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2018 ; 97
(8
): e0004
Nephropedia Template TP
gab.com Text
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English Wikipedia
Primary cutaneous amyloidosis associated with autoimmune hepatitis-primary
biliary cirrhosis overlap syndrome and Sjögren syndrome: A case report
#MMPMID29465536
Yan X
; Jin J
Medicine (Baltimore)
2018[Feb]; 97
(8
): e0004
PMID29465536
show ga
RATIONALE: Primary cutaneous amyloidosis (PCA) is a localized skin disorder
characterized by the abnormal deposition of amyloid in the extracellular matrix
of the dermis. The association between PCA and other diseases, although rare, has
been documented for various autoimmune diseases. PCA associated with autoimmune
hepatitis-primary biliary cirrhosis (AIH-PBC) overlap syndrome and Sjögren
syndrome (SS) has not been previously reported in the literature. PATIENT
CONCERNS: A 50-year-old woman presented with progressive abnormal liver enzyme
levels and was referred to our department. DIAGNOSES: Due to the patient's
symptoms, laboratory test results, radiographic findings, and pathologic results,
she was diagnosed with PCA associated with AIH-PBC overlap syndrome and SS.
INTERVENTIONS: She was subsequently treated with a combination of ursodeoxycholic
acid (UDCA), prednisone, and azathioprine. OUTCOMES: While this treatment can
achieve therapeutic success, it cannot prevent complications from cirrhosis. This
patient remains alive but experienced an emergent gastrointestinal hemorrhage.
LESSONS: While we acknowledge that this is a single case, these findings extend
our knowledge of immunological diseases associated with PCA and suggest a common,
immune-mediated pathogenic pathway between PCA, AIH-PBC overlap syndrome, and SS.
After 12 years of follow up, clinical manifestations have developed, and these
autoimmune diseases have progressed. The combination of UDCA, prednisone, and
azathioprine can achieve therapeutic success but cannot prevent disease
progression. Routine follow up for this patient is necessary to document disease
progression.