Increased clinical symptoms of acromegalic arthropathy in patients with long-term disease control: a prospective follow-up study

Arthropathy is an invalidating complication of acromegaly. This arthropathy deteriorates radiographically despite long-term disease control. However, the clinical course and its relationship to the radiographic course are currently unknown. We aimed to investigate the clinical course of arthropathy during follow-up and its relationship to radiographic progression in long-term controlled acromegaly patients. Prospective follow-up study. We studied 58 patients (mean age 62 years, women 41 %) with controlled acromegaly for a mean of 17.6 years. Clinical progression of joint disease was defined at baseline and after 2.6 years, by the Western Ontario McMaster Universities Osteoarthritis Index (WOMAC) and Australian/Canadian Osteoarthritis Index (AUSCAN) questionnaires for lower limb and hand OA, respectively, and performance tests. Potential risk factors for progression were assessed. The clinical course of arthropathy was related to the radiographic course. On average, hand and lower limb function deteriorated during follow-up, despite large interindividual variations. Joint pain was stable over time. High levels of pain and functional impairment at baseline were related to clinical progression of hand pain and functional limitations. High baseline BMI was a risk factor for functional deterioration in the lower limb. The changes in symptoms and radiographic progression during follow-up were not related. In treated acromegaly patients, joint function deteriorates during prolonged follow-up, despite biochemical disease control, although there was interindividual variation. Clinical and radiographic course of arthropathy were not related. Therefore, in clinical practice, a combination of clinical and radiographic assessment is necessary to evaluate the course of acromegalic arthropathy.     

Two phenotypes of arthropathy in long-term controlled acromegaly? A comparison between patients with and without joint space narrowing (JSN)

BackgroundArthropathy is an invalidating complication of acromegaly, also in long-term controlled patients, and is radiographically characterized by osteophytes and preserved joint spaces. However, joint space narrowing (JSN) is observed in the minority of patients. It is unknown whether JSN is the end-stage of acromegalic arthropathy or whether this feature develops independently of acromegaly.ObjectiveTo gain insight into the pathophysiology of acromegalic arthropathy, and, more specifically, in the process of JSN, risk factors for radiographic JSN were studied in a cross-sectional study.MethodsWe studied hips and knees of 89 well-controlled acromegaly patients (mean age 58.3 yr, 51% female). Joints were divided into two groups based on the presence of JSN, defined as an Osteoarthritis Research Society (OARSI) score ≥ 1. Potential risk factors for JSN were assessed, and its relationship to joint complaints. Individual knees and hips were analyzed in a Generalized Estimating Equations model, adjusted for age, sex, BMI and intra-patient effect.ResultsIn controlled acromegaly, JSN was found in, respectively, 10.3% and 15.4% of the hips and knees. Increasing age and female sex were associated with more JSN; acromegaly-specific risk factors for JSN were joint-site specific. In the hip, JSN was related to more active disease: higher pre-treatment GH/IGF-1, longer and more severe GH exposure and immediate postoperative cure was less frequently achieved. In the knee, especially previous knee surgery, not acromegaly-specific characteristics, was associated with JSN. The presence of JSN was associated with more joint complaints.ConclusionsJSN is an infrequent finding in patients with acromegalic arthropathy, but it is associated with more symptoms. This study indicates that, at least in the hip, early and ongoing GH/IGF-1 activity play a role in JSN development.     

ARTICULAR MANIFESTATIONS OF ACROMEGALY

Forty-five patients with acromegaly or gigantism were reviewed for musculoskeletal abnormalities. Abnormalities of peripheral joints occurred in 74% of the patients and spinal involvement in 47%, leading to significant morbidity. Joint abnormalities most frequently affected the large joints (hips, knees and shoulders) but the wrist and hand were also involved. The radiological features of acromegalic arthropathy are described, including vertical widening of the hip joint, enthesopathy and osteophytosis. A favourable response to treatment is associated with a less severe arthropathy and a good functional outcome.     

Rheumatoid arthritis masquerading as acromegaly recurrence: report of two cases

Acromegaly is a chronic endocrinopathy characterized by hypersecretion of growth hormone (GH) and consequently of insulin-like growth factor-1 (IGF-1). The arthropathy in acromegaly is the most frequent and important cause of morbidity and functional disability in acromegaly. Rheumatoid arthritis (RA) is a rarely reported clinical situation in patients with acromegalic. We herein report 57- and 45-year-old two women, who complained bilateral, symmetric pain, swelling and morning stiffness in the joints of hands after optimal acromegaly treatment resembling acromegaly arthropathy. There was not arthralgia in other joints of the patients. Laboratory and radiological evaluations were carried out. After excluding the acromegaly activation and arthropathy by GH and IGF-1 measurement, according to clinical presentation, laboratory and radiological assessments, patients were diagnosed as RA.     

Acromegalic arthropathy

Arthropathy was assessed in 19 patients with active acromegaly. Axial or peripheral arthropathy was present in 10 patients, was located most often in large joints or the lumbosacral spine, and was osteoarthritic in nature. The mean duration of acromegaly in patients with arthropathy was 21.6 years, while the mean duration in patients without arthropathy was 7.9 years. A mild-to-moderate improvement in symptoms, estimated functional ability, and crepitus occurred in 8 of 9 patients who were prospectively examined during therapy to lower production of growth hormone. We conclude that this therapy did improve the symptoms of acromegalic arthropathy. Whether objective structural improvements occur remains unclear.     

Acromegalic arthropathy. Characteristics and response to therapy

Arthropathy was assessed in 19 patients with active acromegaly. Axial or peripheral arthropathy was present in 10 patients, was located most often in large joints or the lumbosacral spine, and was osteoarthritic in nature. The mean duration of acromegaly in patients with arthropathy was 21.6 years, while the mean duration in patients without arthropathy was 7.9 years. A mild-to-moderate improvement in symptoms, estimated functional ability, and crepitus occurred in 8 of 9 patients who were prospectively examined during therapy to lower production of growth hormone. We conclude that this therapy did improve the symptoms of acromegalic arthropathy. Whether objective structural improvements occur remains unclear.     

ARTHROPATHY IN ACROMEGALIC PATIENTS BEFORE AND AFTER TREATMENT: A LONG-TERM FOLLOW-UP STUDY

The medical records of 90 patients with acromegaly were reviewed. Arthralgias were noted in 76% of the patients with 17% having the onset of joint pain concomitant with the clinical onset of acromegaly. Of 47 patients followed prospectively for 5 or more years after pituitary irradiation, six (12.8%) were unaffected by arthralgias. A statistically higher mean baseline growth hormone level was found for the 19 (40.4%) radiotherapy patients who had severe and disabling arthropathy. Mean intervals between clinical onset of acromegaly and the development of arthropathic symptoms were shorter (4.1 years) for patients over 40 years of age and longer (9.7 years) for those under 31 years of age. Severely affected patients tended to have increased joint spaces in both weight-bearing and non-weight-bearing joints followed by a progressive decrease in joint spaces. Arthropathy is a common complication of acromegaly and may progress independently of a fall in growth hormone, induced by any form of treatment, once significant cartilage overgrowth develops. Cartilage overgrowth is a predisposing factor in the development of an arthropathy associated with the wide range of growth hormone levels characteristic of acromegaly.     

A challenging case of rheumatoid arthritis in an acromegalic patient

A 63-year-old man with complaints of joint pain and ankle swelling was evaluated. The arthralgias he described were mainly in the knees, elbows, and shoulders. Accompanying swelling and erythema in his left ankle and left second metacarpophalangeal (MCP) joint had recently ensued. His past history revealed acromegaly, somatotropinectomy, and radiotherapy. His neck, bilateral wrist, elbow, and shoulder joints were involved; there was pain and limited range of motion. The MCP joints, being worse than the interphalangeal joints, were likewise involved. His left ankle and MCP joints additionally were swollen and erythematous. Laboratory and radiological evaluations were carried out. Radiological and clinical findings confirmed a diagnosis of rheumatoid arthritis and concurrent acromegalic arthropathy. The patient was treated accordingly. Interestingly, he later developed colon cancer.     

Osteoporosis in haemophilia – an underestimated comorbidity?

A relationship between haemophilia and osteoporosis has been suggested, leading to the initiative for a larger study assessing this issue. Bone mineral density (BMD) was measured by osteodensitometry using dual energy X-ray absorptiometry (DEXA) in 62 male patients with severe haemophilia A; mean age 41 +/- 13.1 years, mean body mass index (BMI) 23.5 +/- 3.6 kg m(-2). Using the clinical score suggested by the World Federation of Hemophilia, all patients were assessed to determine the severity of their arthropathy. A reduced BMD defined as osteopenia and osteoporosis by World Health Organization criteria was detected in 27/62 (43.5%) and 16/62 (25.8%) patients, respectively. Fifty-five of sixty-two (88.7%) patients suffered from haemophilic arthropathy. An increased number of affected joints and/or an increased severity were associated with lower BMD in the neck of femur. Pronounced muscle atrophy and loss of joint movement were also associated with low BMD. Furthermore, hepatitis C, low BMI and age were found to be additional risk factors for reduced BMD in the haemophiliac. Our data shows that in haemophilic patients osteoporosis represents a frequent concomitant observation. The main cause for reduced bone mass in the haemophiliac is most probably the haemophilic arthropathy being typically associated with chronic pain and loss of joint function subsequently leading to inactivity. Further studies including control groups are necessary to elucidate the impact of comorbidities such as hepatitis C or HIV on the development of osteoporosis in the haemophiliac.     

Prevalence of vertebral fractures in men with acromegaly

CONTEXT: Data on osteoporotic fractures in acromegaly are limited. An increased prevalence of radiological vertebral fractures was already observed in postmenopausal women with active acromegaly. It is unknown whether this observation may reflect a more general increased risk of fractures in acromegaly. DESIGN: This was a cross-sectional study. SETTING: The study was conducted at referral centers. PATIENTS AND CONTROL SUBJECTS: Subjects included 40 males with acromegaly (25 patients with controlled disease and 15 patients with active disease) and 31 control males, with age and gonadal status comparable with the patients. INTERVENTIONS: Evaluation of vertebral fractures (quantitative morphometric analysis) and bone mineral density (BMD) at lumbar spine and total hip (dual energy X-ray absorptiometry) was done. MAIN OUTCOME MEASURE: Vertebral fractures were assessed. RESULTS: Although BMD was not significantly different between acromegalic patients and control subjects, the prevalence of vertebral fractures was higher in acromegalic patients as compared with the control subjects (57.5 vs. 22.6%; chi(2): 8.7; P = 0.003). Fractured and nonfractured acromegalic patients showed no significant difference in age and BMD Z-score. However, acromegalic patients with fractures had serum IGF-I values significantly higher and duration of active disease significantly longer with respect to patients without fractures. Moreover, patients with fractures showed significantly longer untreated hypogonadism as compared with patients without fractures. In a multivariate logistic regression analysis, the duration of active acromegaly was the only risk factor significantly correlated with the occurrence of fractures (odds ratio 1.1, confidence interval 1.04-1.6). CONCLUSIONS: This study reports for the first time a high prevalence of osteoporotic vertebral fractures in an unselected acromegalic male population generally considered at low risk of osteoporosis, suggesting that complicated osteoporosis is an important comorbidity of acromegaly.     

Carotid artery protrusion and dehiscence in patients with acromegaly

Purpose

Acromegaly is a systemic disease which causes multiple bony alterations. Some authors reported that acromegalic patients have risk factors for an intraoperative vascular injury due to the specific anatomical features of their sphenoid sinus. The objective of our study was to analyze the anatomic characteristics of sphenoid sinus in acromegalic patients compared with controls, by evaluation of computed tomography (CT) findings.

Methods

We examined 45 acromegalic (acromegaly group) and 45 non-acromegalic patients (control group) with pituitary adenomas who were matched for sex, age, height, tumor size, and cavernous sinus invasion (Knosp grade). Preoperative CT of the pituitary region including the sphenoid sinus was used to evaluate the following anatomic characteristics: type of sphenoid sinus (sellar or pre-sellar/conchal); intrasphenoid septa (non/single or multiple); carotid artery protrusion; carotid artery dehiscence; intercarotid distance.

Results

Sixteen acromegalic patients (35.5 %) and 6 controls (13.3 %) had carotid artery protrusion. Additionally, 10 acromegalic patients (22.2 %) and 3 controls (6.6 %) had carotid artery dehiscence. Carotid artery protrusion and dehiscence were more frequent in the acromegaly group than in control group (p = 0.013 and 0.035, respectively). Other anatomic characteristics (type of sphenoid sinus, intrasphenoid septa, and intracarotid distance) showed no significant differences between acromegaly and control groups.

Conclusions

Our study suggests that carotid artery protrusion and dehiscence occur more frequently among acromegalic patients, compared with non-acromegalic patients. It is important for surgeons to be aware of these anatomic variations to avoid vital complications, such as carotid injuries, during surgery.

     

The longitudinal effect of body adiposity on joint mobility in young males with Haemophilia A

Summary. Although body adiposity and disease severity in haemophilia have been found in cross‐sectional studies to be negatively associated with joint mobility, it is not clear how these two factors affect the rate of joint mobility loss over time. Over a 10‐year period, repeated measures of joint range of motion (ROM) were collected annually using universal goniometers on bilateral hip, knee, ankle, shoulder and elbow joints in 6131 young males with haemophilia A aged ≤20 years. Body mass index (BMI) was calculated using data on weight and height during follow up. The effect of body adiposity, adjusted for disease severity, on the rate of joint mobility loss over time was assessed using a longitudinal model. Compared with haemophilia males with normal BMI, those who were obese had lower ROM at initial visit and a faster rate of joint mobility loss in the lower limbs. Overweight subjects experienced similar loss in ROM, although to a lesser degree. A decline in ROM with age was also observed in upper limb joints but the rate was not significantly affected by body adiposity. Haemophilia severity, joint bleeding and the presence of an inhibitor were other significant contributors to joint mobility loss in both upper and lower limb joints. Excess body adiposity accelerates joint mobility loss in weight bearing joints particularly among those with severe haemophilia. Our findings suggest that body weight control and effective treatment of bleeds should be implemented together to achieve better joint ROM outcomes in males with haemophilia.     

Erosive and inflammatory joint changes in hereditary hemochromatosis arthropathy detected by low-field magnetic resonance imaging

The aim of the article is to describe and characterize the hemochromatosis arthropathy of the hand by low-field MRI in symptomatic and asymptomatic patients. Forty-nine patients with hereditary hemochromatosis (37 with and twelve without arthropathy) were examined clinically and by low-field MRI of the hands. The examination showed heterogeneous degenerative and inflammatory joint changes such as erosions (in 84 % of all symptomatic patients), synovitis (77 %), bone marrow edema (38 %), subchondral cysts (30 %), tenosynovitis (30 %), joint space narrowing (73 %) and osteophytes (59 %) including hook-shaped osteophytes at MCP joints (32 %). Mild joint changes were also seen in a lower percentage of asymptomatic patients. This is the first larger study addressing the joint changes of the hand in hereditary hemochromatosis using low-field MRI. Our study emphasizes the inflammatory and destructive character of the arthropathy besides the well-known degenerative joint changes described in conventional X-ray. The impact of joint changes in asymptomatic patients deserves further investigation.     

Janus kinase (JAK) 2 V617F mutation as the cause of primary thrombocythemia in acromegaly with severe visceromegaly and divergence between growth hormone and insulin-like growth factor-1 concentrations during the follow-up: causal or casual association?

ObjectiveAn increased prevalence of hematological abnormalities is reported in acromegaly, but to date no reports about the presence of the Janus Kinase (JAK) 2 mutation in acromegalic patients have been described.DesignWe report the complex clinical presentation of the unique case, never described, of acromegaly due to GH-secreting pituitary adenoma associated with JAK2 V617F mutation.ResultsThe patient shows primary thrombocythemia and myelofibrosis, due to JAK2 V617F mutation, severe visceromegaly and a peculiar clinical course of the disease characterized by discrepant values of GH and IGF-1 during somatostatin analog (SA) treatment despite a significant reduction in pituitary adenoma size and therapeutic resistance both to SA and pegvisomant.ConclusionsThe presence of JAK2 V617F mutation is a cause of primary thrombocythemia and myelofibrosis in acromegaly. In this patient, a peculiar clinical course of acromegaly was observed, with the difficulty in controlling the disease. More data, on a larger cohort of patients, could clarify whether JAK2 V617F mutation has a serious impact on the clinical features and course of acromegaly.     

Craniofacial abnormalities and their relevance for sleep apnoea syndrome aetiopathogenesis in acromegaly

OBJECTIVE: To explain the effect of craniofacial relations on the development of the sleep apnoea syndrome (SAS) in acromegaly, and to elucidate how the activity of acromegaly affects the severity of SAS. DESIGN: Prospective observational study. METHODS: Cephalometry and sleep ventilation measurements were performed in 26 acromegalic men and in 96 men with SAS. RESULTS: SAS was found in 20 acromegalic men. Compared with non-acromegalic men with SAS, patients with acromegaly and SAS were found to have: enlargement of almost all linear dimensions; increased angle indicating mandibular protrusion; increased difference between maxillary and mandibular protrusion; articular angle decrease; soft palate lengthening; and pharyngeal airway space (PAS) enlargement in the palatal and uvular-tip planes. A comparison of acromegalic men with and without SAS revealed no significant difference in the craniofacial skeleton, although there was a narrowing of the minimal PAS (MinPAS) and of PAS in the uvular-tip plane in patients with SAS. SAS was more frequent in the patients with active acromegaly. MinPAS in the patients with active acromegaly was narrower than in those without disease activity. CONCLUSION: Skeletal abnormalities in acromegalic men with SAS were different from those in SAS patients without acromegaly. Upper airway narrowing due to changes in pharyngeal soft tissues takes a more relevant share in the development of SAS in acromegalic men than skeletal anomalies.     

Cardiac hypertrophy and function in asymptomatic acromegaly.

Heart disease frequently occurs in advanced acromegaly. In order to investigate cardiac mass and function in acromegaly in the absence of obvious cardiac disease, we performed Doppler echocardiography in 15 asymptomatic acromegalic patients (six of them had systemic hypertension). The data were compared with those of a group of 10 age-matched controls. Left ventricular mass index (LVMI) was increased in acromegaly (110 +/- 32 vs 32 +/- 12 g m-2, P = 0.02), but shortening fraction and systolic time intervals did not differ. Mitral EF slope was decreased (80 +/- 21 vs 101 +/- 30 mms-1, P less than 0.02), while the duration of the isovolumic relaxation period (IRP) was increased (92 +/- 13 vs 69 +/- 16 ms, P less than 0.01). Hypertensive acromegalic patients (n = 6) had a higher LVMI than normotensive acromegalic patients (n = 9) (133 +/- 27 vs 94 +/- 24 g m-2, P = 0.02) and this was confirmed by a meta-analysis of data in the literature: the prevalence of hypertrophy was 76% in the presence of hypertension vs 50% in its absence, P less than 0.002. IRP was prolonged in normotensive acromegalic patients vs normal controls (90 +/- 11 vs 69 +/- 16 ms, P less than 0.01). In conclusion, subclinical cardiac abnormalities occur frequently in acromegaly in the absence of obvious heart disease, and hypertrophy is observed in asymptomatic hypertensive acromegaly. Moreover, diastolic abnormalities are found in asymptomatic acromegaly and could be caused by several heart-related factors.     

Cross-sectional prevalence of pancreatic cystic lesions in patients with acromegaly,a single-center experience

Purpose

Acromegaly is a disease associated with an increased risk for several kinds of neoplasms including colon and thyroid cancer. Although the association between acromegaly and pancreatic neoplasms has not been elucidated, it has recently been reported that GNAS gene mutations were found in 58% of intraductal papillary mucinous neoplasms (IPMNs), which are representative pancreatic cystic lesions, suggesting a link between IPMNs and acromegaly. To assess the prevalence of pancreatic cystic lesions in patients with acromegaly, we performed a retrospective cross-sectional single institute study.

Methods

Thirty consecutive acromegalic patients (20 females and 10 males; mean age, 60.9?±?11.9 years) who underwent abdominal contrast-enhanced computed tomography or magnetic resonance imaging between 2007 and 2015 at Kobe University Hospital were recruited. We also analyzed the relationship between presence of pancreatic cystic lesions and somatic GNAS mutations in pituitary tumors.

Results

Seventeen of 30 (56.7%) patients studied had pancreatic cystic lesions. Nine of 17 patients (52.9%) were diagnosed with IPMNs based on imaging findings. These results suggest that the prevalence of IPMNs may be higher in acromegalic patients in acromegalic patients than historically observed in control patients (up to 13.5%). In patients with pancreatic cystic lesions, the mean patient age was higher and the duration of disease was longer than in those without pancreatic cystic lesions (67.0?±?2.3 vs. 53.0?±?2.7 years, p?<?0.001, 15.5?±?2.4 vs. 7.3?±?2.8 years, p?=?0.04). There were no differences in serum growth hormone levels or insulin-like growth factor standard deviation scores between these two groups (21.3?±?6.4 vs. 23.0?±?7.4 ng/ml, p?=?0.86, 6.6?±?0.5 vs. 8.0?±?0.6, p?=?0.70). Neither the presence of somatic GNAS mutation in a pituitary tumor nor low signal intensity of the tumor in T2 weighted magnetic resonance imaging was associated with the presence of pancreatic cystic lesions.

Conclusions

These data demonstrate that old or long-suffering patients with acromegaly have a higher prevalence of pancreatic cystic lesions. Moreover, the prevalence of pancreatic cystic lesions may be increased in acromegalic patients.

     

A Hyperkinetic Heart in Uncomplicated Active Acromegaly

Cardiac function was studied by echocardiography in 12 patients with active acromegaly and in 12 age- and sex-matched healthy control subjects. None of the patients had cardiovascular diseases or other endocrine diseases than acromegaly. The patients had a mean age of 39±5 years and were short-term acromegalic with a mean duration of disease of 6±3 years. Mean left ventricular mass was 163±43 g/m² in the acromegalic group versus 120±24 g/m² in the control group. Preload (the diastolic diameter of the left ventricle) was within normal limits, while afterload (end-systolic meridional wall stress) was significantly decreased in the acromegalic group. Myocardial contractility assessed as fractional shortening of the left ventricle was 39.9±3.6% in the acromegalic group versus 32.9±5.1% in the control group, and cardiac output was increased by 52% in the acromegalic group because of increased heart rate and stroke volume. We suggest that augmented peripheral blood flow is responsible for the condition of cardiac hyperkinesia in short-term acromegaly and involved in the development of hypertension, which is a frequent complication of long-term acromegaly.     

The role of disease severity in influencing body mass index in people with haemophilia: a single‐institutional cross‐sectional study

The aim of this study was to evaluate the effect of haemophilia disease severity and potential intermediaries on body mass index (BMI) in patients with haemophilia. A secondary analysis of a cross‐sectional study of 88 adults with haemophilia was undertaken. On bivariate analysis, persons with severe haemophilia had 9.8% lower BMI (95% CI ?17.1, ?3.0) than persons with non‐severe haemophilia. The effect of haemophilia severity on BMI varied significantly by human immunodeficiency virus (HIV) status. Among HIV‐positive subjects, haemophilia severity was not associated with BMI (+5.0%, 95% CI ?22.4, 41.9). Among HIV‐negative subjects, severe haemophilia was associated with 15.1% lower BMI (95% CI, ?23.6, ?5.7). Older (>41 years) HIV‐negative subjects with severe haemophilia had a BMI that was 24.8% lower (95% CI ?39.1, ?7.0) than those with non‐severe haemophilia. No statistically significant association was detected between BMI and severe vs. non‐severe haemophilia for younger HIV‐negative subjects. Although joint disease, as measured by the World Federation of Hemophilia (WFH) joint score, did not influence the association between haemophilia disease severity and BMI, adjustment for the atrophy component of the WFH score reduced the association between haemophilia severity and BMI by 39.1–69.9%. This suggested that muscle atrophy mediated at least part of the relationship between haemophilia severity and BMI. Haemophilia disease severity is associated with BMI and appears to be mediated by muscle atrophy of surrounding joints. This association appears to be modified by HIV status and possibly age.     

Radiographic features of hereditary articular chondrocalcinosis. A comparative study with the sporadic type

To assess the radiological features of hereditary articular chondrocalcinosis, we performed a blind comparative study between 21 randomly selected patients with hereditary disease and 21 cases of sporadic pseudogout matched for age and sex. Each individual had AP projections of the hands, pelvis and knees. The films were evaluated for the presence of articular chondrocalcinosis and for the severity of the associated degenerative arthropathy. A grade of 0 to 3+ was assigned to each of the 4 variables of osteoarthritis: joint space narrowing, sclerosis, osteophytosis and subchondral cysts. The mean number of joints with chondrocalcinosis and its distribution was similar in both groups. In addition, no differences were found in the overall severity of the associated degenerative arthropathy. In both groups the disease was characterized by oligoarticular calcification and a mild degenerative arthropathy. These data along with data from other reported pedigrees, show that the radiological appearance in the hereditary type is frequently indistinguishable from that commonly observed in sporadic articular chondrocalcinosis.