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Myasthenia gravis — Not only a neuromuscular disease
Abstracts
a b
Department of Nephrology, Charles Nicolle Hospital, Tunis, Tunisia Department of Nephrology, Mongi Slim Hospital, La Marsa, Tunisia
doi:10.1016/j.ejim.2013.08.654
DP
Introduction: Microscopic polyangiitis (MPA) is a systemic small vessel vasculitis that is included in the pulmonary renal symptoms and is associated with ANCA directed against myeloperoxidase (MPO). Although glomerulonephritis represents the major clinical feature of MPA indicative of renal involvement, diffuse alveolar hemorrhage is the classical manifestation of pulmonary involvement. We describe the clinical, biological and therapeutic characteristics of 22 patients in our unit. Patient and method: A retrospectively study was performed for 22 patients from 1976 to 2012. Results: The mean age of MPA patient diagnosis was 49.68 years (18–70 years). The male to female ratio was 1. All our patients presented renal insufficiency with mean serum creatinine: 670 μmol/l. Five patients presented a rapidly progressive glomerulonephritis. The most clinical manifestations of renal involvement were proteinuria and microscopic hematuria in 20 patients. Two patients presented anuria. Renal biopsy showed in all cases glomerular crescents with minimal deposition of immunoglobulins or complement in the glomeruli and vessels. Twelve patients had pulmonary manifestation wich include hemoptysis, dyspnea and alveolar hemorrhage. For the other clinical manifestations: 2 patients presented purpura, 4 patients neurological manifestation and one patient a cardiac manifestation. Inflammatory syndrome was present in 20 patients. The presence of circulating antineutrophil cytoplasmic antibodies (ANCAs) was attested in 15 cases in which 5 of them were anti myeloperoxidase (MPO). Twenty one of patients were treated with corticosteroid and cyclophosphamid. Seventeen patients improved their renal function and hadn't presented pulmonary symptoms. Conclusion: The diagnosis of MPO relies on the physician drawing together elements of the patients' clinical history and symptoms with diagnostic tests such as renal biopsy and autoantibody testing. Prognosis of MPO has greatly improved with the use of cyclophosphamid and glucocorticoids. The future of treatment appears bright as newer medication like rituximab shows great promise as effective alternative therapeutic agents with potentially less toxicity.
were diagnosed with Behçet's disease during this period, and 5 (17.2%) of them had demyelinating lesions in MRI. Mean age at diagnosis was 31.6 (15–62) years and 4 (80%) were women. Clinical manifestations: headache in all, alteration of cranial nerves in 3 (60%) — two with optic neuritis and one with peripheral facial paralysis, seizures in 2 (40%), sensory deficits in 3 (60%), transient motor deficits in 1. Three patients had low titer of positive ANA. The cerebrospinal fluid analysis showed no pleocytosis or elevated protein in any of them and three showed oligoclonal bands. The MRI with gadolinium showed T2-weighted signal abnormality in the white matter in all of them (supratentorial and infratentorial). One patient had progression of lesions with involvement of the spinal cord and brainstem. Treatment: Colchicine in 3, azathioprine in 3, oral corticosteroids in 3 and pulses of methylprednisolone in 4. In evolution, two patients are stable with low doses of corticosteroids; one remains stable without any treatment; one presents relapse despite infliximab and another progresses clinically and radiologically associated with poor adherence to treatment. Conclusions: Demyelinating lesions in Behçet's disease are frequent and the clinical course may clinically and radiologically mimic MS. All patients with demyelinating lesions should be required about symptoms of systemic disease and a full autoantibody investigation should be carried out. Patients should be followed for years, since the possibility of presenting a systemic disease is high (even if the autoantibodies are negative in the first investigation).
OF
ID: 617 Microscopic polyangiitis: Experience of our unit S. Barboucha, H. Gaiedb, R. Aoudiaa, M. Jerbia, K. Ben Abdelghnia, R. Gouchab, A. Khedera
RO
e254
ID: 624 Myasthenia gravis — Not only a neuromuscular disease P. Cunha, F. Coutinho, C. Gonçalves
TE
Internal Medicine Department, Centro Hospitalar do Médio Tejo, Unidade de Abrantes, Abrantes, Portugal
doi:10.1016/j.ejim.2013.08.653
RR
EC
Introduction: Myasthenia gravis is an autoimmune disease that affects the neuromuscular junction wherein antibodies to nicotinic acetylcholine receptors located in postsynaptic membrane are identified. It presents a picture of fatigue and muscle weakness exacerbated by exercise and relieved by rest, with a predominance of facial and bulbar muscles in the early phase of the disease, which can be generalized to other muscle groups at a later stage. Case description: The authors present the case of a male patient, 79 years old, with personal history of Myasthenia Gravis, Alopecia Universalis, Diabetes Mellitus Type 2, Obstructive Sleep Apnea Syndrome, Cataract and Glaucoma, admitted to definitive pacemaker implantation after identification of a trifascicular block in Holter electrocardiogram in the context of syncope etiological study. Discussion: The diagnosis of an autoimmune disease is, by itself, a risk factor for other similar illnesses. The presented case shows an association between Myasthenia Gravis and Alopecia Universalis (a more extensive and rare form of Alopecia Areata). Although Myasthenia Gravis association with cardiac disease has now been demonstrated in several studies, it is often overlooked because of the overlapping clinical features (for example, easy fatigability in the context of heart failure). Some studies have reported the association of Myasthenia Gravis with intracardiac conduction disturbances. The primary relationship and causal disease or a consequence of the use of parasympathomimetic drugs in the treatment of Myasthenia Gravis is still a controversial subject with no definitive answer. It is presented as a typical clinical case of Autoimmunity, with a multisystem pathology basis and several multiorgan complications which demands, more than others, a holistic and comprehensive look in the process of diagnosis and treatment. In addition, a theoretical review on Myasthenia Gravis, complications, treatment and comorbidities is performed.
a
CO
ID: 618 Demyelinating lesions in Behçet's disease L. González-Vázqueza, J.R. González-Lorenzob, M. González-Pérezc, E. Santos-Armentíad, J. De la Fuentea
UN
Department of Internal Medicine, Povisa Hospital, Vigo, Spain Department of Neurology, Povisa Hospital, Vigo, Spain c Department of Rheumatology, Povisa Hospital, Vigo, Spain d Department of Radiology, Povisa Hospital, Vigo, Spain b
Objective: Neurologic involvement in Behçet's disease occurs in 5–10% of cases. There are two patterns: parenchymal and non parenchymal involvement. Demyelinating lesion is a rare form of parenchymal afectation. Our objective is to describe the clinical manifestations, laboratory test, location of demyelinating lesions, treatment and evolution of these patients. Methods: A retrospective study was carried out in patients diagnosed with Behçet's disease between January 1990 and December 2010. The diagnosis was based on international criteria for diagnosis of Behçet's disease, magnetic resonance imaging (MRI) of the brain and results of cerebrospinal fluid. Results: 29 patients
doi:10.1016/j.ejim.2013.08.655
a b
Department of Nephrology, Charles Nicolle Hospital, Tunis, Tunisia Department of Nephrology, Mongi Slim Hospital, La Marsa, Tunisia
doi:10.1016/j.ejim.2013.08.654
DP
Introduction: Microscopic polyangiitis (MPA) is a systemic small vessel vasculitis that is included in the pulmonary renal symptoms and is associated with ANCA directed against myeloperoxidase (MPO). Although glomerulonephritis represents the major clinical feature of MPA indicative of renal involvement, diffuse alveolar hemorrhage is the classical manifestation of pulmonary involvement. We describe the clinical, biological and therapeutic characteristics of 22 patients in our unit. Patient and method: A retrospectively study was performed for 22 patients from 1976 to 2012. Results: The mean age of MPA patient diagnosis was 49.68 years (18–70 years). The male to female ratio was 1. All our patients presented renal insufficiency with mean serum creatinine: 670 μmol/l. Five patients presented a rapidly progressive glomerulonephritis. The most clinical manifestations of renal involvement were proteinuria and microscopic hematuria in 20 patients. Two patients presented anuria. Renal biopsy showed in all cases glomerular crescents with minimal deposition of immunoglobulins or complement in the glomeruli and vessels. Twelve patients had pulmonary manifestation wich include hemoptysis, dyspnea and alveolar hemorrhage. For the other clinical manifestations: 2 patients presented purpura, 4 patients neurological manifestation and one patient a cardiac manifestation. Inflammatory syndrome was present in 20 patients. The presence of circulating antineutrophil cytoplasmic antibodies (ANCAs) was attested in 15 cases in which 5 of them were anti myeloperoxidase (MPO). Twenty one of patients were treated with corticosteroid and cyclophosphamid. Seventeen patients improved their renal function and hadn't presented pulmonary symptoms. Conclusion: The diagnosis of MPO relies on the physician drawing together elements of the patients' clinical history and symptoms with diagnostic tests such as renal biopsy and autoantibody testing. Prognosis of MPO has greatly improved with the use of cyclophosphamid and glucocorticoids. The future of treatment appears bright as newer medication like rituximab shows great promise as effective alternative therapeutic agents with potentially less toxicity.
were diagnosed with Behçet's disease during this period, and 5 (17.2%) of them had demyelinating lesions in MRI. Mean age at diagnosis was 31.6 (15–62) years and 4 (80%) were women. Clinical manifestations: headache in all, alteration of cranial nerves in 3 (60%) — two with optic neuritis and one with peripheral facial paralysis, seizures in 2 (40%), sensory deficits in 3 (60%), transient motor deficits in 1. Three patients had low titer of positive ANA. The cerebrospinal fluid analysis showed no pleocytosis or elevated protein in any of them and three showed oligoclonal bands. The MRI with gadolinium showed T2-weighted signal abnormality in the white matter in all of them (supratentorial and infratentorial). One patient had progression of lesions with involvement of the spinal cord and brainstem. Treatment: Colchicine in 3, azathioprine in 3, oral corticosteroids in 3 and pulses of methylprednisolone in 4. In evolution, two patients are stable with low doses of corticosteroids; one remains stable without any treatment; one presents relapse despite infliximab and another progresses clinically and radiologically associated with poor adherence to treatment. Conclusions: Demyelinating lesions in Behçet's disease are frequent and the clinical course may clinically and radiologically mimic MS. All patients with demyelinating lesions should be required about symptoms of systemic disease and a full autoantibody investigation should be carried out. Patients should be followed for years, since the possibility of presenting a systemic disease is high (even if the autoantibodies are negative in the first investigation).
OF
ID: 617 Microscopic polyangiitis: Experience of our unit S. Barboucha, H. Gaiedb, R. Aoudiaa, M. Jerbia, K. Ben Abdelghnia, R. Gouchab, A. Khedera
RO
e254
ID: 624 Myasthenia gravis — Not only a neuromuscular disease P. Cunha, F. Coutinho, C. Gonçalves
TE
Internal Medicine Department, Centro Hospitalar do Médio Tejo, Unidade de Abrantes, Abrantes, Portugal
doi:10.1016/j.ejim.2013.08.653
RR
EC
Introduction: Myasthenia gravis is an autoimmune disease that affects the neuromuscular junction wherein antibodies to nicotinic acetylcholine receptors located in postsynaptic membrane are identified. It presents a picture of fatigue and muscle weakness exacerbated by exercise and relieved by rest, with a predominance of facial and bulbar muscles in the early phase of the disease, which can be generalized to other muscle groups at a later stage. Case description: The authors present the case of a male patient, 79 years old, with personal history of Myasthenia Gravis, Alopecia Universalis, Diabetes Mellitus Type 2, Obstructive Sleep Apnea Syndrome, Cataract and Glaucoma, admitted to definitive pacemaker implantation after identification of a trifascicular block in Holter electrocardiogram in the context of syncope etiological study. Discussion: The diagnosis of an autoimmune disease is, by itself, a risk factor for other similar illnesses. The presented case shows an association between Myasthenia Gravis and Alopecia Universalis (a more extensive and rare form of Alopecia Areata). Although Myasthenia Gravis association with cardiac disease has now been demonstrated in several studies, it is often overlooked because of the overlapping clinical features (for example, easy fatigability in the context of heart failure). Some studies have reported the association of Myasthenia Gravis with intracardiac conduction disturbances. The primary relationship and causal disease or a consequence of the use of parasympathomimetic drugs in the treatment of Myasthenia Gravis is still a controversial subject with no definitive answer. It is presented as a typical clinical case of Autoimmunity, with a multisystem pathology basis and several multiorgan complications which demands, more than others, a holistic and comprehensive look in the process of diagnosis and treatment. In addition, a theoretical review on Myasthenia Gravis, complications, treatment and comorbidities is performed.
a
CO
ID: 618 Demyelinating lesions in Behçet's disease L. González-Vázqueza, J.R. González-Lorenzob, M. González-Pérezc, E. Santos-Armentíad, J. De la Fuentea
UN
Department of Internal Medicine, Povisa Hospital, Vigo, Spain Department of Neurology, Povisa Hospital, Vigo, Spain c Department of Rheumatology, Povisa Hospital, Vigo, Spain d Department of Radiology, Povisa Hospital, Vigo, Spain b
Objective: Neurologic involvement in Behçet's disease occurs in 5–10% of cases. There are two patterns: parenchymal and non parenchymal involvement. Demyelinating lesion is a rare form of parenchymal afectation. Our objective is to describe the clinical manifestations, laboratory test, location of demyelinating lesions, treatment and evolution of these patients. Methods: A retrospective study was carried out in patients diagnosed with Behçet's disease between January 1990 and December 2010. The diagnosis was based on international criteria for diagnosis of Behçet's disease, magnetic resonance imaging (MRI) of the brain and results of cerebrospinal fluid. Results: 29 patients
doi:10.1016/j.ejim.2013.08.655