Peptide receptor radionuclide therapy as a new tool in treatment-refractory sarcoidosis - initial experience in two patients.

Sarcoidosis is a multisystem granulomatous disorder of unknown etiology that can involve virtually all organ systems. Whereas most patients present without symptoms, progressive and disabling organ failure can occur in up to 10% of subjects. Somatostatin receptor (SSTR)-directed peptide receptor radionuclide therapy (PRRT) has recently received market authorization for treatment of SSTR-positive neuroendocrine tumors. Methods: We describe the first case series comprising two patients with refractory multi-organ involvement of sarcoidosis who received 4 cycles of PRRT. Results: PRRT was well-tolerated without any acute adverse effects. No relevant toxicities could be recorded during follow-up. Therapy resulted in partial response accompanied by a pronounced reduction in pain (patient #1) and stable disease regarding morphology as well as disease activity (patient #2), respectively. Conclusion: Peptide receptor radionuclide therapy in sarcoidosis is feasible and might be a new valuable tool in patients with otherwise treatment-refractory disease. Given the long experience with and good tolerability of PRRT, further evaluation of this new treatment option for otherwise treatment-refractory sarcoidosis in larger patient cohorts is warranted.

Light and Laser Modalities in the Treatment of Cutaneous Sarcoidosis: A Systematic Review.

Sarcoidosis is a systemic non-caseating granulomatous disease of unknown aetiology. Cutaneous manifestations are present in approximately 10-30% of the patients with the systemic form. Therapy is indicated in case of disabling symptoms, organ dysfunction or cosmetically distressing manifestation. Despite different therapeutic possibilities, cutaneous sarcoidosis remains exceptionally difficult to treat. Light and laser therapy may be a promising alternative. In this systematic review, we summarised the available treatments according to the literature concerning light and laser therapy for cutaneous sarcoidosis. Publications written in English and German, published between January 1990 and July 2016 in the database PubMed, MEDLINE, Embase, and Scopus were analysed. Light therapy with intense pulsed light, photodynamic therapy, and ultraviolet A light therapy, as well as laser therapy with pulsed dye laser, YAG laser, and Q-switched ruby laser were described. The results are based on individual case reports and small case series. Randomised controlled studies are lacking.

The use of lasers in cutaneous sarcoid: Is there a role?

Sarcoid is a granulomatous disease that can affect the skin solely or as part of a systemic involvement. Cutaneous lesions of sarcoid may be difficult to treat and lasers are rarely used. In this article we report our case series and review the literature on the use of lasers in cutaneous sarcoid.

Remission of refractory neurosarcoidosis treated with brain radiotherapy: a case report and a literature review.

Sarcoidosis is a chronic disease of unknown etiology characterized by the presence of T lymphocytes, mononuclear phagocytes, and noncaseating epithelioid granulomas in the tissues. Central nervous system involvement occurs in about 5% of the cases. The chronic form of neurosarcoidosis is particularly resistant to medical treatments. No universally accepted therapeutic protocols are currently available. Corticosteroids are the first line of therapy, but other immunosuppressive treatments are frequently added to the patient's regimen, although this strategy is not adequately supported by controlled clinical trials. For patients resistant to or not tolerating multiple alternate immunotherapeutic drugs, some authors suggest central nervous system radiotherapy. We present a case of a patient with neurosarcoidosis involving the hypothalamo-hypophyseal region and causing panhypopituitarism who had a poor response to and experienced severe side effects from conventional immunosuppressive treatments. The patient experienced a good clinical response to cranial irradiation. We review the literature on this subject.

Treatment of periocular and non-ocular sarcoids in 18 horses by interstitial brachytherapy with iridium-192.

Treatment of the equine sarcoid has posed a significant challenge to clinicians for years and many different methods have been tried with varying success, including ionising radiation. The aim of this study was to review the efficacy of iridium-192 interstitial brachytherapy for the treatment of eight periocular sarcoids and 15 non-ocular sarcoids on 18 horses. All the periocular sarcoids and 13 of the 15 non-ocular sarcoids were treated successfully.

Orbital lymphoma with concomitant sarcoid-like granulomas.

A 41-year-old man presented with chronic eyelid swelling, conjunctival injection, and decreased ocular motility in all gaze directions. MRI showed bilateral enlarged extraocular muscles, including the tendons. Laboratory tests revealed elevated levels of angiotensin-converting enzyme. An orbital biopsy showed collections of monotonous small lymphocytes, and granulomatous inflammation that included multinucleated giant cells, predominantly Langhans type. Flow cytometric analysis of tissue demonstrated a light chain-restricted clonal population of B cells, a finding that confirmed the morphologic impression of lymphoma. This case demonstrates that elevated angiotensin-converting enzyme and granulomatous inflammation can occur in lymphoma. Careful histopathologic examination and flow cytometric analysis are essential to avoid an erroneous diagnosis that could lead to inappropriate management.

Neurosarcoidosis: an unusual indication for radiotherapy.

Up to 70% of patients with systemic sarcoidosis developing neurosarcoidosis do so within the first 2 years of their systemic illness. Central nervous system (CNS) involvement as the only manifestation of sarcoidosis can be seen both at first time of disease and at recurrence in a few isolated cases. A young man showed neurological symptoms caused by isolated CNS sarcoidosis after unsuccessful treatment of primary pulmonary sarcoidosis by steroids. MRI scans of the head showed a distinct structural lesion temporodorsal in the left hemisphere and in the left-sided basal ganglia. The diagnosis was proved by neurosurgical resection. Post-operative systemic treatment with long-term corticosteroids was ineffective. After low-dose whole-brain irradiation of the isolated CNS lesion with 20 Gy, partial resolution of the clinical features and stabilization of disease proved by MRI ensued. In neurosarcoidosis the use of radiation therapy remains an appropriate therapy option with minimal adverse sequelae if primary medical treatment fails.

Sciatic nerve sarcoidosis: utility of magnetic resonance peripheral nerve imaging and treatment with radiation therapy.

Sarcoidosis may involve both the central and peripheral nervous system, although peripheral nerve manifestations are usually seen late in the disease. In this report, the authors describe a case of sarcoidosis in a 22-year-old woman who presented with a foot drop. Although results of conventional lumbar magnetic resonance (MR) imaging were normal, MR peripheral nerve imaging of the thigh showed a mass in the sciatic nerve indicating tumor. An intraoperative biopsy sample revealed noncaseating granulomas consistent with sarcoid. The patient was treated with steroid drugs to control the manifestations of her disease but exhibited early signs of femoral bone necrosis, which required discontinuation of the steroids. She was then treated with local radiation therapy. At her 2-year follow-up visit the patient demonstrated relief of her symptoms and improvement on MR peripheral nerve imaging. This case demonstrates that sarcoidosis may present with peripheral nerve manifestations. The appearance of a diffusely swollen nerve on MR imaging should prompt clinicians to include sarcoidosis in the differential diagnosis and plan surgery accordingly. Patients who are not responsive to or who are unable to tolerate medical therapy may be treated with radiation therapy.

Role of radiotherapy in the treatment of neurosarcoidosis.

Neurosarcoidosis is usually managed with steroids, immunosuppressives, and other medications. Several small series suggest that radiotherapy might be useful in patients whose disease is refractory to conventional treatment. The purpose of this article is to report the outcome of 4 patients with neurosarcoidosis who were treated at the University of Florida. With long-term follow up, partial regression of disease was observed in 2 patients, stabilization of disease in 1 patient, and disease progression in 1 patient. Our experience and review of the related literature suggest the following conclusions: (1) radiotherapy is often effective in preventing the progression of local symptoms from neurosarcoidosis, but has limited application in reversing established neurologic deficits; (2) sarcoid meningitis is responsive to radiotherapy; and (3) radiation dose for the palliation of symptoms related to neurosarcoidosis is 20 to 25 Gy.

[Medium-dose UV-A1 phototherapy. Successful treatment of cutaneous sarcoidosis]. / Medium-dose-UV-A1-Phototherapie. Erfolgreiche Behandlung einer kutanen Sarkoidose.

A 82-year-old female had a 2-year history of cutaneous sarcoidosis without systemic involvement. Various treatments including local glucocorticosteroids and tacrolimus ointment had failed. Therefore, we treated our patient with medium-dose UVA1 phototherapy. After 50 sessions with a total dose of 2.640 J/cm(2) all lesions had disappeared. Clinical follow up showed no recurrence of skin lesions after 5 months.

Management of treatment-resistant cutaneous sarcoidosis with radiation.

Cutaneous manifestations of sarcoidosis frequently respond to medical management. Occasionally, however, this problem will reappear with the discontinuation of medication and ultimately result in the scarring and disfigurement of the patient's skin. Such a patient was treated with radiation therapy and had considerable improvement in her acute disease.