Проблема с лёгкими. Врачи не могут определить заболевание

[kesha]

Здравствуйте!

Моей тёте 75 лет, она живёт в Бельгии. Примерно неделю назад её госпитализировали с проблемами лёгких: она находится на искусственном дыхании. Местные врачи говорят, что они не могут диагностировать болезнь т.к. с такой болезнью не встречались.
Может кто-то сталкивался с подобным и может подсказать в каком направлении искать причину болезни.
Ниже опубликую описание на английском языке и снимки лёгких.

Заранее благодарен за любой совет.
С уважением, Александр.

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KUZMINA NADEJDA, was admitted to the Medical Intensive Care Unit (MICU) on 24/06/2025.

Type I respiratory failure – MDA-5 AL positive myositis

Referred from AZ Vitaz. The patient was initially admitted to the pulmonary department with findings consistent with cryptogenic organizing pneumonia (COP). Bronchoalveolar lavage (BAL) showed a weakly positive PCR for Pneumocystis jirovecii (PJP), Bactrim was initiated. Despite this, respiratory deterioration continued, leading to transfer to the intensive care unit at AZ Vitaz on 18/06 with high flow nasal oxygen. During the stay at Vitaz, redness was noted on the forehead and upper arms. Skin biopsy from the forehead showed perivascular dermatitis, compatible with lupus. However, after reassessment based on a myositis blot, the diagnosis was revised. The myositis blot on 24/06 was positive for MDA-5 AL and NXP-2 AL, with suspicion of pulmonary involvement of a systemic disease, prompting transfer to UZ Gent. Prior to transfer, treatment with Cyclophosphamide 1.2 g and SoluMedrol 1 g (started on 23/06) was already initiated.

At admission to MICU, the patient was hemodynamically stable but remained dyspneic under high-flow nasal oxyten therapy. Biochemistry showed a low inflammatory profile with CRP 7.8 mg/L. In coordination with reumatologists, Prograf was added (target level 7–9) to the regimen of Cyclophosphamide and SoluMedrol 1 g. After 3 days of SoluMedrol 1 g IV, therapy was switched to Methylprednisolone 1 mg/kg PO daily. On pneumology staff discussion, continuation of multimodal immunosuppressive therapy was advised. Given the high dose corticosteroids, supportive treatment with calcium and vitamin D supplementation as well as a PPI for gastric protection was initiated.

On 01/07, spontaneous pneumothorax and pneumomediastinum were diagnosed, with radiological evidence of increased ground-glass opacities and consolidations in both lower lobes. After consultation with Rheumatology, intravenous immunoglobulins 2 mg/kg (120 g total) were administered over 3 days. On pneumology staff meeting (02/07), the option of starting JAK-inhibition was discussed. Following Rheumatology advice, treatment with Xeljanz 5 mg twice daily was started on 03/07.

At this point in treatment we can not objectify significant improvement / respons to therapy.

Thoracic pain left hemithorax

On 01/07, the patient developed respiratory effort-related pain in the left hemithorax. Chest X-ray revealed a small pneumothorax. Additional thoracic CT confirmed a left pneumothorax with a maximum pleural separation of approximately 8 mm, as well as pneumomediastinum. Known interstitial lung disease showed diffuse subpleural reticulations and increased ground-glass opacities and consolidations in both lower lobes. Respiratory status remained stable with steady Optiflow settings. In consultation with thoracic vascular surgery, a conservative approach was adopted.

Hypotonic hyponatremia – 124 mmol/L

At admission, hyponatremia was noted at 124 mmol/L despite a sodium level of 134 mmol/L a few hours earlier. Urine osmolality was 210 mOsm/kg and urine sodium 30 mmol/L. This was likely related to high-volume IV 5% glucose administration used as a flush after Cyclophosphamide treatment. The infusion was switched from 5% glucose to 0.9% NaCl, resulting in favorable correction of natremia.

Mild inflammatory blood profile

Subfebrile temperature with mild inflammatory markers and dyspnea led to empiric initiation of Ceftriaxone. Given negative cultures, antibiotics were stopped after 3 days. Subsequently, CRP remained stable, fluctuating between 40–50 mg/L without fever. This was presumably due to insufficiently controlled inflammation as described in point 1

[Dr.Vad]

достаточно известное во всем мире заболевание, кратко об общих принципах лечения - здесь:

A Review of MDA-5 Dermatomyositis and Associated Interstitial Lung Disease 2024

[Ссылки доступны только зарегистрированным пользователям ]

если нет эффекта от стандартной терапии - тотальное замещение плазмы: дорого, но работает у большинства пациентов

Effectiveness and safety of plasma exchange for anti-MDA5 antibody-positive clinically amyopathic dermatomyositis with rapidly progressive interstitial lung disease refractory to intensive immune suppression therapy: A case series
[Ссылки доступны только зарегистрированным пользователям ]

Short-term efficiency of plasma exchange in combination with immunosuppressants and/or biologics in the treatment of idiopathic inflammatory myopathy with rapidly progressive interstitial lung disease: a systematic review and meta-analysis
[Ссылки доступны только зарегистрированным пользователям ]

One-year survival benefit of plasma exchange in idiopathic inflammatory myositis patients with progressive interstitial lung disease-a systemic review and meta-analysis
[Ссылки доступны только зарегистрированным пользователям ]