Thomas M. Zizic

Neuropsychiatric manifestations of systemic lupus erythematosus: diagnosis, clinical spectrum, and relationship to other features of the disease.

1. Among patients with SLE, 71 (51%) had significant neuropsychiatric problems during the course of the disease. In 52 (37%), the nervous system manifestations were secondary to SLE. 2. The most frequent manifestations were psychiatric dysfunction, seizures, long tract signs, cranial neuropathy, and peripheral neuropathy. 3. Psychiatric abnormalities secondary to SLE were characterized by organic features (present in 22 of 24) and by the association of neurologic lesions which were often diffuse or multifocal. 4. An abnormal cerebrospinal fluid was found in 32% of neuropsychiatric episodes in which specimens were obtained. The most frequently abnormal study was the electroencephalogram (71%), and the least frequent was the brain scan (8%). These studies did not correlate with specific clinical patterns. 5. In 63% of the patients, NP manifestations preceded the diagnosis of SLE or occurred within the first year of diagnosed disease, and in most episodes were associated with evidence of clinical and/or serologic activity of the underlying illness. 6. Only two clinical features showed significant and striking correlations with neuropsychiatric involvement, namely vasculitis and thrombocytopenia. The possible pathogenic implications have been discussed. 7. Only 2 of the 140 patients were felt to have steroid-induced psychoses. In approximately one-half of the NP episodes secondary to SLE, patients were receiving no corticosteriods on presentation. Of those developing while patients were on steroids, the majority occurred on low doses or after tapering from higher levels. 8. The immediate prognosis for improvement in neuropsychiatric function was good with 84% of episodes showing complete or partial resolution. Corticosteroids appeared to be of benefit in a substantial number of patients although their precise role is difficult to quantitate. 9. Five and 10 years survivals for the overall population were 94% and 82%, respectively. There were no significant differences in survival for patients with or without nervous system involvement.

Corticosteroid therapy associated with ischemic necrosis of bone in systemic lupus erythematosus

In this prospective study, 28 (52 percent) of 54 patients with systemic lupus erythematosus (SLE) had ischemic necrosis of bone in 93 sites. All but two of the patients had multiple sites of involvement, with hips, knees, and shoulders affected in decreasing order of frequency. Demographic, clinical, and laboratory features were comparable in patients with and without ischemic necrosis of bone except for cushingoid changes in 24 (86 percent) of the 28 patients with ischemic necrosis of bone versus four (15 percent) of the 26 patients without ischemic necrosis of bone (p less than 0.0001). The duration of steroid therapy, total cumulative steroid dose, and the mean daily prednisone dose for the first one, three, six, and 12 months of therapy were not significantly different between the two groups. Mean daily prednisone dose for the highest single month as well as the highest consecutive three, six, and 12 months of therapy was significantly higher in patients with ischemic necrosis of bone. The mean daily dose of prednisone for the highest month of therapy was greater than 40 mg per day in 93 percent and greater than 20 mg per day in all patients with ischemic necrosis of bone. In patients with ischemic necrosis of bone, there was a statistically significant correlation between higher mean prednisone dose at all time intervals and increased number of bony sites involved. A lower mean dose of prednisone was required to produce ischemic necrosis of bone in patients with Raynauds phenomenon.

Acute abdominal complications of systemic lupus erythematosus and polyarteritis nodosa.

Fifteen (11 percent) of 140 patients with systemic lupus erythematosus (SLE) and five (31 percent) of 16 patients with polyarteritis nodosa (PA) developed disease-related signs and symptoms of an acute surgical abdomen. Peripheral vasculitis (p less than 0.025), nervous system involvement (p less than 0.05), ischemic necrosis of bone (p less than 0.05), thrombocytopenia (p less than 0.01), and circulating rheumatoid factor (p less than 0.01) are all significantly more prevalent in patients with SLE and abdominal involvement compared with those without an abdominal syndrome. Similarly, except for ischemic necrosis of bone, these same features are more prevalent in patients with PA and acute abdomens. Eleven (73 percent) of 15 patients with SLE had exploratory laparotomy for acute abdominal events. Nine were found to have evidence of intra-abdominal arteritis and only two had polyserositis without gross evidence of vasculitis. Eight (53 percent) of the 15 patients with SLE and acute abdomens died as a result of their abdominal crises. Six of the seven surviving patients represent those most recently seen and aggressively treated. All five patients with PA and acute abdomens required operative intervention and all had gross evidence of mesenteric arteritis with large ischemic segments resulting in infarction and perforation. All died in contrast to only two (18 percent) of the 11 PA patients without abdominal involvement.

Alcoholism associated ischemic necrosis of the femoral head. Early diagnosis and treatment.

Thirty-eight hips in 26 patients with alcoholism associated ischemic necrosis of the femoral head (INFH) have been evaluated by measurement of intraosseous pressure, (IOP) pressure response to a 5 ml saline load injected intraosseously and intraosseous venography. All stages of INFH were characterized by elevated IOP and/or an increased pressure response to the saline load compared to normal controls. Intraosseous venography in INFH was characterized by poor or absent visualization of the main efferent veins draining the proximal femur, diaphyseal reflux and delayed evacuation of dye from the bone. It has been possible to identify both a preradiologic and even preclinical stage of INFH. Core decompression, the removal of an 11 mm plug of bone from the head and neck of the femur, has been carried out on all hips in the study. All patients had pain prior to surgery. Eight patients in Stage I (preradiologic stage) are symptom free and without radiologic progression at follow-up, average 24 months. Only one of 5 patients in Stage II (no collapse of the femoral head) has shown X-ray progression while all remain asymptomatic. In Stage III (collapse, without acetabular involvement), core decompression offers relief of symptoms but does not appear to prevent further head collapse.

Ischemic necrosis of bone in systemic lupus erythematosus

Twenty-three patients with systemic lupus erythematosus (SLE) and ischemic bone necrosis are reported. All patients had received corticosteroids prior to the onset of ischemic necrosis, although one patient had received none for 13 years previously. Nineteen (83%) patients had multiple bone lesions including the femoral heads in 21 (91%) which were bilaterally involved in 15. In addition, humeral heads were affected in seven patients and the tibial plateaus, in three. The most striking feature of this group was the high incidence of Raynauds phenomemon present in 14 (61%) of the 23 patients. Furthermore, central nervous system involvement was present in 10 (43%) patients, more prominent in those without Raynauds (67%) than in those with vasospasm (29%). Thus, 20 of the 23 patients, or 87%, evidenced vascular abnormalities either in the form of Raynauds phenomenon and/or systemic vasculitis. The pathogenesis of ischemic bone necrosis is discussed. In SLE, vasospasm or vasculitis, or both, augmented by corticosteroid therapy, could impede the microcirculation and result in the ischemic lesion.

Ischemic bone necrosis in systemic lupus erythematosus: I. The early diagnosis of ischemic necrosis of bone

It would appear from our experience thus far, that increased bone marrow pressure and altered venous drainage, as documented by venography, are present in all stages of ischemic necrosis of bone, including the pre-radiologic, and may be the earliest detectable changes in this disorder. This implies that any postulated pathogenetic mechanism must take into account the early appearance of significant venous derangement, although not necessarily indicating that the venous abnormalities are primary. The data indicate that the venous circulation participates in and undoubtedly contributes to the progression of the disorder. In addition the successful results of core decompression reported in Part II, lend support to such a pathogenetic sequence, and may well be due to the interruption of this vicious circle.

Long-term experience with piroxicam in osteoarthritis

The long-term efficacy and safety of piroxicam 20 mg once daily in the treatment of osteoarthritis was evaluated in 30 patients--eight for three to five years, seven for six years, and 15 for more than seven years--for a total mean duration of six years. More than 93 percent (28/30) reported feeling well at the last visit, while 12 said they were either asymptomatic or had only mild symptoms. Total joint pain scores, total joint swelling and specific functional activity levels improved and remained improved throughout the study period. Side effects were relatively frequent but mostly in the mild to moderate class (159/166) and these were mostly gastrointestinal. Patients preferred remaining on the drug for a mean of six years rather than changing to alternative therapies made available to them.

THE TREATMENT OF RHEUMATOID ARTHRITIS OF THE HAND WITH PULSED ELECTRICAL FIELDS

The safety and effectiveness of stimulation from pulsed electrical fields using the Bionicare® Stimulator System Model BIO-1000TM was investigated for the treatment of rheumatoid arthritis (RA) of the hand. Eighty-nine patients were enrolled in a multi-center, double-blind, placebo device controlled clinical study. All patients met the inclusion and exclusion criteria of the American College of Rheumatology (ACR) for rheumatoid arthritis1 and had active symptomatic synovitis. Background arthritis medications were maintained constant throughout the study. Device was used for 8±2 hours daily for a four week treatment period. The weekly efficacy assessments included the physician’s global evaluation of the treated hand, the patient’s assessments of pain/symptoms and function, joint tenderness and swelling, range of motion, grip strength, morning stiffness and activities of daily living. The active device group demonstrated significant improvement compared to the placebo device group using repeated measures models for the physician’s global evaluation, the patient’s evaluation of pain/symptoms and the patient’s evaluation of function, all of the treated hand. There were no consistently significant trends for the other outcome measures. Transient skin rash, the only type of adverse event reported, was comparable between groups. This study suggests the Bionicare stimulator is safe and effective for treating rheumatoid arthritis of the hand.

A Pulsed Electrical Joint Stimulator for the Treatment of Osteoarthritis of the Hand and Wrist

The hand is commonly affected by osteoarthritis (OA). The development and progression of OA are believed to involve inflammation, even in the early stages of the disease. Inflammatory and proinflammatory cytokines have also been shown to be elevated in the flexor tenosynovium of idiopathic carpal tunnel syndrome (CTS). A large percentage of patients with hand OA also have a concomitant CTS. This study evaluated the results of a pulsed electrical joint stimulator in patients who had hand OA with or without CTS. Pain, tenderness, and swelling; grip strength and pinch force; and Patient and Physician Global Assessment and Disabilities of the Arm, Shoulder and Hand (DASH) results were evaluated. The primary efficacy outcome was pain due to OA in the study hand in the past 48 hours. Secondary outcomes consisted of OA pain in the study thumb in the past 48 hours, grip strength, pinch force, and Patient and Physician Global Assessment and DASH results. All 7 outcome parameters improved in OA patients. On physical examination, individual finger and wrist joints had also improved regarding pain, swelling, and tenderness. In the subset of patients with CTS, CTS pain, paresthesia, weakness, and all CTS symptoms had significantly improved. Patient and Physician Global Assessment and DASH results and pinch force were also significantly improved. This pulsed electrical joint stimulator is effective in providing clinically relevant and statistically significant reductions in the signs and symptoms of OA of the hand and CTS. It could be a useful modality for the treatment of patients who have one of these conditions or both. [Orthopedics. 2018; 41(4):e550-e556.].