Hiperparatireoidismo primário em paciente com lúpus eritematoso sistêmico

Abstract

RESUMOO hiperparatireoidismo primário (HPP) é uma doença metabólica que resulta da secreção anormal do hormônio da paratireóide (PTH), sendo uma das causas mais comuns de hipercalcemia na população geral. Apresentamos o caso de uma paciente de 47 anos, com diagnóstico prévio de lúpus eritematoso sistêmico (LES) há dois anos, que apresentou manifestações clínicas (dor óssea difusa, labilidade emocional, tumoração em ramo mandibular esquerdo) e laboratoriais (cálcio séri-co= 13,5mg/dL, fosfato= 1,8mg/dL, fosfatase alcalina= 3.028U/L, PTH intacto= 1472pg/dL) que culminaram com o diagnóstico de HPP, complementado pelo estudo anátomo-patológico, que evidenciou adenoma de paratireóide. Após o tratamento com calcitonina nasal 400UI/dia, pamidronato 90mg IV/semana e, por fim, a paratireoidectomia subtotal, a paciente apresentou melhora do quadro clínico e normalização dos exames laboratoriais. A presente descrição se constitui no segundo caso relatado na literatura, com tal associação de doenças. Embora os mecanismos etiopatogênicos do HPP em LES não estejam ainda definidos, essa associação deve ser sempre lembrada, uma vez que a estratégia terapêutica tende a mudar completamente. Primary hyperparathyroidism (PHP) is a metabolic illness that results from autonomous secretion of parathyroid hormone and is one of the most common causes of hypercalcemia. We present the case of a 47-yearold female with a previous diagnosis of systemic lupus erythematosus (SLE) in whom clinical (diffuse bone pain, emotional lability, jaw tumor) and laboratory features (calcium= 13.5mg/dL, phosphate= 1.8mg/dL, alkaline phosphatase= 3028U/L, PTH intact= 1472pg/dL) prompted the diagnosis of PHP secondary to parathyroid adenoma as demonstrated by the anatomopathology. After treatment with calcitonin spray 400UI per day, IV pamidronate 90mg/week, and subtotal parathyroidectomy, the patient status improved with normal laboratory tests. This is the second report to describe the coexistence of these two disorders in a single patient. Although the pathophysiology of the association of PHP and SLE is not known, the recognition of this association has a practical implication since the therapeutical strategy is completely different.

Functional parathyroid cyst in a patient with systemic lupus erythematosus: a case report

“…Two cases have been previously reported of primary hyperparathyroidism and SLE in a single patient (13) (14). Together with this case, all three patients were women aged over 40 years with previously diagnosed SLE.…”

Concomitant primary hyperparathyroidism and systemic lupus erythematosus: coincidence or not? A new case report

“…It can occur at any age but the majority of cases occur around 40 to 50 years old. Women are affected two to three times more than men [ 2 ]. Approximately 80% to 85% of patients with PHP have parathyroid adenoma [ 3 ].…”

“…Mechanisms by which hypercalcemia may occur in patients with SLE have been only partially clarified. The possible pathogenesis involves elevated blood PTH level, excessive endogenous production of PTH related peptide (rP) or the presence of autoantibodies against PTH receptor antibodies [ 2 , 4 - 6 ]. PTHrP may be produced by non-malignant lymphoid tissue in SLE.…”

Lupus Nephritis With Mild Asymptomatic Hypercalcemia in Children: A Case Report and Literature Review

“…All cases had no history of taking excessive calcium and vitamin D preparations. Among 17 cases reported, there were 15 females and 2 males (7, 15); 1 mild hypercalcemia (7) (male), 5 moderate hypercalcemia (2, 8, 12, 15, 16) (1 male and 4 females), 11 severe hypercalcemia (1, 3–6, 9–11, 13, 14, 17) (all females); 2 children (all females) (6, 9), 15 adults; there were 14 cases of hypercalcemia in active stage of SLE, 3 cases of hypercalcemia in remission stage of SLE (3, 6, 12) (all female, 2 of them considered the main cause of non-hypercalcemia in SLE); 15 cases of SLE-related hypercalcemia (1, 2, 4–11, 13–17), and 1 case of hypercalcemia were caused by primary hyperthyroidism (3), The coexistence of SLE and hypercalcemia was considered in only 1 case (3); SLE with PTH elevation in 5 cases [including 3 cases of parathyroid adenoma (1, 6, 12), 1 case of parathyroid cyst (3), 1 case was caused by autoantibody of calcium sensitive receptor (9)], SLE with PTHrP elevation in 3 cases (2, 15, 17); 1 case may be caused by false negative PTHrP (11), 2 cases may be caused by anti-PTHrP (4, 8), 5 cases may be caused by PTH-related protein and autoantibodies (7, 10, 13, 14, 16), 1 case may be related to the decrease of fibroblast growth factor 23 (5); 10 cases had increased serum creatinine or decreased creatinine clearance (2, 4–7, 9–11, 13, 15), 5 cases had LN (7, 9, 10, 13, 15); 7 cases had SLE variants, which were described as hypercalcemia-lymphedema Syndrome, characterized by hypercalcemia and serositis (4, 8, 13–17). Among them, 1 case was positive for PTHrP by lymph node biopsy (15); 6 cases were complicated with ectopic calcification (2–4, 9,…”

“…Among them, 1 case was positive for PTHrP by lymph node biopsy (15); 6 cases were complicated with ectopic calcification (2–4, 9, 10, 13, 17). There were 1 case of SLE complicated with tumors (12); 2 cases died, one died of hypercalcemia crisis (1), the other died of refractory septic shock (9); 11 cases of hypercalcemia were effective by glucocorticoid therapy (2, 4, 5, 7, 8, 10, 11, 13–15, 17), 2 cases were ineffective by glucocorticoid therapy (1, 16); after surgical excision of parathyroid gland, blood calcium returned to normal in 3 cases (3, 6, 12) (Table 1).…”