Hair Dye as a Potential Trigger for Relapse of Anti-GBM Disease: A Case Report

Introduction

Anti-glomerular basement membrane (GBM) disease is a rare, monophasic, but serious autoimmune disease involving small vessels of the kidneys and lungs. It typically presents with rapidly progressive glomerulonephritis with or without pulmonary hemorrhage.¹ The mainstay of treatment is plasma exchange and prednisolone, with cyclophosphamide. Relapses are infrequent, but may be triggered by certain environmental irritants. This case reports an unusual relapse, which was successfully treated with a non-standard regimen of plasmapheresis, intravenous immunoglobulins (IVIG), and rituximab.

Case Report

A 43-year-old male, a food production industry worker, presented with fever, burning micturition, and AKI (creatinine 2.2 mg/dL). Despite antibiotics for presumed pyelonephritis, renal function worsened. Further testing revealed markedly elevated anti-GBM antibody titers (>200 U/mL), and the kidney biopsy showed crescentic glomerulonephritis with bright, linear IgG deposition [Figure 1]. The high-resolution computed tomography (HRCT) chest had no pulmonary involvement. He was treated with intravenous pulse methylprednisolone, plasmapheresis (14 cycles), and intravenous cyclophosphamide (2 doses). Though his anti-GBM titers decreased to 24 U/mL, he remained dialysis dependent.

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Figure 1:

Immunofluorescence picture of a kidney biopsy showing linear ribbon-like staining for IgG along the glomerular basement membrane, 400x.

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After using a new hair dye 2 months later, he was re-admitted with cough and hemoptysis. Anti-GBM titers rose to 269 U/mL, and HRCT chest revealed diffuse alveolar hemorrhage [Figure 2]. This confirmed a relapse of anti-GBM disease with lung involvement. He also had a central line-related bloodstream infection with high procalcitonin (84 ng/mL), which was treated with culture-sensitive antibiotics, and the TCC was replaced. Considering active infection and recent use of a strong immunosuppressant, he was managed with plasmapheresis and IVIG (1 g/kg). Even after five sessions of plasmapheresis, due to high anti-GBM titer (236 U/mL), he was administered two doses of rituximab (500 mg each - 15 days apart), which resulted in significant titer reduction (68 U/mL) and clinical improvement. He was discharged on maintenance hemodialysis with cotrimoxazole and acyclovir prophylaxis.

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Figure 2:

HRCT chest showing bilateral (right & left) patchy opacities suggestive of diffuse alveolar hemorrhage. HRCT: High-resolution computed tomography.

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Discussion

Anti-GBM disease is a small-vessel vasculitis caused by autoantibodies targeting the non-collagenous (NC1) domain of the α3 chain of type IV collagen.¹ The disease course is usually monophasic; relapses or recurrences are uncommon.^1,2 Risk factors associated with relapse include concomitant anti-neutrophil cytoplasmic antibody (ANCA) positivity or exposure to certain environmental toxins, including hair dyes.^1,2 Hair-coloring agents contain various aromatic amines that can injure the lung’s epithelial barrier and have been cited among potential triggers for Goodpasture syndrome.³ Povey et al. reported a similar case linking relapse to hair dye use.³ In our case, the new hair dye likely was the “second hit” that triggered the relapse. Patients recovering from anti-GBM disease should therefore be counseled to avoid known triggers (e.g., smoking, hydrocarbons, cocaine, and possibly hair dyes).

Management of relapsing disease poses challenges, especially with concurrent infection. We therefore used an alternate strategy comprising initially of plasma exchange and IVIG. Despite five plasma exchange sessions, antibody titers remained markedly elevated, and rituximab was introduced as it has shown efficacy in refractory or relapsing cases where standard cytotoxic therapy is contraindicated.⁴ Touzot et al. reported remission in 7/8 patients treated with rituximab with good renal survival at ∼2 years follow-up.⁵ Similarly, Povey et al. documented successful treatment of an anti-GBM relapse triggered by hair dye with plasmapheresis, steroids, and rituximab.³ In this case, pulmonary stabilization and reduction in antibody titer affirmed the efficacy of rituximab in controlling relapse.

This case illustrates an unusual relapse of anti-GBM disease precipitated by hair dye exposure, successfully managed with a combination of plasmapheresis, IVIG, and rituximab. It underscores the need for vigilance regarding potential triggers of relapse. It also highlights rituximab as a valuable therapeutic option in relapsing cases, where the standard cytotoxic therapy is contraindicated.