Elderly Lupus Patient Presenting with Myositis � An Uncommon Presentation of a Common Disease

A 52-year-old married female presented with a history of generalized weakness for last 4 months aggravating over last two weeks. On clinical examination, we found Hyperpigmentation on her face and proximal muscle weakness in all four limbs, Alopecia; in routine investigations we found Anaemia, Thrombocytopenia, Raised Creatine Kinase. In imaging we found consolidation in left lower lobe, Nerve Conduction Velocity test showed brachial plexopathy and Sural Sensory Neuropathy, Electromyography showed fibrillation potentials and increased insertional activity in Gastrocnemius medial head, Tibialis anterior, Biceps brachii. We also performed muscle biopsy which showed findings suggestive of Dermatomyositis. Summarizing all the findings, we thought the cause to be an underlying Connective Tissue Disorder, hence we send samples for ANA, ENA profile which showed ANA 4+ homogenous, Ro52+++, RNP++, SS-A++; which led us to the final diagnosis of Systemic Lupus Erythematosus.

Manifestaciones sistémicas asociadas al consumo de cocaína: comunicación de un caso / Systemic manifestations associated with cocaine use: report of a case

Mujer de 38 años que ingresa a la emergencia por disnea, hemoptisis y pérdida hemática vaginal. Antecedente de hipertiroidismo, tabaquismo, etilismo y consumo de cocaína de 20 años de evolución. Se constató fiebre, petequias en paladar duro y miembros inferiores, úlcera en fosas nasales, equimosis y hepatoesplenomegalia. El laboratorio mostró anemia, plaquetopenia (5.0 K/uL), elevación de enzimas hepáticas, FAN (HeP-2), ANCA C y anticuerpo anti-Ro positivos. El lavado broncoalveolar confirmó la presencia de hemorragia alveolar. Se trató con gammaglobulina, tres pulsos de 1 g de metilprednisona y ciclofosfamida, con respuesta parcial. Desarrolla un accidente cerebrovascular hemorrágico en lóbulo temporal izquierdo, con hematoma subdural. Se agrega rituximab 375 mg/m² semanal durante 4 semanas, mejorando la hemorragia pulmonar, el recuento plaquetario y el cuadro neurológico.

A 38 year old woman came to the emergency room due to shortness of breath, hemoptysis and genital bleeding. Personal history: hyperthyroidism, alcoholism, tobacco and cocain use during the last 20 years. She presented with fever, petechiae on hard palate and legs, ulcers on nasal septum, liver and spleen enlargement, and ecchymosis. Lab exams showed anemia, thrombocytopenia (5.0 K/uL), elevated liver enzymes and positive AAN (HeP-2), C-ANCA and Ro antibodies. Bronchoalveolar lavage confirmed the presence of alveolar hemorrhage, so she was treated with intravenous gammaglobulin, methylprednisone and cyclophosphamide with partial response. The patient developed a cerebrovascular accident in left temporal lobe, with intracranial bleeding. Rituximab 375 mg/m² / week for four weeks was added, improving the alveolar hemorrhage, thrombocytopenia and neurological event.

Diagnóstico de granulomatosis de Wegener en pacientes con enfermedades oculares inflamatorias / Diagnosis of Wegener's granulomatosis in patients with ocular inflamatory disease

Objetivo. Informar las manifestaciones oftalmológicas en pacientes con granulomatosis de Wegener (GW). Método. Se revisó la base de datos del Instituto de Oftalmología Conde de Valenciana. Se recolectó la exploración oftalmológica completa, los exámenes de laboratorio y de gabinete, el tratamiento y la evolución. Resultados. Se incluyeron 11 pacientes con GW (18 ojos). Siete pacientes masculinos y 4 femeninos con edad promedio de 43.7 años (28-55). Tres habían tenido diagnóstico previo de GW y los 8 restantes se diagnosticaron en nuestro departamento. Siete presentaron cuadros bilaterales y 4 unilaterales. Las formas de presentación clínica fueron escleritis necrosante con queratitis ulcerativa periférica (QUP) (7/18), escleritis difusa (3/18), escleritis nodular (1/18), uveítis anterior no granulomatosa (1/18), neuropatía óptica isquémica (1/18), neuropatía óptica retrobulbar (1/18), desprendimiento de retina seroso (2/18) y dacriocistitis (2/18). De los 18 ojos, la capacidad visual final fue mejor o igual a 20/40 en 13, 20/400 en 3, cuenta dedos a 30cm o no-percepción de luz en 1. Actualmente 7 pacientes se encuentran en fase inactiva. Conclusiones. Las manifestaciones oftalmológicas más frecuentes en pacientes con GW fueron: escleritis necrosante y QUP. En la mayoría, la GW se diagnosticó después de las manifestaciones oftalmológicas, sin embargo, todos presentaron síntomas sistémicos u oftalmológicos previos.

OBJECTIVE: Report the ophthalmologic manifestations among patients with Wegener 's Granulomatosis (WG). METHOD: We reviewed the database of the Instituto de Oftalmologia Fundación Conde de Valenciana in order to collect information regarding complete ophthalmic examination, laboratory and cabinet tests, treatment, and disease progression. RESULTS: We included 11 patients with WG (18 eyes). Seven men and four women, mean age 43.7 years (range = 28-55). Three patients had a prior diagnosis of WG and the remaining eight patients were diagnosed by our study team. Seven subjects developed a bilateral affection and four had unilateral involvement. The clinical presentation was necrotizing scleritis with peripheral ulcerative keratitis (PUK) (7/18), diffuse scleritis (3/18), nodular scleritis (1/18), non-granulomatous uveitis (1/18), optic ischemic neuropathy (1/18), retrobulbar neuritis (1/18), serous retinal detachment (2/18), and dacryocystitis (2/18). Final visual acuity was better or equal to 20/40 (13/18), 20/400 (3/18), finger-counting or no-perception of light in 1/18. Currently, seven patients are symptom free. CONCLUSIONS: The most frequent ophthalmic manifestations among our patients with WG were: necrotizing scleritis and PUK. In most cases, WG was diagnosed after ophthalmic manifestations; however, all patients displayed prior systemic or ocular symptoms.