Pulsed versus continuous wave low-level light therapy on osteoarticular signs and symptoms in limited scleroderma (CREST syndrome): a case report.

Limited cutaneous systemic sclerosis (lcSSc) was formerly known as CREST syndrome in reference to the associated clinical features: calcinosis, Raynaud's phenomenon, esophageal dysfunction, sclerodactyly, and telangiectasias. The transforming growth factor beta has been identified as a major player in the pathogenic process, where low-level light therapy (LLLT) has been shown to modulate this cytokine superfamily. This case study was conducted to assess the efficacy of 940 nm using millisecond pulsing and continuous wave (CW) modes on osteoarticular signs and symptoms associated with lcSSc. The patient was treated two to three times a week for 13 weeks using a sequential pulsing mode on one elbow and a CW mode on the other. Efficacy assessments included inflammation, symptoms, pain, health scales, patient satisfaction, clinical global impression, and adverse effects monitoring. Considerable functional and morphologic improvements were observed after LLLT, with the best results seen with the pulsing mode. No adverse effects were noted. Pulsed LLLT represents a treatment alternative for osteoarticular signs and symptoms in limited scleroderma (CREST syndrome).

Anemia and leukopenia in a long-term parenteral nutrition patient during a shortage of parenteral trace element products in the United States.

Recently, drug shortages in the United States have affected multiple components of the parenteral nutrition (PN) solution. A 62-year-old patient with systemic sclerosis who was dependent on home PN due to intestinal dysmotility developed anemia and leukopenia approximately 4 months after parenteral copper was withheld from her PN solution due to drug shortages. The patient was not able to tolerate a sufficient amount of oral multivitamins with trace elements due to severe dysphagia. Her serum copper and ceruloplasmin concentrations were undetectable, confirming the diagnosis of severe copper deficiency. The hematological abnormalities promptly resolved with copper supplementation. This report emphasizes the importance of close monitoring for nutrient deficiencies during drug shortages and supplementing with oral or enteral nutrition when feasible, particularly in high-risk patients such as those with intestinal malabsorption or short bowel syndrome who are dependent on long-term PN.

Talidomida no tratamento de angiodisplasias intestinais em paciente com síndrome CREST: relato de caso e revisão da literatura / Thalidomide as a treatment of intestinal angiodisplastic lesions in a patient with CREST syndrome: case report and literature review

A anemia ferropriva grave secundária à hemorragia digestiva por angiodisplasias intestinais representa um grande desafio terapêutico. Comumente, as ectasias vasculares são múltiplas e dispersas ao longo do intestino, limitando a eficácia do tratamento hemostático local. Nos últimos anos houve significativo avanço no tratamento anti-angiogênico sistêmico das angiodisplasias intestinais, sendo talidomida a droga mais empregada para tal fim. Relatamos o caso de uma paciente de 49 anos com angiodisplasias intestinais secundárias a síndrome CREST (Calcinose, Raynaud, Dismotilidade Esofágica, Esclerodactilia e Telangiectasias). A paciente apresentava quadro de melena recorrente e alta necessidade transfusional, e não obteve resposta clínica após realização de enteroscopia e eletro-coagulação das lesões com plasma de argônio. Após a introdução de talidomida 100mg ao dia, a paciente evoluiu de forma bastante satisfatória. O caso apresentado neste texto, além de demonstrar sucesso da talidomida no tratamento de angiodisplasias intestinais refratárias à eletro-coagulação com plasma de argônio, também revela eficácia da droga na situação específica da síndrome CREST. Tal fato pode ser de grande valia quando da abordagem de hemorragia intestinal por angiodisplasias nesses pacientes, representando nova opção terapêutica...

The severe ferropenic anemia secondary to digestive bleeding due to intestinal angiodisplastic lesions represents a great challenge. Commonly, angiodisplastic lesions are multiples and disperse through the intestine and that fact limits local treatments. Over the last years, there was a great advance in the antiangiogenic treatment of intestinal angiodisplastic lesions and thalidomide was the most employed drug for this purpose. We report a case of a 49 year-old patient with intestinal angiodisplastic lesions due to CREST syndrome (Calcinosis, Raynaud phenomenon, Esophageal dysmotility, Sclerodactyly, and Telangiectasia). The patient presented repeated episodes of digestive bleeding and did not achieve clinical improvement after enteroscopy and argon plasma coagulation. The treatment consisting of the introduction of thalidomide 100mg per day demonstrated success. The case presented in this text reveals success in the use of thalidomide in the treatment of intestinal angiodisplastic lesions, probably representing a new therapeutic option...

Primary localized cutaneous nodular amyloidosis and CREST syndrome: a case report and review of the literature.

Primary localized cutaneous nodular amyloidosis (PLCNA) is a form of primary localized cutaneous amyloidosis (PLCA) that presents as yellowish waxy nodules on the extremities, face, trunk, or genitalia. We report the case of a patient with PLCNA and CREST (calcinosis, Raynaud phenomenon, esophageal motility disorders, sclerodactyly, and telangiectasia) syndrome. A diagnosis of her extensive PLCNA was made after biopsy specimens from the bilateral shins stained positive for amyloid extending from the superficial papillary dermis to the subcutis. Results of a workup were negative for paraproteinemia or signs of systemic amyloidosis and have remained so after 8 years of follow-up. We present a review of the literature describing the presentation and histopathology of the varying forms of amyloidosis.

Acoustic pressure wound therapy in the treatment of a vasculopathy-associated digital ulcer: a case study.

Digital vasculopathy and subsequent digital ulceration are common and painful complications of limited cutaneous systemic sclerosis. Although the use of hydrocolloid occlusive dressings has been found to reduce pain, frequently required surgical or chemical debridement can be intensely painful in such ulcers. Acoustic pressure wound therapy is a noncontact, low-frequency ultrasound therapy used for painless debridement in a variety of acute and chronic wounds. It was administered to treat an intensely painful, methicillin-resistant Staphylococcus aureus-infected finger ulcer resulting from peripheral, bilateral vasculopathy in a 68-year-old man with a history of three prior fingertip amputations secondary to limited cutaneous systemic sclerosis-associated digital vasculopathy. At treatment initiation, 90% of the 11 cm2 wound was covered with firmly adherent fibrin slough. Acoustic pressure wound therapy was performed three times weekly for 5 minutes per treatment and the wound was covered with a hydrocolloid occlusive dressings. Pain scores decreased from 10 (visual analog scale, 0 = none, 10 = extreme) at the beginning of treatment to 0 at the week 8 assessment and his analgesics were discontinued. After 10 weeks (31 acoustic pressure wound therapy treatments), the wound was completely closed.

[Manifestations of scleroderma at the hand--options for hand surgery in an interdisciplinary concept]. / Handbeteiligungen im Rahmen der progressiven systemischen Sklerodermie--Möglichkeiten der Handchirurgie im interdisziplinären Konzept.

Clinical manifestations of scleroderma at the hand include Raynaud's phenomenon, calcinosis cutis, sclerodactylia and teleangiectasia. With the progression of the disease, cutaneous and joint contractions, acro-osteolysis, necrosis of the finger tips, and even extensive digital ulceration are likely to occur. These painful and often rapidly advancing lesions cause loss of function and disfigurement and, untreated, often lead to mutilation of the affected hand. Only an interdisciplinary management including the hand surgeon, the rheumatologist, and the physiotherapist can guarantee optimal treatment. Drug therapy should be included as well as physical therapy. Both should be made use of before and accompanying surgical treatment. Surgical therapy consists of treatment of the infections, excision of calcinosis, arthrodesis, in particular of the proximal interphalangeal joints, and sympathectomy. Amputation remains a final option, whereas with timely and sufficient treatment, amputations can be avoided and an improvement of function and an alleviation of the symptoms can be achieved. Among the non-operative treatment options, behavioural training, calcium antagonists, prostacyclin derivatives, topical nitrates as well as plexus anesthesia and stellatum blocks have proved to be effective. Recent drug therapies include endothelin-receptor antagonists for the prevention of digital ulceration and phosphodiesterase-V antagonists in treatment of Raynaud's phenomenon and induction of ulcer healing. With reference to several cases seen at our institution, we propose an interdisciplinary treatment concept for acral manifestations of scleroderma.

Local implantation of autologous mononuclear cells from bone marrow and peripheral blood for treatment of ischaemic digits in patients with connective tissue diseases.

OBJECTIVE: CD34-positive bone marrow mononuclear cells (MNCs) have been successfully used for regeneration of small arteries in Buerger's disease. The objective of this study is to examine the angiogenetic potential of autologous MNCs from bone marrow and peripheral blood implanted into the ischaemic digits from patients with connective tissue diseases. METHODS: Three patients with systemic sclerosis, two with mixed connective tissue disease, and one with CREST syndrome were enrolled who had painful ischaemic digits with necrosis refractory to several vasodilators including intravenous prostaglandins. MNCs obtained from 7 ml/kg bone marrow blood and 400 ml peripheral blood were implanted into 20 different sites in palms and/or soles. The study was performed open-labelled. RESULTS: Pain in the numeric rating scale improved remarkably up to 1 month after implantation of bone marrow or peripheral MNCs to the same extent, although no significant differences were found in transcutaneous oxygen pressure and thermogram before and after the implantation. Bone marrow MNCs increased blood flow of the hand determined by intra-arterial digital subtraction angiography, while peripheral MNCs did not. CONCLUSIONS: Implantation of autologous MNCs from peripheral and bone marrow into the ischaemic digits was so effective in pain-relief and more clinical trials would be warranted to see whether this could be a new treatment modality for angiogenesis in connective tissue diseases as in Buerger's disease.

Síndrome de Crest, sclerodermia sistémica progresiva: ¿cómo tratar y mantener un periodonto? Caso clínico / Crest syndrome, progressive systemic scleroderma: how to treat and mantein a periodontium? Clinical case

El síndrome de Crest es una enfermedad autoinmune. Su diagnóstico es un desafío, los tratamientos sistémicos yperiodontal son muy difíciles. El caso clínico que presentamos, tratado en la práctica privada, ofrece una visión de esta enfermedad. Asimismo, permite hacer una crítica a los sistemas de salud que no la contemplan y categorizan como discapacitados a este tipo de pacientes.

Síndrome de Crest, sclerodermia sistémica progresiva: ¿cómo tratar y mantener un periodonto? Caso clínico / Crest syndrome, progressive systemic scleroderma: how to treat and mantein a periodontium? Clinical case

El síndrome de Crest es una enfermedad autoinmune. Su diagnóstico es un desafío, los tratamientos sistémicos yperiodontal son muy difíciles. El caso clínico que presentamos, tratado en la práctica privada, ofrece una visión de esta enfermedad. Asimismo, permite hacer una crítica a los sistemas de salud que no la contemplan y categorizan como discapacitados a este tipo de pacientes.(AU)

[Pulmonary arterial hypertension in collagenoses: clinical features, epidemiology, pathogenesis, diagnosis and treatment]. / Pulmonale arterielle Hypertonie bei Kollagenosen: Klinik, Epidemiologie, Pathogenese, Diagnostik und Therapie.

Pulmonary arterial hypertension (PAH) is a severe vasculopathy, which is characterised by progressive narrowing and obliteration of the pulmonary arterioles and increased endothelin-1 levels. The increase of vascular resistance in the lung vessels leads to chronic pressure overload and to right heart failure, if untreated. PAH often occurs in association with rheumatic-inflammatory diseases (e.g., in 15% of patients with systemic sclerosis (SSc), especially in the limited form or in CREST patients) and determines their prognosis: in advanced stages, untreated patients die within a short period. Therefore all SSc patients, particularly the newly diagnosed ones, should be screened for PAH with echocardiography. If PAH is suspected, a right heart catheter should be performed, and if PAH is confirmed, adequate treatment should be initiated. While few years ago lung transplantation was the only option for patients with severe PAH, in recent years enormous progress was seen in drug treatment. Today prostanoids (Ventavis) and the endothelin receptor antagonist bosentan (Tracleer) are available for patients with PAH in WHO/NYHA stage III: they have substantially improved the prognosis of PAH in the last years. Since few months, also the phosphodiesterase inhibitor sildenafil (Revatio) is available. The combination of drugs with different mode of action will likely further improve the prognosis of PAH patients.

Treatment of cutaneous calcinosis in CREST syndrome by extracorporeal shock wave lithotripsy.

We describe the unusual case of a 78-year-old woman consulting for extensive and painful wound leg ulcerations and calcifications secondary to CREST syndrome that was treated by extracorporeal shock wave lithotripsy. This treatment was considered because of the severity of our patient's symptoms and her failure to respond to various medical and surgical treatment.

Scleroderma with type III glomerulonephritis and MPO-ANCA antibodies in the serum.

Scleroderma is an autoimmune disease characterized by early inflammatory infiltrates followed by fibrosis in the skin and internal organs. CREST is a relatively benign cutaneous variant of scleroderma that features calcinosis, Raynaud's phenomenon, oesophageal dysfunction, sclerodactyly and telangiectases. Glomerulonephritis is a rare association of CREST. We are reporting a patient with CREST who developed glomerulonephritis and had anticentromere and antineutrophil cytoplasmic autoantibodies (ANCA) in her serum.

[Extracorporeal photopheresis in progressive systemic sclerosis: discrimination of responders and non-responders]. / Die Extrakorporale Photopherese bei Progressiver Systemischer Sklerodermie: Unterscheidung von Respondern und Non-respondern.

BACKGROUND: The benefit of extracorporeal photopheresis (ExP) in progressive systemic sclerosis (PSS) is controversial. There is limited experience with the long-term use of ExP in PSS. The purpose of the present study was to distinguish between responders and non-responders by using ExP in PSS and to evaluate activation markers for PSS. PATIENTS AND METHODS: 20 subjects with PSS were treated for 12 months with ExP (interval: 1x/month) as an immunomodulating monotherapy (11/20) or combination therapy (9/20). The course of PSS was assessed by both specially designed clinical score and serological parameters (CRP, ANA, beta-galactosidase, P-III-P, CD4/CD8-ratio, TNF-alpha, 11-2-R, 11-6). RESULTS: After 12 cycles of ExP, 30% of the subjects showed a partial remission and 25%, stable disease (55% responders) while 45% had a progression (non-responders). Although there was no correlation between the clinical course and the serological parameters, an increase of beta-galactosidase during therapy marked a progression of PSS in non-responders. Responders with a short PSS-course before ExP, moderate ANA titres, normal TNF-alpha levels and lack of Scl-70 had a good prognosis. CONCLUSIONS: About the half of the subjects with PSS profited by the long-term use of ExP. Thereby the mild immunomodulating effect of ExP seems to be insufficient to control markedly progressive courses of PSS.

Pulmonary hypertension with limited cutaneous scleroderma (CREST syndrome).

A patient is described with a typical manifestation of pulmonary hypertension associated with limited cutaneous scleroderma, also known as CREST syndrome. The patient was treated with a calcium antagonist, oral anticoagulation and, because of evidence for parenchymal inflammation of the lung, with low-dose prednisone and cyclophosphamide. This treatment resulted in initial improvement of diffusion capacity and exercise tolerance, however, 1 year after diagnosis the patient died of progressive pulmonary hypertension.