Rheumatic Manifestations of Endocrine Diseases

Joseph A. Markenson

Disclosures

Curr Opin Rheumatol. 2010;22(1):64-71. 

In This Article

Hypoparathyroidism

Seen infrequently, the clinical manifestations are a result of hypocalcemia. Albright's osteodystrophy or pseudohypoparathryoidism (pseudo HoPT) results from end-organ resistance (bone and kidney) to PTH and presents with elevated levels of PTH, hypocalcemia and hyperphosphatemia. Type 1a HoPT (autosomal dominant) is inherited maternally and characterized by calcification of the perispinal ligaments, short stature, and mental retardation. Patients clinically have shortened metatarsal and metacarpral bones and a defect in the genes encoding the alpha subunit of the cell membrane-associated guanine nucleotide stimulating unit of adenyl cyclase[39] type 1b HoPT also has resistance to PTH, but has a normal phenotype and is inherited paternally.[40,41]

Soft tissue calcifications not clinically relevant (in basal ganglia, cataracts shoulder joints or subcutaneous tissues of the hands) have been reported in HoPT and infrequently in pseudo HoPT.[42]

Surgically induced HoPT may also be accompanied by muscle weakness usually related to the degree of hypocalcemia and responsive to treatment with vitamin D and calcium.

Renal disease can result in crystal deposition disease [sodium urate, calcium pyrophosphate and basic calcium phosphate (hydroxyapatite)] as a result of hyperphosphatemia from reduced glomerular filtration and secondary hyperparathyroidism.[43] Sodium urate deposition results in acute gout seen more commonly in renal insufficiency. Gout is rare in patients on dialysis but can be seen after renal transplantation with decreased creatinine clearance and use of cyclosporine. CPPD deposition is seen less in renal disease than gout or hydroxyapatite and is rare in dialysis. Hydroxyapatite deposition can cause acute synovitis and periarticuar inflammation. Painful subcutaneous nodules or chronic asymptomatic nodules (uremic tumoral calcinosis) can occur. Prevention is achieved through phosphate restriction, adequate dialysis, and oral phosphate binding agents.

Renal osteodystrophy[44] is a result of osteomalacia, osteitis fibrosa cystic, osteosclerosis, aluminum toxicity, osteoporosis and B-2 microglobulin amyloid deposition and presents with bone pain, muscle wasting and myalgias as well as bone fractures.

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