Systemic and thyroid autoimmune diseases often overlap with each other. HT and GD may be most common among MCTD, SSc and SS patients. On the other hand, these systemic diseases are often present in HT subjects. Therefore it is clinically important to screen patients with systemic autoimmune diseases for the co-existence of thyroid disorders.
By using nailfold capillaroscopy, abnormalities in capillary morphology can be detected, and by using LDI, the reduced blood flow in the capillaries can be detected. These investigations can be useful in the detection of SRS, or in distinguishing whether the reduced blood flow is due to primary/systemic autoimmune diseases.
Primary Raynaud's disease is generally a disease of younger females; however, there are cases where symptoms present over the age of 40. These cases are described as late onset. In our current prospective study we compared the characteristics of early and late onset types of primary Raynaud's in 127 patients. In addition to the collection of medical records, we performed capillary-microscopy and hand perfusion scintigraphy using Tc-99 m DTPA to evaluate the microcirculation of each patient's fingers. Regarding the spectrum of the capillary-microscopic findings, we did not find any significant difference between the early and late onset forms. However, in hand perfusion examinations done using Tc-99 m DTPA, we measured a significantly lower finger/palm ratio (FPR) in the early onset group of patients. We also observed a correlation between the duration of the disease and the FPR, as well as between the age and FPR. Longer disease duration resulted in a significantly lower FPR. On the basis of our results, we believe that late onset Raynaud's should be treated as a separate entity. Due to its different characteristics found on examination and follow-up of our patients, functional hand perfusion examination should be recommended independently of the age-related characteristics of the disease.
We describe a rare case of concurrent polymyositis and Crohn's disease in a female patient. A 69-year-old female presented in December 2007 with a 5-month history of proximal muscle weakness, pain, fatigue and difficulty in walking and swallowing. Blood tests revealed elevated creatine kinase (3,429 U/l) and lactate dehydrogenase (2,013 U/l) levels. Magnetic resonance imaging found lumbar disc protrusion. Review by immunologists showed a diagnosis of idiopathic inflammatory myopathy. Though electromyography and muscle biopsy at this point were non-specific, corticosteroid treatment was commenced. Her condition worsened precipitously leading to hospitalisation under immunologists. As the provisional diagnosis was polymyositis, we commenced 1.5 mg/kg per day corticosteroid but her muscle power did not improve. Recurrent abdominal symptoms lead to ultrasonography showing intestinal inflammation. While tumour markers were elevated, thorough investigation failed to identify a tumour. Corticosteroid therapy was continued. Persistent abdominal symptoms lead to repeat colonoscopy and biopsy confirming Crohn's disease. Repeat electromyography and muscle biopsy confirmed the diagnosis of polymyositis. Her corticosteroids were tapered off and 5-aminosalicylic acid and azathioprine were started. Her myositic symptoms gradually abated with improvement in her Crohn's disease. She is now able to walk independently and takes 8 mg/day corticosteroids and her muscle enzyme levels are normal. Remember rare systemic associations when dealing with immune-mediated disease. Consider myositis in the differential diagnosis of Crohn's disease associated myopathy. Treating Crohn's disease may lead to improvement in steroid-resistant myositis where the two are associated.
Antisynthetase syndrome is a type of Idiopathic Inflammatory Myopathy (IIM) associated with anti-Jo1 antibody. Kennedy's disease or X-linked spinal and bulbar muscular atrophy (SBMA) is a rare neuromuscular disease. We describe the case report of a 53-year-old man who presented with proximal muscle weakness and a history of bilateral hand tremor. Initial physical examination demonstrated "mechanic's hands", Raynaud's phenomenon, having elevated creatine kinase and lactate dehydrogenase levels and anti-Jo1 antibody positivity. His muscle biopsy demonstrated inflammatory infiltrate characteristic of IIM. Considering these findings, we reached the diagnosis of antisynthetase syndrome and commenced immunosuppressive therapy. On follow-up examination, he had developed dysphagia, and his tremor had worsened. His electroneurogram result was characteristic of Kennedy's disease, and the genetic test result showed an allele with 44 CAG repeat expansion in the androgen receptor gene of the X chromosome. This confirmed that in addition to antisynthetase syndrome, he also had Kennedy's disease. This patient now receives immunology and neurology follow-up. His symptoms have improved with low dose corticosteroids, propranolol for tremor, vitamin B supplementation, and physiotherapy. This article presents a rare case report of a patient with concurrent antisynthetase syndrome and Kennedy's disease, both of which lead to elevated creatine kinase levels and muscle weakness, thus, underpinning the importance of careful follow-up of patients with IIM and maintaining an open mind to other diagnoses when faced with refractory and/or new symptoms.
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