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.
Subject(s)
Amyloidosis/complications , Behcet Syndrome/complications , Nephrotic Syndrome/etiology , Adult , Amyloidosis/drug therapy , Behcet Syndrome/drug therapy , Fatal Outcome , Humans , Male , Middle AgedABSTRACT
Although peripheral neuropathy is a common complication of microscopic angiitis, manifestations involving the muscle and the central nervous system have been rarely reported. We describe a 48-year-old man who rapidly developed a clinical picture of mononeuritis multiplex. A month after the appearance of the primary symptoms, he became comatose and had left hemiplegia in relation with a massive cerebral haematoma. Laboratory data revealed signs of inflammation, glomerular dysfunction with microhaematuria, and positive myeloperoxidase-antineutrophil cytoplasmic antibodies. The neuromuscular biopsy disclosed a small-vessel vasculitis, consisting with microscopic angiitis, associated with myositis and extensive axonal loss. The patient had surgical evacuation of the haematoma and received immunosuppressive therapy with good outcome. Thus, microscopic angiitis should be considered as a differential diagnosis in cases of myositis and intracerebral haemorrhage.
Subject(s)
Central Nervous System/pathology , Microscopic Polyangiitis/pathology , Peripheral Nervous System/pathology , Action Potentials/physiology , Anti-Inflammatory Agents/therapeutic use , Biopsy , Cyclophosphamide/therapeutic use , Electromyography , Humans , Immunosuppressive Agents/therapeutic use , Intracranial Hemorrhages/complications , Intracranial Hemorrhages/pathology , Male , Microscopic Polyangiitis/complications , Middle Aged , Mononeuropathies/complications , Mononeuropathies/pathology , Muscle, Skeletal/innervation , Muscle, Skeletal/pathology , Myositis/complications , Myositis/pathology , Peroneal Nerve/pathology , Prednisone/therapeutic use , Recovery of Function , Tomography, X-Ray Computed , Vasculitis, Central Nervous System/complications , Vasculitis, Central Nervous System/pathologyABSTRACT
INTRODUCTION: Langerhans cell histiocytosis of the thoracic spine is a rare condition in adults. It is a serious condition with an increased risk of spinal cord compression. EXEGESIS: A 55-year-old man complained of lumbar and back pain. He developed a spastic paraparesis. Magnetic resonance imaging of the spine showed a spinal cord compression due to bone and epidural tumoral lesions of the eleventh and twelfth thoracic vertebrae, located in the posterior elements of the spine. A surgical biopsy showed an infiltrate of eosinophilic cells, positive for CD1a in immunohistochemistry studies. Final diagnosis was therefore langerhans cell histiocytosis of the thoracic spine complicated by spinal cord compression. The patient received glucocorticoid therapy combined with vinblastine followed by local radiotherapy and etoposide. However, the neurological deficit persisted and the patient finally died. CONCLUSION: Langerhans cell histiocytosis is a rare cause of spinal cord compression.