Enfermedad relacionada a IgG4. Serie clínica de pacientes chilenos / IGG4-related disease. Report of 52 patients

BACKGROUND: IgG4-related disease (IgG4 RD) is an immune-mediated fibro-inflammatory disorder, with tissue infiltration of IgG4+ plasma cells. It causes pseudotumors, tumors, and a wide spectrum of clinical manifestations. AIM: To report the clinical, laboratory, histopathological and treatment characteristics of a group of Chilean patients with IgG4 RD. MATERIAL AND METHODS: Review of medical records of 52 patients aged 18 to 76 years with IgG4 RD seen at six medical centers. RESULTS: Elevated IgG4 serum levels (> 135 mg/dl) were found in 18 of 44 (41%) patients. There was histological confirmation of the disease in 46 patients. The most common sites of involvement were lungs, eyes and kidneys. Eighteen (35%) patients had only one organ involved, 34 (65%) patients had two organs and 13 (25%) patients had three or more organs. The involvement of two organs was significantly more common in men (p < 0.05). In patients with only one organ involvement, the most frequent location was orbital and meningeal. All patients with kidney or lung disease had multiorgan involvement. All patients received corticosteroid therapy, 67% synthetic immunosuppressants, and 16% rituximab. CONCLUSIONS: ER-IgG4 can affect any tissue. Multiorgan involvement was more common in this series, with preference for lungs, eyes and kidneys. An excellent response to steroids is characteristic of the disease, but with a high relapse rate that requires additional immunosuppression.

Poliadenopatías de origen reumatológico y las claves del diagnóstico diferencial: Análisis de 19 casos / Lymphadenopathies in patients with rheumatic diseases. Review of 19 cases

Background: The presence of multiple lymphadenopathies can be a diagnostic challenge. Aim: To describe the clinical, laboratory and imaging characteristics of 19 patients with lymphadenopathies of rheumatologic origin. Material and Methods: Review of medical records of 19 patients aged 16 to 72 years (68%) with lymphadenopathies presumably secondary to a rheumatic disease. Results: Six patients had systemic lupus erythematosus, six had Sjogren's disease, three had sarcoidosis, two had rheumatoid arthritis, one had IgG4 related disease and one had mixed connective tissue disease. A lymph node biopsy was performed in 11 patients and in eight a lymphoid follicular hyperplasia was found. Systemic symptoms were reported by 68% of patients. Blood lactate dehydrogenase was elevated only in cases associated with hemolytic anemia. There was no specific or predictable localization of the lymphadenopathies in imaging studies, except in the cases of sarcoidosis. The average size of the lymphadenopathies was 13.5 mm in diameter in short axis and there was no presence of necrosis, calcification, or conglomerate formation. Only one case presented splenomegaly. All patients responded favorably to corticosteroids. Conclusions: Lymphadenopathies associated with rheumatologic diseases can occur in a wide variety of diseases, especially systemic lupus erythematosus and Sjögren's disease. The absence of LDH elevation and splenomegaly and the absence of imaging findings such as conglomerates can orient to a rheumatologic origin.

Infección por citomegalovirus en pacientes reumatológicos: análisis de un caso y revisión de la literatura / Cytomegalovirus infection in rheumatologic patients: a case report and literature review

El Citomegalovirus es un microorganismo capaz de generar infecciones severas en pacientes inmunosuprimidos. Existe abundante información respecto a la infección en pacientes inmunosuprimidos por VIH o en relación a trasplante de órganos sólidos o hematopoyéticos. No ocurre lo mismo con los pacientes portadores de enfermedades autoinmunes. Si bien la clínica puede ser inespecífica y dificultar la sospecha diagnóstica, la clave está en determinar al paciente de riesgo para la infección y así realizar un diagnóstico precoz. Se presenta el caso de una mujer de 56 años, portadora de una polimiositis de difícil tratamiento, que en un contexto de terapia en base a corticoides e inmunosupresores (azatioprina y metotrexato), desarrolla cuadro febril asociado a fatiga, cuyo estudio concluyó una infección por Citomegalovirus, tratado exitosamente con Valganciclovir.

Cytomegalovirus is a microorganism associated with severe infections in immunosuppressed patients. There is abundant information regarding infection in HIV immunosuppressed patients or in relation to solid or hematopoietic organ transplantation. The same does not happen with patients with rheumatic diseases. Although the clinic can be nonspecific and hinder diagnostic suspicion, the key is to determine the patient at risk for the infection and thus make an early diagnosis. We present a case of a 56-year-old woman with a difficult-to-treat polymyositis, who, in a context of corticosteroid and immunosuppressive agents (azathioprine and methotrexate), develops a fever associated with fatigue, whose study con-cluded an infection due to Cytomegalovirus, successfully treated with Valganci-clovir.

Oligoartritis, paniculitis y pioderma gangrenoso como manifestaciones de un Linfoma no Hodgkin / Oligoarthritis, panniculitis and pyoderma gangrenosum associated with non-Hodgkin lymphoma: report of one case

An assortment of clinical and laboratory abnormalities may occur as paraneoplastic syndromes in lymphomas. Rheumatological and dermatological manifestations such as paraneoplastic arthritis and pyoderma gangrenosum must be underscored. We report a 28 years old woman who developed pyoderma gangrenosum and two years later presented with arthritis of knees and ankles associated with panniculitis interpreted as erythema induratum that was pathologically confirmed. She developed a reactivation of pyoderma gangrenosum, that was refractory to treatment. Complementary studies showed a pulmonary nodule and a right paravertebral mass with involvement of the psoas muscle. Biopsies of both masses and a new pathological skin study demonstrated a large B-cell non-Hodgkin's lymphoma.

Hemoptisis recurrente asociada a falla renal y hematuria: otra de las mil caras de la enfermedad relacionada a IgG4 / IgG4 associated nephritis and recurrent hemoptysis: case report

IgG4 disease is a multi-systemic condition involving pancreas, salivary glands and lymph nodes. Less frequently, it causes interstitial nephritis and involves the lungs. We report a 58 years old male with a four years history of hemoptysis and renal dysfunction characterized by hematuria and proteinuria, responsive to steroidal therapy. The renal biopsy established the diagnosis of IgG4 associated interstitial nephritis. Lung involvement was considered secondary to the same systemic disease.

Fibrosarcoma de arteria pulmonar simulando un síndrome de Hughes Stovin / Pulmonary artery sarcoma mimicking a Hughes Stovin syndrome. Report of one case

Pulmonary artery sarcoma is an uncommon entity with high mortality. Its clinical presentation is usually indistinguishable from pulmonary embolism, which leads to a significant delay in diagnosis. Hughes-Stovin syndrome is characterized by venous thrombosis and aneurysms of the pulmonary or bronchial artery. We report a 59 year-old female with a history of recurrent pulmonary embolism. In the last thromboembolic episode a pulmonary artery aneurysm was found on a CT scan. The patient was operated performing a left inferior lobectomy. The patient died five days after surgery and the pathological examination of the surgical piece revealed a pulmonary artery sarcoma.

Pneumorraquis, espondilitis y meningitis secundarios a cistitis enfisematosa / Pneumorrhachis, spondylitis and meningitis secondary to emphysematous cystitis. Report of one case

We report a 57-year-old woman who presented with low back pain, fever and impairment of consciousness. The patient was admitted to the intensive care unit in Glasgow 8, with neck stiffness, peritoneal irritation, leukocytosis, hyperglycemia requiring insulin and a urine test suspecting an infection. Brain CT was unremarkable, while CT of the abdomen and pelvis evidenced emphysematous cystitis, retropneumoperitoneum and pneumorrhachis. Blood, urine and cerebrospinal fluid cultures were positive to Escherichia coli. She was treated with ceftriaxone, ciprofloxacin and amikacin during one month followed by ciprofloxacin until completing 100 days. The air in the spinal canal and bladder decreased. However she suffered several infectious complications such as multiple paravertebral, epidural and psoas abscesses, L5-S1 spondylitis and a L3 fracture. As an inflammatory complication she developed a bulbar infarction and tetraparesis. She had a good clinical response with medical treatment, partial improvement of the paresis and reduction of epidural abscesses.

Enfermedad relacionada a IgG4, el nuevo "gran simulador": caso clínico / IgG4-related disease, the new "great mimicker": Report of one case

Due to its multisystem involvement, IgG4 -related disease should be considered in the differential diagnosis of medical conditions such as lymphadenopathies, aortitis, serositis and retroperitoneal fibrosis. It shares features with other entities historically described as "great mimickers" such as syphilis, tuberculosis, sarcoidosis, and systemic lupus erythematosus. We report a 40 year-old male with recurrent effusive - constrictive pericarditis, lymphadenopathy and aortitis. The study revealed an inactive tuberculosis with negative cultures for acid fast bacilli. The patient had high serum levels of IgG4 and a mediastinal lymph node biopsy was consistent with IgG4 -related disease. The patient was treated with prednisone 40 mg/day with an excellent response.