arthropathy and dermatological lesions, is less frequent in Arab patients [18].…”