ABSTRACT
In this report, we present a rare case of a 52-year-old man with a unique form of hypertrophic pachymeningitis involving the anterior part of the falx and who was positive for rheumatoid factor. The clinical symptom was only headache, without any cranial nerve palsies or ataxia. Diagnosis was made by gallium scintigraphy and magnet resonance imaging but was not confirmed by dural biopsy. Treatment with corticosteroid alone was extremely effective for him, while in most cases hypertrophic pachymeningitis recurs or progresses despite the treatment.
Subject(s)
Arthritis, Rheumatoid/diagnosis , Cranial Sinuses/pathology , Dura Mater/pathology , Meningitis/diagnostic imaging , Rheumatoid Factor/blood , Arthritis, Rheumatoid/complications , Biomarkers/blood , C-Reactive Protein/analysis , Follow-Up Studies , Gallium , Humans , Hypertrophy/complications , Hypertrophy/diagnostic imaging , Hypertrophy/drug therapy , Magnetic Resonance Imaging/methods , Male , Meningitis/complications , Meningitis/drug therapy , Middle Aged , Prednisolone/therapeutic use , Radionuclide Imaging , Risk Assessment , Severity of Illness Index , Treatment OutcomeABSTRACT
Abstract We report a rare case of a 17-year-old female with overlap syndrome (systemic lupus erythematosus and systemic sclerosis) who developed severe abdominal pain and bloody diarrhea accompanied by central nervous system lupus. Colonoscopy revealed multiple irregular and linear ulcers throughout the colon, which were resistant to corticosteroid pulse therapy and plasma exchange. The patient finally recovered after treatment with a relatively low dose of monthly intravenous cyclophosphamide (250 mg/m(2)) pulse therapy.
