Immunoglobulin A Vasculitis Complicated with Clostridium difficile Infection: a Rare Case Report and Brief Review of the Literature

Abstract

Immunoglobulin A (IgA) vasculitis, formerly called Henoch-Schönlein purpura, is a leukocytoclastic type of vasculitis affecting small vessels with a deposition of immune IgA complexes, clinically characterized by the classic tetrad of nonthrombocytopenic palpable purpura, arthralgia (or arthritis), and gastrointestinal and renal involvement. Although the cause of the disease remains unknown, immune complexes of IgA and unidentified antigens seem to play a central pathogenic role. The diagnosis is easily established in the presence of purpura, but may be challenging in its absence, especially when colicky abdominal pain precedes the cutaneous lesions. IgA vasculitis is usually a self-limited disease with a benign course and symptomatic treatment is sufficient for most; in severe cases, however, corticosteroids are necessary. We describe the case of a young adult male presenting with severe abdominal pain, vomiting and fever (38.4ºC). Clinical examination, abdominal ultrasound and plain abdominal radiography excluded an acute abdomen. The occurrence of arthralgia involving both knees and erosive duodenitis at endoscopy, 48 hours upon admission, suggested the diagnosis of IgA vasculitis, confirmed on the following day by the presence of typical purpuric rash on the lower extremities. Corticosteroid therapy led to the resolution of all gastrointestinal and joint manifestations as well as to a significant improvement of cutaneous purpura. However, during the 3rd week of corticosteroid treatment, the patient developed watery diarrhea and the clinical suspicion of Clostridium difficile infection (CDI) was confirmed. The treatment with metronidazole led to the resolution of diarrhea.The peculiarity of this case resides in several aspects: the gastrointestinal and joint manifestations preceded purpura, making diagnosis more difficult; CDI is an extremely rare complication of IgA vasculitis, being, in fact, the second case reported in adults in the literature.

ANCA Vasculitis and Hemophagocytic Lymphohistiocytosis following a Fecal Microbiota Transplant

“…Cases of patients with CDIs and vasculitic processes have been reported; however, it remains relatively unclear as to whether CDIs serve as a clear trigger, risk factor, or consequence of vasculitis [ 6 – 9 ]. The majority of reported cases within this realm describe CDIs in previously diagnosed Henoch-Schönlein purpura (HSP) or IgA-associated vasculitis [ 7 – 9 ]. One case report describes a CDI which may have triggered a limited form of granulomatosis with polyangiitis to evolve systemically within a time course of two weeks [ 5 ].…”

Gastrointestinal Manifestations of IgA Vasculitis-Henoch-Schönlein Purpura

“…The classic tetrad symptoms are rash, arthralgia/arthritis, abdominal pain, and renal manifestations. The clinical diagnosis is easily made in the presence of all these symptoms but may be omitted when the clinical picture is incomplete; in the absence of the classic purpuric rash, the diagnosis of Ig A vasculitis may not be obvious [14,15].…”

“…Gastrointestinal involvement occurs in 50-75% of adult patients-abdominal pain and bloody diarrhea may precede the typical purpuric rash [15,23]. The digestive symptoms are often present in patients with IgA vasculitis, and although less commonly found in adults, they are more severe and often atypical.…”

Infection‐associated vasculitides

“…Several bacteria have been implicated in the pathogenesis of different categories of infection‐associated vasculitides. IgA vasculitis has been reported to occur in association with various bacteria, including Clostridium difficile and Mycoplasma pneumoniae . Aerococcus viridians , Listeria monocytogenes , Ureaplasma urealyticum and Burkholderia cepacia have been incriminated in cutaneous LCV.…”