Schönlein‐henoch syndrome in patients with familial mediterranean fever

Abstract

Ten episodes of Schonlein-Henoch purpura (SHP) in 8 patients with familial Mediterranean fever (FMF) were observed. Five episodes developed 3-14 days after penicillin injections, suggesting an etiologic association. FMF and SHP have clear clinical similarities, and if the frequency of association of the 2 diseases is indeed high, perhaps a common etiologic factor should be sought. An immune complex mechanism might be the link between these 2 disease entities.Familial Mediterranean fever (FMF) and Schonlein-Henoch purpura (SNP) have a remarkable clinical similarity. This includes episodes of abdominal pains, arthritis, fever, skin eruptions, and transient or permanent renal damage. Little attention has been paid to the simultaneous occurrence of both diseases: only 20 cases have been described, most of them without details (1-3). Ten episodes of typical SHP were observed in 8 of 50 patients who were hospitalized in the Central Emek Hospital between 1966 and 1975 and diagnosed as cases of FMF. The purpose of the present study is to investigate the association of SHP and FMF. PATIENTSHospital charts of all the patients diagnosed as suffering from FMF between 1966 and 1975 were reviewed. Fifty cases fulfilling the following criteria were included in the present study (3); 1) recurrent attacks of fever and abdominal pains lasting 1-3 days, with or without joint pain; 2) an elevated erythrocyte sedimentation rate (ESR) and/or leukocytosis during the attack; 3) absence of other diseases that could explain the clinical picture; and 4) at least a 2-year duration of the disease.The diagnosis of SHP was based on the following criteria: 1) the appearance of nonthrombocytopenic puvura with 1 or several of the following: gastrointestinal tract bleeding, abdominal colicky pains, hematuria, localized edema, and arthritis; and 2) positive results of biopsies from affected skin (4 patients) and kidney biopsies (2 patients). CASE REPORTSRelevant clinical and laboratory data are presented in Table 1.Case 1 involved a 23-year-old Jewish man of Moroccan extraction. Two of his 5 siblings have FMF. At the age of 8 years, he was diagnosed as having FMF and, since then, he has experienced repeated abdominal and synovial attacks. In 1966, at the age of 9 years, he was hospitalized because of migratory polyarthritis (ankles, knees, elbows, hands) and high fever that lasted for more than 2 months and responded to corticosteroid treatment. Antistreptolysin-0 (ASLO) titers ranged between 1 :625-1:2500. The diagnosis was FMF, but the possibility of rheumatic fever could not be ignored. A year later, he was hospitalized once more because of migratory polyarthritis and high fever. One week after admission, he

MEFV mutations in Iranian Azari Turkish patients with Henoch–Schönlein purpura

“…Current findings were different from the result obtained by Nikibakhsh et al, in which a high rate of M694V mutations in HSP subjects was reported (15). In contrast to the current findings, two other studies conducted by Altug et al and Bayram et al revealed the lower mutation rate for M694V in the HSP patient group (12,13). However, none of the patients with HSP showed the M694V mutation in a Chinese population (12).…”

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“…Acute mono arthritis usually appears spontaneously [4]. Some types of vasculitis are more frequent in FMF, including Henoch-Schönlein purpura (HSP) and Polyarteritis nodosa (PAN) [5]. Physical and emotional stress, hormonal changes (estrogen reduction, menstrual cycle etc.)…”

“…cisplatin) may trigger the FMF attacks. Chronic and protracted manifestations, particularly nephropathic amyloidosis, leading to chronic renal failure, chronic arthritis and protracted myalgia, may also occur in the disease [1,2,5].…”

Imbalance of Interleukin-18 and Interleukin-18 Binding Protein in Children with Henoch-Schönlein Purpura

“…1 -4 Renal failure in HSP as a result of progressive nephritis is the primary cause of mortality. 5 HSP may occur either as a primary disease or secondarily to other inflammatory diseases, such as familial Mediterranean fever, 6 polyarteritis nodosa and Behçet's disease.…”