Discussion
Herein, we described a case of TMA due to acquired complement factor I deficiency in a male receiving interferon-beta treatment for multiple sclerosis.
The immunomodulatory effect of interferon therapy has been recorded in the literature to generate autoimmune disorders, particularly with interferon-alpha, including examples of TMA in chronic myelogenous leukemia patients where high-dose treatment for a long period has been advised [5].
With IFNβ, only a few autoimmune signs have been documented even though it can cause flu-like symptoms, temporary laboratory abnormalities, menstrual problems, and local dermal injection site responses. IFNβ-induced TMA has been rarely reported [6].
A few mechanisms have been proposed to explain how interferon causes TMA, but insufficient immunological studies have been unable to pin this phenomenon down to a single pathophysiologic pathway [5, 7].
Some authors inferred that complement-mediated TMA was excluded due to normal complement levels [7]. However, serum complement factor C3 and C4 measurements have poor predictive value in determining the underlying pathophysiology of TMA [9]. Indeed, during most aHUS presentations, serum C3 and C4 levels are generally within reference values [10, 11].
Published literature identified 25 patients who developed TMA with renal impairment after receiving IFNβ as a disease-modifying treatment [1].
Orvain et al. [12] described a 52-year-old man who got TMA-associated severe renal failure due to severe ADAMTS13 insufficiency as a result of an anti-ADAMTS13 IgG antibody generated during IFN treatment for multiple sclerosis. Treatment included IFNβ discontinuation, immediate plasma exchange therapy, corticosteroids, and hemodialysis. Rituximab was introduced in face of hemolysis relapse.
The patient did not experience hematological relapse but remained dependent on hemodialysis.
Our case is very similar to that of Orvain et al. except that the clinical presentation of our patient was compatible with aHUS while the underlying pathophysiological mechanism was an acquired factor I deficiency in the context of IFNβ treatment for multiple sclerosis.
Due to its ability to destroy activated complement proteins C3b and C4b in the presence of cofactors, factor I is a critical inhibitor governing all complement pathways. Complete lack of factor I, which is mostly generated in the liver, is uncommon and results in excessive complement consumption, which can lead to repeated severe infections, glomerulonephritis, or autoimmune disorders. The incomplete factor I deficiency, as noted with our patient, is in turn associated with aHUS [13].
The factor I deficiency in the reported patient doesn’t seem important, this could be explained by the complement investigation after the instauration of the treatment but far from the plasma exchange sessions so as not to distort the result. unfortunately, exploration of the complement system was not done at the start of support.
In this report, two arguments support the hypothesis of an acquired incomplete deficiency of factor I due to antibodies generated by IFNβ. The first is the subsequent normalization of factor I level and the second is immunoglobulin deposition on kidney biopsy which indicates an underlying antibody-mediated process.
The state of autoimmunity secondary to IFNβ treatment is on another hand attested by the presence of anti-neutrophil cytoplasmic antibodies without specificity in our patient while Orvain et al. found antinuclear antibodies positivity [12].
Standard treatment with corticosteroids and plasma exchange was sufficient to control the hemolytic process in the reported case. Rituximab was necessary to control the situation in some cases reported in the literature [12, 14].