Marta Lucia Cuellar

Refractory Nephrotic Syndrome in Lupus Nephritis: Favorable Response to Indomethacin Therapy

The effects of indomethacin on urinary protein excretion, levels of serum albumin and renal function were studied prospectively in six patients with systemic lupus erythematosus (SLE) and refractory nephrotic syndrome due to lupus nephritis. Two had membranoproliferative glomerulonephritis, two had diffuse proliferative glomerulonephritis, and one each had mesangioproliferative and membranous glomerulonephritis. All experienced a considerable reduction in urinary protein excretion and an increase in serum albumin. Indomethacin was discontinued in two patients because of side effects, and proteinuria recurred to pretreatment levels. The decrease of proteinuria continued during long-term treatment in three patients. Indomethacin did not cause a permanent decline in renal function. Our results suggest that therapy with indomethacin may be beneficial for the treatment of refractory nephrotic syndrome in selected SLE patients. However, because of potential side effects the administration of indomethacin should be monitored closely.

Pneumatosis-Cystoides-Intestinalis in Systemic Lupus-Erythematosus with Intestinal Vasculitis - Treatment with High-Dose Prednisone

SummaryPneumatosis cystoides intestinalis (PCI) is an uncommon disorder usually associated with intestinal and pulmonary obstructive diseases, recent abdominal procedures and systemic illnesses. PCI has been reported in patients with systemic lupus erythematosus associated with intestinal vasculitis. We describe herein a patient with a month history of intermittent abdominal pain, diarrhoea, hyporexia, and weight loss who underwent intestinal resection for acute abdomen. Post-operatively she gave a three-month history of arthritis of the right knee, ankles and feet, arthralgia of the wrists, MCPs and shoulders. She also described weakness, weight loss, Raynauds phenomenon, and a skin rash. Laboratory examination revealed an increased ESR, low haemoglobin and haematocrit, positive rheumatoid factor, a positive ANA with a speckled pattern, as well antibodies to DNA, SS-A and cardiolipin. The abdominal symptomatology especially pain, cramps and bouts of diarrhoea persisted after the surgery and became worse two months later. Abdominal X-ray showed distention of bowel with cyst formation in the wall of the entire colon. A diagnosis of PCI was made radiologically. The intestinal pathology was reviewed and vasculitis was identified. The patient received treatment with high dose prednisone with an excellent response; prednisone was progressively tapered and she has been asymptomatic without abdominal complaints or other symptoms for over a year.

HIV INFECTION–ASSOCIATED INFLAMMATORY MUSCULOSKELETAL DISORDERS

Inflammatory musculoskeletal complaints are relatively common during the course of HIV infection, although they tend to be more frequent during late stages. The clinical spectrum is varied, ranging from arthralgias to distinct rheumatic disorders, such as Reiters syndrome and psoriatic arthritis. The therapeutic management often poses a challenge, although most patients respond to conventional first- and second-line anti-inflammatory medications.

METHOTREXATE USE IN PSORIASIS AND PSORIATIC ARTHRITIS

Methotrexate is an extremely effective drug in the treatment of psoriasis and psoriatic arthritis. In addition, it possesses a very high benefit-to-toxicity ratio compared with other therapies and immunosuppressive agents used in these disorders. Fortunately, most adverse events related to methotrexate are mild, but serious and life-threatening reactions, particularly liver toxicity, may occur. Careful monitoring is essential to prevent most undesirable side effects.

Treatment of psoriatic arthritis

The treatment of psoriatic arthritis has acquired relevance in the past few years because of advances and better understanding of the pathogenetic mechanisms implicated in the disease, and also because of recognition that this disorder is not a benign disease as was previously thought. The general principles of management for any inflammatory arthritis, including pharmacological, surgical and rehabilitative treatment, are to be used, with concomitant therapeutic management of skin involvement. Non-steroidal anti-inflammatory drugs constitute the mainstay of pharmacological therapy for most patients, with a good clinical response observed in 75-85%. Early use of remittive agents, especially methotrexate, is indicated in patients with a poor response to NSAIDs or those with polyarticular and progressive joint involvement. Patients who are refractory to conventional therapy should be considered for newer and potentially more toxic therapeutic modalities such as cyclosporin A and retinoids.

Management of spondyloarthropathies.

Better understanding of the etiopathogenic mechanisms and increasing recognition of the natural course of the spondyloarthropathies are leading to a more rational therapeutic approach to several of the disorders included within this large group of arthritides. Our traditional therapeutic approach to these conditions is being challenged, and a more aggressive therapeutic regimen is being advocated in a manner not too much different from that advocated for the treatment of rheumatoid arthritis. Combination therapy with either methotrexate and sulfasalazine or methotrexate and cyclosporine is being used more often and earlier, particularly in psoriatic arthritis. An issue, however, that remains unresolved is the proper use of antibiotic therapy.

Recent developments in psoriatic arthritis

Psoriatic arthritis affects 5% to 7% of patients with psoriasis. Genetic, immunologic, and environmental factors play a role in its pathogenesis. The role of inflammatory cytokines has been better defined, and recent immunohistochemical studies of the synovial membranes have shown important differences and similarities between psoriatic arthritis and rheumatoid arthritis. The association of psoriatic arthritis with infection, particularly HIV, remains an interesting observation. The most common clinical presentation appears to be peripheral polyarticular, and extra-articular manifestations including the SAPHO (synovitis, acne, pustulosis, hypertosis, and osteitis) syndrome are not common. Methotrexate and sulfasalazine therapy are effective in patients who do not respond to nonsteroidal anti-inflammatory drugs.

Eosinophilic hepatitis: A new feature of the clinical spectrum of the eosinophilia-myalgia syndrome

SummaryThe eosinophilia-myalgia syndrome associated with L-tryptophan-containing products is highlighted by eosinophilia, incapacitating myalgias, and diverse multisystemic manifestations. In addition to involvement of the skin, skeletal muscle, and peripheral nerves, visceral damage has been quite prominent, particularly affecting the lungs, the heart, and the liver. Hepatic involvement has been manifested by altered liver tests but is clinically silent. We report the unique case of a woman with this syndrome who developed abdominal pain, a clinical picture of hepatitis and chronically abnormal liver tests. Histologic examination of the liver disclosed eosinophilic hepatitis with piecemeal necrosis. The occurrence of clinically overt hepatic involvement has not been reported previously. Potential mechanisms of liver damage in eosinophilia-myalgia syndromes are discussed.