Polyarteritis nodosa and Henoch–Schönlein purpura nephritis in a child with familial Mediterranean fever: a case report

Abstract

Familial Mediterranean fever is an autosomal recessive disease characterized by recurrent self-limited attacks of fever accompanied by peritonitis, pleuritis, and arthritis. Approximately 5% of individuals with familial Mediterranean fever have been reported to have Henoch-Schonlein purpura and about 1% to have polyarteritis nodosa. A 7-year-old girl presenting with complaints of purpuric rash, abdominal pain, arthritis, hematuria, and proteinuria and having IgA depositions on renal biopsy was diagnosed as Henoch-Schönlein nephritis. She had a history of recurrent fever, abdominal and joint pain and M694 V compound homozygote mutation. Colchicine treatment was started for the diagnosis of FMF. When constitutional symptoms such as myalgia, weight loss, fatigue, fever, and hypertension were added to the clinical picture, the diagnosis of polyarteritis nodosa HSP was thought and confirmed by the demonstration of microaneurisms on renal arteries. There was no response to corticosteroid and cyclophosphamide treatments; however, the symptoms were rapidly and dramatically reduced after the administration of intravenous immunoglobulin. In conclusion, polyarteritis nodosa and Henoch-Schonlein purpura can be seen together with familial Mediterranean fever. It is also suggested that IVIG might be an important adjunct therapy in selected patients with polyarteritis nodosa, especially in the lack of response to steroids and immunsuppressive drugs.

Familial Mediterranean Fever

“…Based on the data of Turkish FMF study group (18), the incidences of HSP and PAN are 2.7% and 0.9%, respectively. There was a case report in which the concomitance of HSP and PAN responding to intravenous immunoglobulin therapy was reported in a pediatric FMF patient (50).…”

Overlap Syndrome between Familial Mediterranean Fever and Tumor Necrosis Factor Receptor-Associated Periodic Syndrome in a Lupus Patient

“…Additionally, heterozygous mutations in exon 2 of the MEFV gene act as disease-severity modifiers in inflammatory diseases such as arthritis (Lachmann et al 2006). Autoinflammatory gene mutations have also been described in patients affected with vasculitis; autoinflammatory gene mutations in these patients are known to modify disease severity, response to agents, and/or clinical manifestations (Atagunduz et al 2003;Girisgen et al 2012). SLE is caused by multiple genetic and environmental factors.…”

Intravenous Immunoglobulin Therapy in Primary Vasculitides

“…In Henoch-Schönlein purpura some authors (Heldrich et al, 1993, Rostoker et al, 1995, Ruellan et al, 1997) administered low-dose or lower limit of high-dose regimens, others (Rostoker et al, 1994, Lamireau et al, 2001, Fagbemi et al, 2007 strictly apply 2 g/kg. Apart from the dose applied all authors emphasize dramatic improvement of IVIG treatment already after 24 hours after beginning (Girisgen et al, 2011). It could be supposed that patients who respond to high-dose IVIG therapy would probably also respond to much lower doses, in many rheumatological indications vasculitides not excluded.…”

“…Asano et al (2006) postulated that the effect of IVIG therapy on PAN is temporary, but in situation that PAN is induced by infections, IVIG therapy leads to a complete remission of the disease due to the neutralization of immune activation triggers, such as Parvovirus B19 and Streptococcus. Also Girisgen et al (2011) based on the case report of 7 years old girl with polyarteritis nodosa and Henoch-Schönlein purpura nephritis suggested that IVIG might be an important adjunct therapy in selected patients with polyarteritis nodosa, especially in the lack of response to steroids and immunosuppressive drugs. On the contrary González-Fernández & García-Consuegra (2007) published the case-report of a child with polyarteritis nodosa that was unresponsive to conventional treatment, as well as IVIG treatment during her first and second hospitalizations.…”