ABSTRACT
The association between microscopic polyangiitis (MPA) and primary biliary cirrhosis (PBC) has seldom been reported. We describe here a patient who presented with sensorimotor neuropathy along with hypothyroidism, renal failure and liver dysfunction. Detection of antinuclear antibodies at a titer of 1/800, anti-SSA, anti-SSB, anti-GP210, anti-microsomial and p-ANCA anti-myeloperoxydase antibodies along with renal, salivary and liver biopsy led to a diagnosis of MPA associated with PBC, Sjogren's syndrome and Hashimoto's thyroiditis.
Subject(s)
Hashimoto Disease/complications , Liver Cirrhosis, Biliary/complications , Microscopic Polyangiitis/complications , Sjogren's Syndrome/complications , Antibodies, Antinuclear/blood , Biomarkers/blood , Biopsy , Female , Hashimoto Disease/blood , Hashimoto Disease/diagnosis , Hashimoto Disease/drug therapy , Hashimoto Disease/immunology , Humans , Immunosuppressive Agents/therapeutic use , Liver Cirrhosis, Biliary/blood , Liver Cirrhosis, Biliary/diagnosis , Liver Cirrhosis, Biliary/drug therapy , Liver Cirrhosis, Biliary/immunology , Microscopic Polyangiitis/blood , Microscopic Polyangiitis/diagnosis , Microscopic Polyangiitis/drug therapy , Microscopic Polyangiitis/immunology , Middle Aged , Predictive Value of Tests , Renal Insufficiency/etiology , Sjogren's Syndrome/blood , Sjogren's Syndrome/diagnosis , Sjogren's Syndrome/drug therapy , Sjogren's Syndrome/immunology , Steroids/therapeutic use , Treatment OutcomeABSTRACT
Behcet's disease (BD) is a multisystem vasculitis with protean manifestations. It is characterized by a heightened state of inflammation, although the factors that initiate and sustain this inflammation are not clear. We report some cases of BD-associated amyloidosis and have similar features. The patients developed nephrotic syndrome due to secondary amyloidosis, which was refractory to the immunosuppressive agents. Two patients expired and the third was lost to follow-up during the course. The BD complicated with amyloidosis is associated with high mortality despite the current aggressive therapy.
