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Contents | Director | One | Two | Three | Four | Topic | EKG | Rad 1 | Rad 2 Albany Medical Review - January 2002 |
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Case Report-
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Kanwar Saini , MD
The patient is a 39-year-old Haitian man brought to Albany Medical Center from jail for progressive muscle weakness and recurrent facial edema, especially prominent in lips and eyelids. The edema started about 2-3 months ago and progressively worsened to the point that the patient was unable to open his mouth or his eyes. He experienced difficulty swallowing solids as well as liquids on and off for about the same time and had also developed a rash on his upper torso and back, and the extensor surfaces of all four extremities. The patient complained of generalized muscle weakness that had been present for a few years but had now progressed to the point that he was unable to rise from a chair, ascend the stairs or even brush his teeth. A month prior to admission, he was seen by a doctor at the correctional facility and was started on prednisone 50mg QD. The steroids were tapered, and his symptoms, which had improved, returned once he reached a dose of 5mg QD. Past medical history and surgical history are unremarkable, and the patient denied the use of any tobacco or recreational drugs, but prior to incarceration, drank "a few beers" on weekends. He had worked as a cab driver in NYC. Medications at the time of admission included only prednisone 5mg QD. In review of systems, the patient denied any nausea, vomiting, trauma, abdominal pain, fever or recent infections. He admitted to losing about 20 pounds in the 2 months prior to admission, with accompanying decrease in appetite and dysphagia. As stated above, he also had muscle weakness and a rash. He denied any previous episodes similar to this, and reported no family history of musculoskeletal disease. On physical examination, his temperature was 97.3 degrees, blood pressure 112/70, pulse 100, respirations 20, and oxygen saturation 100% in room air. In general, the patient was a thin Haitian male lying in bed in mild distress with marked facial edema. His head was normocepahalic atraumatic. His pupils were equal, round, reactive, and accommodating to light. His extra ocular muscles were intact. He had marked swelling of his periorbital region with dry oral mucosa. His neck was supple without jugular venous distention, bruits or lymphadenopathy. His heart had a regular rate and rhythm without murmur. Lungs were clear to ausculate without wheezing or rales. His abdomen was soft with positive bowel sounds. His skin had purple and pinkish confluent, flat rash around his eyes with areas of hyperpigmentation. The rash was also present on his upper torso, back, knees, and elbows. He was alert and oriented to person, place and time, but unable to speak articulately secondary to his facial edema and weakness. Cranial nerves were grossly intact. His strength was 3/5 in bilateral hip flexors and 3/5 in bilateral shoulder abductors, with normal reflexes. On admission, laboratory values included sodium 134, chloride 98, potassium 5.5, bicarbonate 27, BUN 26, creatinine 0.8, and glucose 113. His albumin 2.5, ALP 49, AST 308, ALT 76, INR 0.9, PTT 29.7, ESR 82, and ANA positive. His CPK 6670, MB fraction 27.3, (0.4%), LDH 363, and Calcium 8.8. He had a normal thyroid function test and was negative for serum Hepatitis A, B and C and HIV screens. His urine and blood cultures were negative. Chest X-ray was unremarkable and EKG had no ST changes. During the course of his hospital stay the rheumatology service was consulted. They recommended EMG, Nerve conduction studies, and muscle biopsy for further evaluation and definitive diagnosis. EMG and Nerve conduction studies were consistent with myositis. Muscle biopsy from the patient’s proximal quadriceps showed perivascular lymphohistiocytic inflammation, which was non-diagnostic, but raised the possibility of inflammatory myopathy. At admission, the patient was started on Solumedrol 40 mg IV BID and then switched to Prednisone 40 mg BID. He was also started on Methotrexate 20 mg SQ once a week with folic acid supplements. The patient’s symptoms improved remarkably. His muscle strength improved and he was able to participate in physical therapy. His periorbital swelling decreased, but he was still not tolerating oral intake, so a Video Esophagram was ordered and showed marked aspiration due to poor control of the upper airway as well pharyngeal muscle weakness. A Gastro-Jejunumostomy (G-J) tube was therefore recommended for long term feeding and to prevent aspiration. He received a G-J tube and tolerated tube feeds well. An EGD and colonoscopy were performed and were unremarkable. The patient was discharged back to his facility with prednisone, methotrexate, and a G-J tube with an outpatient follow up with Rheumatology.
DISCUSSION This patient was diagnosed with dermatomyositis. His symptoms and signs fit all the diagnostic criteria and demonstrated a classical presentation. Idiopathic inflammatory muscle disease is typically associated with proximal muscle weakness, muscle enzyme elevations (CPK, LDH), characteristic EMG, inflammatory infiltrations on muscle biopsy and skin rash. If no rash is present, diagnosis is consistent with polymyositis, and if rash is present, a diagnosis of dermatomyositis can be made. Our patient exhibited elevated muscle enzymes, characteristic EMG with spontaneous fibrillation, and muscle biopsy consistent with inflammatory myopathy. Dermatomyositis usually occurs in 1/200,000 people and peaks in childhood or late adulthood. It is twice as common in women. The etiology of the disease is unknown, but it is believed to be initiated by viral infection and altered immune response. Lymphocyte mediated muscle cell damage and small vessel damage are important central pathogenetic factors. Usually vascular deposits of immune complexes and complement are associated with endothelial cell injury and small vessel obstruction. Dermatomyositis is usually associated with CD4 T cells and B cells infiltrating the muscles whereas polymyositis is associated with CD8 cytotoxic T cells. Other features of dermatomyositis includes a heliotrope rash, which is a violet discoloration and swelling of the eyelids, erythematous papules over MCP and PIP joints, called Gottron’s sign, and erythematous skin and hypertrophic changes of palms and fingers, called Mechanic’s hands. This disease process has a gradual and steady progression of symmetrical skeletal muscle weakness of proximal, upper, and lower extremities. The patients are also prone to congestive heart failure, arrhythmias, chronic interstitial lung disease, aspiration pneumonitis and inflammatory arthritis. Autoantibodies are present in 80-90% of the patients, the most common being ANA, but RO Ab, LA Ab, Synthetase Ab, Jo-1 Ab, SRP Ab are also seen. Risk of malignancy is increased by 10-25 % with dermatomyositis as compared with polymyositis, especially cancers of the lung, gastrointestinal tract, breast, uterus, and ovaries. Treatment is usually with high dose corticosteroids and immunosuppressive agents such as methotrexate, azathioprine and cyclosporine. Physical therapy is essential to prevent contractures. For steroid resistant patients, IV immunoglobulin can be used. The differential diagnosis includes hyperthyroidism, which has normal CPK levels, hypothyroidism, which may demonstrate proximal muscle weakness, elevated CPK, but abnormal thyroid function tests, and Cushing syndrome, which can cause proximal muscle weakness but has normal enzymes. Drugs like cholesterol-lowering drugs, corticosteroids, AZT, and colchicine can also cause muscle weakness. Other muscle diseases like muscular dystrophies and metabolic muscle diseases usually have a family history associated with them. Some neurological diseases can also present with muscle weakness, for example: myasthenia gravis, amyotrophic lateral sclerosis, multiple sclerosis, but also have other neurological deficits associated with them. Other diseases that can present with muscle weakness include Lyme disease, HIV, toxplasmosis, influenza, and vasculities such as SLE, rheumatoid arthritis, progressive systemic sclerosis and Sjogren’s syndrome. Sarcoidosis can also have inflammatory muscle involvement, but biopsy usually shows non-caseating granuloma, increased ACE levels and chest x-ray showing bilateral hilar adenopathy.
References
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30.01.2002 |
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