Autoimmune polyglandular syndrome type III associated with antineutrophil cytoplasmic autoantibody-mediated crescentic glomerulonephritis

Abstract

Rationale: Polyglandular autoimmune syndromes (PAS) are a heterogeneous group of rare diseases characterized by the association of at least 2 organ-specific autoimmune disorders, concerning both the endocrine and nonendocrine organs. Type III is defined as the combination of autoimmune thyroid disease and other autoimmune conditions (other than Addison disease), and is divided into 4 subtypes. We describe a patient with Hashimoto thyroiditis, adult-onset Still disease, alopecia, vasculitis, antineutrophil cytoplasmic antibody (ANCA)-mediated crescentic glomerulonephritis, and hyperparathyroidism. Co-occurrence of these 5 diseases allowed us to diagnose PAS type IIIc. The rare combination of these different diseases has not been reported before. Patient concerns: A 51-year-old woman was admitted in April, 2019 after the complaint of an enlarged thyroid. She was diagnosed with Hashimoto thyroiditis at the age of 36. At age 40, she was diagnosed with an adult-onset Still disease. Three months before admission, she experienced renal insufficiency. After admission, she was diagnosed with hyperparathyroidism. Diagnosis: Renal biopsy revealed renal vasculitis and crescentic nephritis. Antineutrophil cytoplasmic autoantibody showed that human perinuclear ANCA and myeloperoxidase ANCA were positive. Therefore, the patient was diagnosed with vasculitis and ANCA-mediated crescentic glomerulonephritis. After admission, parathyroid single-photon emission computed tomography/computed tomography fusion image demonstrated the presence of hyperparathyroidism. Interventions: The patient was treated with high-dose methylprednisolone pulse therapy (0.1 g/d) for vasculitis and ANCA-mediated crescentic glomerulonephritis, calcium and vitamin D3 (600 mg/d elemental calcium [calcium carbonate] and 2.5 μg/d active vitamin D3) for hyperparathyroidism, and levothyroxine sodium (50 ug/d) for Hashimoto thyroiditis. Outcomes: Up to now, serum thyroid-stimulating hormone, total triiodothyronine, total thyroxine, free triiodothyronine, and free thyroxine were within the normal ranges. Patient's renal function did not deteriorate. Lessons: We report a patient with Hashimoto thyroiditis, adult-onset Still disease, alopecia, vasculitis, ANCA-mediated crescentic glomerulonephritis, and hyperparathyroidism, which is a very rare combination. We present this case as evidence for the coexistence of several different immune-mediated diseases in the clinical context of a PAS IIIc.

Hashimoto's thyroiditis, vitiligo, anemia, pituitary hyperplasia, and lupus nephritis—A case report of autoimmune polyglandular syndrome type III C + D and literature review

“…APS type III is the most common type of APS in adults ( 3 ). Tian et al ( 6 ) have well summarized the APS type III cases reported from 1989 to 2019, with a total of 64 relatively detailed cases. Our case of childhood APS type III C + D (HT, vitiligo, anemia, pituitary hyperplasia, SLE) has not been reported yet ( 3 , 6 , 7 ), and the specific mechanism is not fully elucidated.…”

Autoimmune polyglandular syndrome type 3: A case report of an unusual presentation and literature review

“… 1 , 5 There are several subtypes which are categorized based on other organ‐specific autoimmune involvement; type 3a exhibits TAD with type 1 diabetes, type 3b exhibits TAD with pernicious anemia (PA), and 3c exhibits TAD with vitiligo, alopecia, or other organ‐specific autoimmune disease. 1 , 5 , 6 APS type 4 is characterized as findings of two or more autoimmune endocrine organ destruction, which does not fit the other APS types. 1 …”

“…However, it may be prudent to keep this diagnosis in mind in patients with TAD as other studies have suggested that 52% of patients with TAD could fit an APS type 3 diagnosis. 5 , 6 Since there are no established guidelines for APS, we have highlighted important diagnosis and management information for the commonly involved organ‐specific autoimmune findings of TAD and pernicious anemia in APS type 3.…”

Síndrome poliglandular autoinmune tipo 3 con compromiso hipofisiario en una paciente latinoamericana

“…Los SPA generalmente se clasifican en cuatro subtipos (2). El SPA tipo 1 se caracteriza por el desarrollo de al menos dos de los tres componentes cardinales que comprenden candidiasis mucocutánea crónica, hipoparatiroidismo y enfermedad de Addison.…”