Systemic sclerosis sine scleroderma: an unusual presentation in scleroderma renal crisis.

Renal involvement in systemic sclerosis (SSc) has a negative influence on prognosis. Cases of SSc sine scleroderma have been reported in which organ failure occurred but was not accompanied by cutaneous damage, which in some instances did develop later. We describe a patient who, after 6 months of symmetric polyarthritis, developed rapid progressive renal failure without skin changes. A diagnosis of scleroderma renal crisis, confirmed histologically, was made. Anti-RNAP III antibodies were positive. The patient developed typical scleroderma skin changes after renal failure. Despite treatment, SSc advanced to fatal endstage renal disease.

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

The 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.

Pneumatosis cystoides intestinalis in systemic lupus erythematosus with intestinal vasculitis: treatment with high dose prednisone.

Pneumatosis 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, Raynaud's 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.

[Prolactin, a link between the neuroendocrine and immune systems. Role in the pathogenesis of rheumatic diseases]. / La prolactine, un lien entre le système neuro-endocrinien et le système immunitaire. Rôle dans la pathogénie des maladies rhumatismales.

Increasing evidence suggest that prolactin (PRL) has important immunoregulatory properties and may play a role in the pathogenesis and disease expression of certain autoimmune diseases. Prolactin is co-mitogenic on murine and human lymphocytes, induces the formation of IL-2 cell surface receptor and modulates the expression of various growth factor related genes. Prolactin also stimulates antibody production both in vivo and in vitro. Specific high affinity prolactin-receptors have been described on T and B lymphocytes as well as on monocytes. They are distributed on heterogeneous lymphocyte subsets and they showed imbalance in autoimmune situations. Lymphocytes may produce PRL-like proteins biologically active that function as autocrine growth factors for lymphoproliferation. Hyperprolactinemia has been found in male patients with systemic lupus erythematosus (SLE) and also during pregnancy in SLE patients. Hyperprolactinemia is correlated with clinical and serological activity in a subset of SLE patients. High levels of PRL aggravates disease activity and accelerates mortality in the B/W mouse model of SLE. In rheumatoid arthritis an excessive and upregulated secretion of PRL has been shown. Hyperprolactinemia has also been shown in a subset of patients with primary Sjögren's syndrome. High PRL levels have been found in Reiter's syndrome patients and bromocriptine treatment has been reported effective in these patients and psoriatic arthritis patients. These data support a potential role of this immunoregulatory hormone in the pathogenesis of some rheumatic diseases.

Digital vasculitis following allergic desensitization treatment.

Systemic necrotizing vasculitis or a polyarteritis nodosa-like clinical presentation, is an unusual complication of immunotherapy (hyposensitization therapy). We describe a patient who developed features of vasculitis several years after beginning hyposensitization treatment for allergic rhinitis. In the 7 months preceding the onset of the vasculitis he experienced 4 episodes of anaphylaxis immediately after receiving desensitization injections. The vasculitis was characterized by the abrupt onset of pain and discoloration of the middle 3 digits of his right hand. Cyanosis and small areas of frank necrosis of these digits were present. Erythrocyte sedimentation rate and C-reactive protein were elevated and total serum complement was decreased. The development of digital vasculitis following hyposensitization is clearly illustrated in this patient.

Candida arthritis.

Septic arthritis can be caused by Candida species. Its true incidence is unknown because only a few cases have been reported. Two clinical syndromes have been identified. The first is an isolated monarthritis caused by the direct intra-articular inoculation of fungi that inhabit the skin, by means of an injection, or during surgery. The second is the development of a mono- or polyarthritis as a complication of hematogenously disseminated candidiasis. The optimum treatment of this condition is not yet known, although the combination of medical and surgical treatment seems to be the most convenient therapeutic modality. Amphotericin-B continues to be the drug of choice.

Other fungal arthritides.

Fungi are not a common cause of musculoskeletal infection; however, reports of fungal bone and joint infections have lately surged and are expected to increase more. There is evidence of an increasing incidence of pathogenic and opportunistic fungal infections and emergence of new species of disease-causing fungi, particularly in immunosuppressed patients. Distribution is worldwide, signs of infection can be mild, and chronic evolution as well as delayed diagnosis are common.