pituitrin and Granulomatosis-with-Polyangiitis

Topics: Churg-Strauss Syndrome; Cyclophosphamide; Granulomatosis with Polyangiitis; Humans; Immunoglobulins, Intravenous; Inappropriate ADH Syndrome; Male; Methylprednisolone; Middle Aged; Vasopressins

Topics: Addison Disease; Adrenal Cortex Hormones; Adrenal Glands; Adrenocorticotropic Hormone; Anti-Inflammatory Agents; Asthma; Corticotropin-Releasing Hormone; Facial Paralysis; Glucocorticoids; Granulomatosis with Polyangiitis; Hepatitis; Humans; Hypothalamus; Melanocyte-Stimulating Hormones; Myasthenia Gravis; Pituitary Gland; Pulmonary Edema; Retroperitoneal Fibrosis; Shock, Septic; Vasopressins

A 78-year-old woman with a history of bronchial asthma presented with distal dominant sensory disturbance and weakness in the upper and lower extremities. A biopsy of the left peroneus brevis muscle showed active vasculitis with inflammation extending into muscle fascicles and fibrinoid necrosis of the vessel wall, consistent with eosinophilic granulomatosis with polyangiitis (EGPA). Despite her decreased serum osmolarity, her serum antidiuretic hormone level was not reduced, consistent with the syndrome of inappropriate antidiuretic hormone (SIADH). Intravenous and oral steroid therapy improved her neurological symptoms. Clinicians should consider EGPA as a concurrent, and potentially causative, disorder in cases of SIADH.

Topics: Adrenal Cortex Hormones; Aged; Eosinophilia; Female; Granulomatosis with Polyangiitis; Humans; Inappropriate ADH Syndrome; Vasopressins

Involvement of brain parenchyma or meninges in ANCA-associated small-vessel vasculitis such as Wegener's granulomatosis (WG) is not uncommon. In contrast, involvement of the pituitary is exceedingly rare with only a few cases reported so far. The diagnosis is usually made on the basis of imaging techniques and abnormal pituitary function tests in the setting of active systemic vasculitis. However, histology-proven involvement of the pituitary by WG has not been reported so far. We report a case of WG with histology-proven granulomatous necrotizing inflammation of the pituitary and hypothalamo-pituitary stalk, disclosed at autopsy after the patient had died suddenly and unexpectedly in his sleep. In a setting of histology-proven WG, these findings were regarded as a pituitary manifestation of the disorder. A distinct cause of death could not be found, hence we speculate that hypothalamo-pituitary inflammation due to WG may have caused the sudden death in this patient.

Topics: Adult; Death, Sudden; Granulomatosis with Polyangiitis; Humans; Immunoglobulins, Intravenous; Magnetic Resonance Imaging; Male; Pituitary Diseases; Pituitary Gland, Anterior; Vasopressins